HAEM5:Primary cutaneous gamma/delta T-cell lymphoma: Difference between revisions

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Haematolymphoid Tumours (WHO Classification, 5th ed.)

Primary Author(s)*

Mahzad Azimpouran, MD; Sumire Kitahara, MD; Cedars-Sinai, Los Angeles, CA

WHO Classification of Disease

Structure	Disease
Book	Haematolymphoid Tumours (5th ed.)
Category	T-cell and NK-cell lymphoid proliferations and lymphomas
Family	Mature T-cell and NK-cell neoplasms
Type	Primary cutaneous T-cell lymphoid proliferations and lymphomas
Subtype(s)	Primary cutaneous gamma/delta T-cell lymphoma

Related Terminology

Acceptable	N/A
Not Recommended	N/A

Gene Rearrangements

Driver Gene	Fusion(s) and Common Partner Genes	Molecular Pathogenesis	Typical Chromosomal Alteration(s)	Prevalence -Common >20%, Recurrent 5-20% or Rare <5% (Disease)	Diagnostic, Prognostic, and Therapeutic Significance - D, P, T	Established Clinical Significance Per Guidelines - Yes or No (Source)	Clinical Relevance Details/Other Notes
Arm‑level chromosomal alterations (e.g., 9p, 18q deletions; 1q, 7q,15q gains)	—	Copy number loss or gain → altered gene dosage of tumour suppressors/oncogenes	Other / chromosomal alteration	Recurrent (5‑20%) (9p del ~22%, 18q del ~22%; 1q/7q/15q gains ~33‑39%)	D / P	No	These structural changes suggest genomic instability and aggressive biology; may help risk stratification though not diagnostic per se^[1]
Fusion: FYN :: (probable partner TRAF3IP2)	TRAF3IP2	Structural alteration – deletion/exon8 deletion → (in other T‑cell lymphomas) FYN::TRAF3IP2 fusion leading to SRC‑family kinase activation; in this PCGDTCL case FYN exon8 deletion noted	Oncogene / Other	Rare (<5%) (single case reported)	T	No	Very recently described; may represent novel driver/target; further cases needed^[2]
Fusion: PCM1 :: JAK2	PCM1	Fusion → juxtaposition of dimerization domain of PCM1 with kinase domain of JAK2 → constitutive JAK2 activation	Oncogene	Rare (<5%) (single documented PCGDTCL case)	T	No	Known in other T‑cell and myeloid neoplasms; in PCGDTCL this double‐hit case had PCM1::JAK2 + TBL1XR1::TP63 fusion; patient refractory to JAK inhibitor^[3]
Fusion: TBL1XR1 :: TP63	TBL1XR1	Fusion → truncation/overexpression of ΔNp63 form → oncogenic p63 signalling	Oncogene / Other	Rare (<5%) (same single case) (	P / T	No	Associated with aggressive behaviour in T‑cell lymphomas; in the reported PCGDTCL case contributed to aggressive course and JAK inhibitor resistance^[3]

Individual Region Genomic Gain/Loss/LOH

Chr #	Gain, Loss, Amp, LOH	Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]	Relevant Gene(s)	Diagnostic, Prognostic, and Therapeutic Significance - D, P, T	Established Clinical Significance Per Guidelines - Yes or No (Source)	Clinical Relevance Details/Other Notes
1p	Loss	1p36.11	ARID1A	P	No	Deleted in ~28% of cases. Indicates epigenetic/chromatin modifier pathway involvement^[1]
1q	Gain (arm‐level amplification)	1q (approx chr1:144,000,000‑249,000,000)	Multiple genes on 1q (unspecified)	P / T	No	Amplification in ~33% of cases. Potential gene dosage effect; specific driver gene not yet defined^[1]
2q	Loss	2q37.3	PDCD1	P	No	Deletion in ~22% of cases. Immune checkpoint gene loss; potential therapeutic‑escape mechanism^[1]
7q	Gain (arm‐level)	7q (approx chr7:100,000,000‑159,000,000)	Multiple genes on 7q (unspecified)	P	No	Amplification in ~39% of cases. Suggests MAPK/other pathway involvement but specific gene not yet defined.
9p	Loss (deletion)	9p21.3 (~ chr9:21,900,000‑22,200,000)	CDKN2A, CDKN2B	P	No	High‐frequency homozygous or biallelic deletion (~61% of cases; 45% biallelic) in PCGDTCL. (PMC) Suggests aggressive biology, prognostic marker candidate^[1]
10q	Loss	10q24.1	FAS	P	No	Deletion in ~22% of cases. Loss of apoptosis regulator; may contribute to immune‑escape^[1]
15q	Gain (arm‐level)	15q (approx chr15:30,000,000‑102,000,000)	Multiple genes on 15q (unspecified)	P	No	Amplification in ~33% of cases. Likely reflects tumour evolution rather than diagnostic biomarker^[1]
18q	Loss	18q (arm level; no precise minimal region specified)	Putative tumour suppressors (unspecified)	P	No	Recurrent deletion ~22% in PCGDTCL cohort. May reflect genomic instability and poor outcome^[1]

Characteristic Chromosomal or Other Global Mutational Patterns

Chromosomal Pattern	Molecular Pathogenesis	Prevalence - Common >20%, Recurrent 5-20% or Rare <5% (Disease)	Diagnostic, Prognostic, and Therapeutic Significance - D, P, T	Established Clinical Significance Per Guidelines - Yes or No (Source)	Clinical Relevance Details/Other Notes
Arm‑level somatic copy‑number variation (SCNV) (average ~4 arm‑level events per case; median ~166.5 SCNVs per sample)^[1]	Reflects genomic instability; multiple gains and losses of whole chromosome arms likely contribute to oncogenesis and progression by altering gene dosage of multiple oncogenes/tumour suppressors simultaneously. (PMC)	Common (>20%) — nearly all cases show multiple arm‑level events (median 4 per sample) (PMC)	P	No	High genomic complexity may explain aggressive behaviour and poor response to therapy. Could impact prognosis or treatment resistance but not yet in guidelines.
High burden of somatic copy‑number variants (SCNVs) relative to single‐nucleotide variants (SNVs) (e.g., median ~166.5 SCNVs per sample) ^[1]	Suggests that structural genomic alterations dominate the mutational landscape, perhaps more so than classical hotspot SNVs, indicating a biology driven by large‑scale genomic disruption rather than just point mutations.	Common (>20%)	P	No	Recognising this pattern may guide expectation of complexity, but this is not currently used clinically for diagnosis or treatment.
Distinct cell‑of‑origin signature: Vδ1 vs Vδ2 subtype (epidermal/dermal Vδ1 vs panniculitic Vδ2) ^[1]	Different tissue compartments (epidermis/dermis vs subcutaneous) correspond to distinct γδ T‑cell subsets (Vδ1 vs Vδ2). The cell‑of‑origin influences mutational signatures (eg UV signature in Vδ1) and clinical phenotype (Vδ2 more aggressive)^[1]	Recurrent (5‑20%) — this pattern applies in a subset of cases defined by tissue involvement and TCR subtype.	D / P	No	This dichotomy may help stratify patients clinically (Vδ2 subtype worse prognosis) but is not currently part of formal diagnostic or therapeutic guidelines.
Ultraviolet (UV) mutational signature in Vδ1 subtype ^[1]	The epidermal/dermal Vδ1 γδ T‑cell lymphomas exhibit a UV signature in their mutation spectrum, likely reflecting skin localization and UV exposure contributing to oncogenesis.	Recurrent (5‑20%) — seen in Vδ1 cases but not all.	P	No	Could suggest etiology and may influence prognosis; though not yet used for therapy selection.
Frequent deletions of 9p21.3 (CDKN2A region) (part of the SCNV pattern) ^[1]	Loss of CDKN2A/p14^ARF leads to cell‑cycle deregulation, loss of tumour suppressor control: a hallmark of many aggressive lymphomas	Common (>20%) (approx 61% of cases)	P	No	Among the most prevalent genomic events in PCGDTCL — potential prognostic marker though not yet guideline‑endorsed.
Multiple gains of oncogenic arms (e.g., 1q, 7q, 15q) and corresponding losses (eg 18q) ^[1]	Gains may increase dosage of oncogenes; losses may reduce tumour suppressor dosage—together contributing to malignant phenotype	Recurrent (5‑20%) for specific arm‑level changes (e.g., 1q gain ~33%, 7q ~39%, 15q ~33%)	P	No	These arm‑level events indicate complexity; may correlate with poorer prognosis; not yet actionable in therapy.
TCR chain repertoire restriction / non‑random Vγ or Vδ usage (eg Vγ3Vδ2 in panniculitic cases) ^[1]	Suggests antigen‑driven or tissue‐resident γδ T‑cell proliferation; highlights non‑random selection of malignant clones	Recurrent (5‑20%) in defined subtypes	D	No	Might help refine subclassification of PCGDTCL; not currently used in routine diagnostic algorithms.

Gene Mutations (SNV/INDEL)

Gene	Genetic Alteration	Tumor Suppressor Gene, Oncogene, Other	Prevalence - Common >20%, Recurrent 5-20% or Rare <5% (Disease)	Diagnostic, Prognostic, and Therapeutic Significance - D, P, T	Established Clinical Significance Per Guidelines - Yes or No (Source)	Clinical Relevance Details/Other Notes
STAT5B	Activating missense (e.g., p.N642H) → constitutive downstream STAT5 signalling	Oncogene	Recurrent (~5‑20 %) — e.g., in the 2020 genomic study: JAK/STAT mutations ~21 % of cases^[1]	T / P: Therapeutic potential (JAK/STAT inhibition); Prognostic implication (pathway addiction/resistance)	No	Mutant STAT5B (especially N642H) shown to induce T‑cell neoplasia in models; in PCGDTCL JAK/STAT addiction shown clinically ^[4]^[5]
STAT3	Activating missense (SH2 domain) → constitutive STAT3 signalling	Oncogene	Rare (<5 %) to Recurrent (≈5‑10 %) (in NK/γδ‑T lymphomas earlier)	T / P	No	Less frequent than STAT5B in PCGDTCL; part of JAK/STAT pathway involvement^[1]^[4]
JAK3	Activating mutation (e.g., p.R657W) → JAK3 tyrosine kinase activation	Oncogene	Rare (<5 %) (noted in the Daniels et al. cohort)	T	No	Supports JAK/STAT involvement; one case report showed response to JAK inhibition^[5]
KRAS	Activating hotspot mutations (e.g., G12D, Q61H, D119N) → RAS/MAPK activation	Oncogene	Recurrent (~5‑20 %) — “KRAS was the most frequently mutated oncogene” ^[1]	T / P	No	MAPK pathway appears relevant; patients with MAPK‑pathway driver mutations had worse survival in the cohort^[1]
NRAS	Activating hotspot mutation → RAS/MAPK activation	Oncogene	Rare (<5 %) to Recurrent (~5‑10 %)	T / P	No	Part of the same RAS/MAPK pathway as KRAS; less common.
MAPK1	Activating mutation → MAPK1 signalling activation	Oncogene	Rare (<5 %)	T	No	Also in MAPK pathway; limited data in PCGDTCL^[1]^[4]
MYC	Activating missense mutation (e.g., p.P74L) → MYC pathway up‑regulation	Oncogene	Rare (<5 %)	P / T	No	MYC pathway involvement may contribute to the aggressive phenotype; direct targeting not yet established^[1]
MYCN	Activating mutation (e.g., p.G34R) → MYCN pathway activation	Oncogene	Rare (<5 %)	P / T	No	Highlights involvement of MYC‑family beyond MYC itself in this disease^[1]

Note: A more extensive list of mutations can be found in cBioportal, COSMIC, and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

Epigenomic Alterations

N/A

Genes and Main Pathways Involved

Gene; Genetic Alteration	Pathway	Pathophysiologic Outcome
DNMT3A (DNA methyltransferase)	Loss‑of‑function mutations or deletions → reduced de novo DNA methylation; “epigenetic writer” defect (DNA methylation pathway)^[6]	Deregulation of gene silencing; tumour suppressor genes may remain unmethylated or aberrantly methylated → genomic instability, aberrant T‑cell differentiation/activation
TET2 (methylcytosine dioxygenase)	Loss‑of‑function mutations → failure of DNA 5‑mC → 5‑hmC demethylation (“epigenetic eraser” defect)^[6]	Aberrant hypermethylation or demethylation patterns; influences T‑cell development and malignant transformation (e.g., in T‑fh lymphomas)
IDH2 (metabolic enzyme altering epigenome)	Gain‑of‑function mutation (e.g., R172) → produces 2‑hydroxyglutarate → inhibits TET family → epigenetic dysregulation^[6]	Oncometabolite‑driven methylation changes, impaired differentiation, proliferation of malignant T cells
ARID1A (SWI/SNF chromatin‑remodeller)	Loss‑of‑function mutation/deletion → impaired nucleosome remodelling, altered chromatin accessibility (“chromatin remodeller”)^[6]	Reduced tumour‑suppressor gene expression due to chromatin compaction; may influence immune microenvironment and genomic instability
KMT2D / KMT2A (H3K4 methyltransferases)	Loss‑of‑function mutations (“histone‑writer” defect) → decreased H3K4 methylation (activating mark)^[7]	Impaired activation of gene expression programs (differentiation, apoptosis) → contributes to malignant transformation
KDM6A (H3K27 demethylase)	Loss‑of‑function → accumulation of H3K27me3 (repressive histone mark) (“histone‑eraser” defect)^[7]	Further chromatin repression of tumour‑suppressor genes; may enhance survival of malignant T cells
EZH2 (PRC2 complex methyltransferase)	Overexpression/gain of function → increased H3K27me3 (“histone‑writer” overactivity) ^[6]	Enhanced silencing of differentiation/apoptosis genes; contributes to aggressive lymphoma phenotypes
CREBBP / EP300 (histone acetyl‑transferases)	Loss‑of‑function mutations (“histone‑writer” defect) → reduced histone acetylation and gene activation^[7]	Diminished transcriptional activation of tumour‑suppressor/immune genes; may drive malignant progression
DNA methylation of specific tumour‑suppressor loci (e.g., CDKN2A promoter; FAS promoter)	Hypermethylation of promoter CpG islands → silencing of tumor suppressor / apoptosis‑initiator genes^[8]	Loss of cell‑cycle control or apoptosis leads to malignant T‑cell survival/proliferation

Genetic Diagnostic Testing Methods

Method	Description	Type of Alteration Detected	Advantages	Limitations	Clinical Use in PCGDTCL
Next-Generation Sequencing (NGS)	High-throughput sequencing of targeted gene panels, whole-exome, or whole-genome sequencing	SNVs, INDELs, copy number variants (CNVs), some fusions (if RNA-seq included)	Comprehensive mutation detection; scalable; can detect multiple variants simultaneously	Requires high-quality DNA/RNA; bioinformatics expertise needed; cost-intensive	Main tool for mutational profiling in PCGDTCL; used in research and increasingly in clinical labs
Targeted Gene Panels (amplicon or hybrid capture-based)	Sequencing of a defined set of genes known to be relevant	SNVs, INDELs, limited CNVs, hotspot fusions (if included)	Faster, cheaper than WES/WGS; focused on clinically relevant genes	May miss novel or unexpected mutations; limited to panel content	Often used clinically to screen for mutations in JAK/STAT, RAS pathways in PCGDTCL
Fluorescence In Situ Hybridization (FISH)	DNA probes hybridize to metaphase or interphase chromosomes	Structural chromosomal alterations, gene fusions, amplifications, deletions	Visualizes gene rearrangements and copy number changes; established clinical use	Limited to known targets; low resolution; labor-intensive	Used to detect known translocations or gene amplifications (e.g., MYC) in lymphoma diagnosis
Array Comparative Genomic Hybridization (aCGH) / SNP Arrays	Genome-wide detection of copy number alterations and LOH	Copy number gains, losses, LOH (Loss of heterozygosity)	Genome-wide coverage; detects submicroscopic CNVs	Cannot detect balanced translocations or point mutations; resolution depends on array density	Useful for detecting large chromosomal alterations in lymphoma samples
RNA Sequencing (RNA-Seq)	Sequencing of transcriptome	Gene fusions, splice variants, expression levels	Detects novel and known fusions; measures gene expression; alternative splicing	RNA quality sensitive; bioinformatics expertise needed	Research use for identifying novel fusion partners or expression signatures in PCGDTCL
Sanger Sequencing	Chain termination sequencing of PCR-amplified regions	SNVs and small indels	Gold standard for validation; high accuracy	Low throughput; not suitable for large panels	Used to confirm NGS-identified mutations
Digital Droplet PCR (ddPCR) / qPCR	Highly sensitive quantification of known mutations or gene rearrangements	Known point mutations, copy number changes	Very sensitive, quantitative; fast turnaround	Limited to known mutations; not comprehensive	Useful for monitoring known mutations (e.g., STAT5B N642H) in minimal residual disease (MRD) or treatment response
Immunohistochemistry (IHC) (surrogate genetic marker)	Antibody staining of protein expression	Protein expression reflecting genetic alterations (e.g., pSTAT5B, MYC)	Widely available; easy to implement	Indirect; may not perfectly correlate with mutation status	Supportive role in diagnosis and prognosis, not definitive genetic test

Familial Forms

There are currently no well-established familial or hereditary forms described in the literature.

Additional Information

N/A

Links

N/A

References

↑ ^1.00 ^1.01 ^1.02 ^1.03 ^1.04 ^1.05 ^1.06 ^1.07 ^1.08 ^1.09 ^1.10 ^1.11 ^1.12 ^1.13 ^1.14 ^1.15 ^1.16 ^1.17 ^1.18 ^1.19 ^1.20 ^1.21 ^1.22 Daniels, Jay; et al. (2020-04-14). "Cellular origins and genetic landscape of cutaneous gamma delta T cell lymphomas". Nature Communications. 11 (1): 1806. doi:10.1038/s41467-020-15572-7. ISSN 2041-1723. PMC 7156460 Check |pmc= value (help). PMID 32286303 Check |pmid= value (help).
↑ Azimpouran, Mahzad; et al. (2024-12-01). "Rapidly Progressive Primary Cutaneous Gamma Delta T-Cell Lymphoma With FYN Gene Alteration". The American Journal of Dermatopathology. 46 (12): e120–e123. doi:10.1097/DAD.0000000000002856. ISSN 1533-0311. PMID 39412302 Check |pmid= value (help).
↑ ^3.0 ^3.1 Fadl, Amr; et al. (2023-09). "Primary cutaneous gamma/delta T-cell lymphoma with simultaneous JAK2 and TP63 rearrangements: a new double-hit?". Histopathology. 83 (3): 492–495. doi:10.1111/his.14973. ISSN 1365-2559. PMC 10524708 Check |pmc= value (help). PMID 37308177 Check |pmid= value (help). Check date values in: |date= (help)
↑ ^4.0 ^4.1 ^4.2 Küçük, Can; et al. (2015-01-14). "Activating mutations of STAT5B and STAT3 in lymphomas derived from γδ-T or NK cells". Nature Communications. 6: 6025. doi:10.1038/ncomms7025. ISSN 2041-1723. PMC 7743911 Check |pmc= value (help). PMID 25586472.
↑ ^5.0 ^5.1 Zhang, Yue; et al. (2025-04-15). "Addiction of primary cutaneous γδ T cell lymphomas to JAK/STAT signaling". The Journal of Clinical Investigation. 135 (8): e180417. doi:10.1172/JCI180417. ISSN 1558-8238. PMC 11996904 Check |pmc= value (help). PMID 40231467 Check |pmid= value (help).
↑ ^6.0 ^6.1 ^6.2 ^6.3 ^6.4 Zhang, Ping; et al. (2020-11-07). "Epigenetic alterations and advancement of treatment in peripheral T-cell lymphoma". Clinical Epigenetics. 12 (1): 169. doi:10.1186/s13148-020-00962-x. ISSN 1868-7083. PMC 7648940 Check |pmc= value (help). PMID 33160401 Check |pmid= value (help).
↑ ^7.0 ^7.1 ^7.2 Ahmed, Nada; et al. (2020-02). "Targeting epigenetic regulators in the treatment of T-cell lymphoma". Expert Review of Hematology. 13 (2): 127–139. doi:10.1080/17474086.2020.1711732. ISSN 1747-4094. PMC 7110907 Check |pmc= value (help). PMID 31903826. Check date values in: |date= (help)
↑ Hara, Natsumi; et al. (2022-03-24). "Epigenetics of Cutaneous T-Cell Lymphomas". International Journal of Molecular Sciences. 23 (7): 3538. doi:10.3390/ijms23073538. ISSN 1422-0067. PMC 8998216 Check |pmc= value (help). PMID 35408897 Check |pmid= value (help).

Notes

*Citation of this Page: Azimpouran M, Kitahara S. “Primary cutaneous gamma/delta T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 01/6/2026, https://ccga.io/index.php/HAEM5:Primary_cutaneous_gamma/delta_T-cell_lymphoma.

*Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the Associate Editor or other CCGA representative. When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.

Prior Author(s): N/A

[:0-1] 1.00 ^1.01 ^1.02 ^1.03 ^1.04 ^1.05 ^1.06 ^1.07 ^1.08 ^1.09 ^1.10 ^1.11 ^1.12 ^1.13 ^1.14 ^1.15 ^1.16 ^1.17 ^1.18 ^1.19 ^1.20 ^1.21 ^1.22 Daniels, Jay; et al. (2020-04-14). "Cellular origins and genetic landscape of cutaneous gamma delta T cell lymphomas". Nature Communications. 11 (1): 1806. doi:10.1038/s41467-020-15572-7. ISSN 2041-1723. PMC 7156460 Check |pmc= value (help). PMID 32286303 Check |pmid= value (help).

[2] Azimpouran, Mahzad; et al. (2024-12-01). "Rapidly Progressive Primary Cutaneous Gamma Delta T-Cell Lymphoma With FYN Gene Alteration". The American Journal of Dermatopathology. 46 (12): e120–e123. doi:10.1097/DAD.0000000000002856. ISSN 1533-0311. PMID 39412302 Check |pmid= value (help).

[:2-3] 3.0 ^3.1 Fadl, Amr; et al. (2023-09). "Primary cutaneous gamma/delta T-cell lymphoma with simultaneous JAK2 and TP63 rearrangements: a new double-hit?". Histopathology. 83 (3): 492–495. doi:10.1111/his.14973. ISSN 1365-2559. PMC 10524708 Check |pmc= value (help). PMID 37308177 Check |pmid= value (help). Check date values in: |date= (help)

[:1-4] 4.0 ^4.1 ^4.2 Küçük, Can; et al. (2015-01-14). "Activating mutations of STAT5B and STAT3 in lymphomas derived from γδ-T or NK cells". Nature Communications. 6: 6025. doi:10.1038/ncomms7025. ISSN 2041-1723. PMC 7743911 Check |pmc= value (help). PMID 25586472.

[:3-5] 5.0 ^5.1 Zhang, Yue; et al. (2025-04-15). "Addiction of primary cutaneous γδ T cell lymphomas to JAK/STAT signaling". The Journal of Clinical Investigation. 135 (8): e180417. doi:10.1172/JCI180417. ISSN 1558-8238. PMC 11996904 Check |pmc= value (help). PMID 40231467 Check |pmid= value (help).

[:4-6] 6.0 ^6.1 ^6.2 ^6.3 ^6.4 Zhang, Ping; et al. (2020-11-07). "Epigenetic alterations and advancement of treatment in peripheral T-cell lymphoma". Clinical Epigenetics. 12 (1): 169. doi:10.1186/s13148-020-00962-x. ISSN 1868-7083. PMC 7648940 Check |pmc= value (help). PMID 33160401 Check |pmid= value (help).

[:5-7] 7.0 ^7.1 ^7.2 Ahmed, Nada; et al. (2020-02). "Targeting epigenetic regulators in the treatment of T-cell lymphoma". Expert Review of Hematology. 13 (2): 127–139. doi:10.1080/17474086.2020.1711732. ISSN 1747-4094. PMC 7110907 Check |pmc= value (help). PMID 31903826. Check date values in: |date= (help)

[8] Hara, Natsumi; et al. (2022-03-24). "Epigenetics of Cutaneous T-Cell Lymphomas". International Journal of Molecular Sciences. 23 (7): 3538. doi:10.3390/ijms23073538. ISSN 1422-0067. PMC 8998216 Check |pmc= value (help). PMID 35408897 Check |pmid= value (help).

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HAEM5:Primary cutaneous gamma/delta T-cell lymphoma: Difference between revisions

Latest revision as of 22:48, 6 January 2026

Contents

Primary Author(s)*

WHO Classification of Disease

Related Terminology

Gene Rearrangements

Individual Region Genomic Gain/Loss/LOH

Characteristic Chromosomal or Other Global Mutational Patterns

Gene Mutations (SNV/INDEL)

Epigenomic Alterations

Genes and Main Pathways Involved

Genetic Diagnostic Testing Methods

Familial Forms

Additional Information

Links

References

Notes

Navigation menu

@@ Line 1: / Line 1: @@
-{{DISPLAYTITLE:Primary cutaneous gamma/delta T-cell lymphoma}}
 [[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
-{{Under Construction}}
-<span style="color:#0070C0">(General Instructions – The focus of these pages is the clinically significant genetic alterations in each disease type. This is based on up-to-date knowledge from multiple resources such as PubMed and the WHO classification books. The CCGA is meant to be a supplemental resource to the WHO classification books; the CCGA captures in a continually updated wiki-stye manner the current genetics/genomics knowledge of each disease, which evolves more rapidly than books can be revised and published. If the same disease is described in multiple WHO classification books, the genetics-related information for that disease will be consolidated into a single main page that has this template (other pages would only contain a link to this main page). Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ <u>HGVS-based nomenclature for variants</u>], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column in a table, click nearby within the table and select the > symbol that appears. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see </span><u>[[Author_Instructions]]</u><span style="color:#0070C0"> and [[Frequently Asked Questions (FAQs)|<u>FAQs</u>]] as well as contact your [[Leadership|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>].)</span>
 ==Primary Author(s)*==
+Mahzad Azimpouran, MD; Sumire Kitahara, MD; Cedars-Sinai, Los Angeles, CA
-Put your text here<span style="color:#0070C0"> (''<span class="blue-text">EXAMPLE:</span>'' Jane Smith, PhD) </span>
 ==WHO Classification of Disease==
@@ Line 36: / Line 29: @@
 {| class="wikitable"
-|+
 |Acceptable
 |N/A
@@ Line 45: / Line 37: @@
 ==Gene Rearrangements==
-Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>
+{| class="wikitable sortable"
-{| class="wikitable"
-|'''Gene / Fusion'''
-|'''Partner Gene (for fusion)'''
-|'''Genetic Alteration & Molecular   Pathogenesis'''
-|'''Tumour Suppressor / Oncogene / Other'''
-|'''Prevalence (common>20%, recurrent 5-20% or rare <5%)'''
-|'''D / P / T'''
-|'''Established clinical significance per guidelines?'''
-|'''Clinical relevance / Other notes'''
 |-
-|'''CDKN2A'''
+!Driver Gene!!Fusion(s) and Common Partner Genes!!Molecular Pathogenesis!!Typical Chromosomal Alteration(s)
-|—
+!Prevalence -Common >20%, Recurrent 5-20% or Rare <5% (Disease)
-|Homozygous or biallelic deletion → loss of  p16^INK4A/p14^ARF function (cell‑cycle control)
+!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
-|Tumour suppressor
+!Established Clinical Significance Per Guidelines - Yes or No (Source)
-|'''Common''' (>20%) (~61% in cohort)  (PubMed)
+!Clinical Relevance Details/Other Notes
-|P
-|No
-|High‐frequency  deletion, suggests aggressive biology and may be a prognostic marker
-|-
-|'''ARID1A'''
-|—
-|Deletion/truncating mutation → loss of chromatin‑remodelling  function
-|Tumour suppressor
-|Recurrent (5‑20%) (~28%) (PMC)
-|Other / P
-|No
-|Indicates involvement of epigenetic/chromatin pathways in  PCGDTCL
-|-
-|'''FAS'''
-|—
-|Focal or biallelic deletion → loss of apoptosis  signalling via FAS‑FASL pathway
-|Tumour suppressor / apoptotic regulator
-|Recurrent (5‑20%) (~22%) (PMC)
-|Other / P
-|No
-|Loss of FAS may contribute to immune‐escape of malignant γδ T‑cells
-|-
-|'''PDCD1'''
-|—
-|Deletion → loss of PD‑1 (immune‐checkpoint) regulatory  function
-|Tumour suppressor / immune‑regulator
-|Recurrent (5‑20%) (~22%) (PMC)
-|Other / P
-|No
-|Suggests immune‐escape  mechanism; potential implications for checkpoint therapy though unproven
-|-
-|'''STAT5B'''
-|—
-|Activating missense (e.g., N642H) → constitutive STAT5B  signalling (JAK/STAT pathway)
-|Oncogene
-|Recurrent (5‑20%) (JAK/STAT mutations ~21%) (PubMed)
-|T / P
-|No
-|JAK/STAT pathway dependency; early data suggest JAK‐inhibitor sensitivity in  analogous T‑cell neoplasms; investigational in PCGDTCL
-|-
-|'''STAT3'''
-|—
-|Activating missense → constitutive STAT3 signalling  (JAK/STAT cascade)
-|Oncogene
-|Rare (<5%) to Recurrent (~5‑20%) (PubMed)
-|T / P
-|No
-|Part of JAK/STAT alterations; less frequent than STAT5B  in PCGDTCL
-|-
-|'''JAK3'''
-|—
-|Activating mutation (e.g., R657W) → JAK3 tyrosine kinase  activation (JAK/STAT pathway)
-|Oncogene
-|Rare (<5%) (PMC)
-|T
-|No
-|Supports JAK/STAT pathway involvement; therapeutic  relevance remains investigational in this disease
-|-
-|'''KRAS'''
-|—
-|Activating hotspot mutations (e.g., G12D, Q61H) →  RAS/MAPK pathway activation
-|Oncogene
-|Recurrent (5‑20%) (PubMed)
-|T / P
-|No
-|MAPK pathway potentially targetable; mutations associated  with poorer outcome in the cohort studied
-|-
-|'''NRAS'''
-|—
-|Activating hotspot mutation → RAS/MAPK pathway activation
-|Oncogene
-|Rare (<5%) to Recurrent (~5‑20%) (PMC)
-|T / P
-|No
-|Part of same pathway as KRAS though less common
-|-
-|'''MYC'''
-|—
-|Activating missense mutation (e.g., P74L) → MYC pathway  up‑regulation
-|Oncogene
-|Rare (<5%) (PMC)
-|P / T
-|No
-|MYC pathway involvement may contribute to more aggressive  phenotype; direct targeting not yet established
-|-
-|'''MYCN'''
-|—
-|Activating mutation (e.g., G34R) → MYCN pathway  activation
-|Oncogene
-|Rare (<5%) (PMC)
-|P / T
-|No
-|Highlights involvement of MYC family beyond MYC itself in  PCGDTCL
 |-
 |'''Arm‑level chromosomal alterations (e.g., 9p,  18q deletions; 1q, 7q,15q gains)'''
@@ Line 159: / Line 49: @@
 |Copy number loss or gain → altered gene dosage of tumour  suppressors/oncogenes
 |Other / chromosomal alteration
-|Recurrent (5‑20%) (9p del ~22%, 18q del ~22%; 1q/7q/15q  gains ~33‑39%) (PMC)
+|Recurrent (5‑20%) (9p del ~22%, 18q del ~22%; 1q/7q/15q  gains ~33‑39%)
 |D / P
 |No
-|These structural changes suggest genomic instability and  aggressive biology; may help risk stratification though not diagnostic per se
+|These structural changes suggest genomic instability and  aggressive biology; may help risk stratification though not diagnostic per se<ref name=":0">{{Cite journal|last=Daniels|first=Jay|last2=Doukas|first2=Peter G.|last3=Escala|first3=Maria E. Martinez|last4=Ringbloom|first4=Kimberly G.|last5=Shih|first5=David J. H.|last6=Yang|first6=Jingyi|last7=Tegtmeyer|first7=Kyle|last8=Park|first8=Joonhee|last9=Thomas|first9=Jane J.|date=2020-04-14|title=Cellular origins and genetic landscape of cutaneous gamma delta T cell lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/32286303|journal=Nature Communications|volume=11|issue=1|pages=1806|doi=10.1038/s41467-020-15572-7|issn=2041-1723|pmc=7156460|pmid=32286303}}</ref>
 |-
 |'''Fusion: FYN :: (probable partner TRAF3IP2)'''
 |TRAF3IP2
-|Structural alteration – deletion/exon8 deletion → (in  other T‑cell lymphomas) FYN::TRAF3IP2 fusion leading to SRC‑family kinase  activation; in this PCGDTCL case FYN exon8 deletion noted (PubMed)
+|Structural alteration – deletion/exon8 deletion → (in  other T‑cell lymphomas) FYN::TRAF3IP2 fusion leading to SRC‑family kinase  activation; in this PCGDTCL case FYN exon8 deletion noted
 |Oncogene / Other
 |Rare (<5%) (single case reported)
 |T
 |No
-|Very recently described; may represent novel  driver/target; further cases needed
+|Very recently described; may represent novel  driver/target; further cases needed<ref>{{Cite journal|last=Azimpouran|first=Mahzad|last2=Bui|first2=Chau M.|last3=Balzer|first3=Bonnie|last4=Kitahara|first4=Sumire|date=2024-12-01|title=Rapidly Progressive Primary Cutaneous Gamma Delta T-Cell Lymphoma With FYN Gene Alteration|url=https://pubmed.ncbi.nlm.nih.gov/39412302|journal=The American Journal of Dermatopathology|volume=46|issue=12|pages=e120–e123|doi=10.1097/DAD.0000000000002856|issn=1533-0311|pmid=39412302}}</ref>
 |-
 |'''Fusion: PCM1 :: JAK2'''
@@ Line 177: / Line 67: @@
 |Fusion → juxtaposition of dimerization domain of PCM1  with kinase domain of JAK2 → constitutive JAK2 activation
 |Oncogene
-|Rare (<5%) (single documented PCGDTCL case) (PubMed)
+|Rare (<5%) (single documented PCGDTCL case)
 |T
 |No
-|Known in other T‑cell and myeloid neoplasms; in PCGDTCL  this double‐hit  case had PCM1::JAK2 + TBL1XR1::TP63 fusion; patient refractory to JAK  inhibitor
+|Known in other T‑cell and myeloid neoplasms; in PCGDTCL  this double‐hit  case had PCM1::JAK2 + TBL1XR1::TP63 fusion; patient refractory to JAK  inhibitor<ref name=":2">{{Cite journal|last=Fadl|first=Amr|last2=Bennani|first2=N. Nora|last3=Comfere|first3=Nneka|last4=Durani|first4=Urshila|last5=Greipp|first5=Patricia T.|last6=Feldman|first6=Andrew L.|date=2023-09|title=Primary cutaneous gamma/delta T-cell lymphoma with simultaneous JAK2 and TP63 rearrangements: a new double-hit?|url=https://pubmed.ncbi.nlm.nih.gov/37308177|journal=Histopathology|volume=83|issue=3|pages=492–495|doi=10.1111/his.14973|issn=1365-2559|pmc=10524708|pmid=37308177}}</ref>
 |-
 |'''Fusion: TBL1XR1 :: TP63'''
@@ Line 186: / Line 76: @@
 |Fusion → truncation/overexpression of ΔNp63 form →  oncogenic p63 signalling
 |Oncogene / Other
-|Rare (<5%) (same single case) (PubMed)
+|Rare (<5%) (same single case) (
 |P / T
 |No
-|Associated with aggressive behaviour in T‑cell lymphomas;  in the reported PCGDTCL case contributed to aggressive course and JAK  inhibitor resistance
+|Associated with aggressive behaviour in T‑cell lymphomas;  in the reported PCGDTCL case contributed to aggressive course and JAK  inhibitor resistance<ref name=":2" />
 |}
+==Individual Region Genomic Gain/Loss/LOH==
 {| class="wikitable sortable"
 |-
-!Driver Gene!!Fusion(s) and Common Partner Genes!!Molecular Pathogenesis!!Typical Chromosomal Alteration(s)
+!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
-!Prevalence -Common >20%, Recurrent 5-20% or Rare <5% (Disease)
 !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
 !Established Clinical Significance Per Guidelines - Yes or No (Source)
 !Clinical Relevance Details/Other Notes
 |-
-|<span class="blue-text">EXAMPLE:</span> ''ABL1''||<span class="blue-text">EXAMPLE:</span> ''BCR::ABL1''||<span class="blue-text">EXAMPLE:</span> The pathogenic derivative is the der(22) resulting in fusion of 5’ BCR and 3’ABL1.||<span class="blue-text">EXAMPLE:</span> t(9;22)(q34;q11.2)
+|1p
-|<span class="blue-text">EXAMPLE:</span> Common (CML)
-|<span class="blue-text">EXAMPLE:</span> D, P, T
-|<span class="blue-text">EXAMPLE:</span> Yes (WHO, NCCN)
-|<span class="blue-text">EXAMPLE:</span>
-The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference). BCR::ABL1 is generally favorable in CML (add reference).
-|-
-|<span class="blue-text">EXAMPLE:</span> ''CIC''
-|<span class="blue-text">EXAMPLE:</span> ''CIC::DUX4''
-|<span class="blue-text">EXAMPLE:</span> Typically, the last exon of ''CIC'' is fused to ''DUX4''. The fusion breakpoint in ''CIC'' is usually intra-exonic and removes an inhibitory sequence, upregulating ''PEA3'' genes downstream of ''CIC'' including ''ETV1'', ''ETV4'', and ''ETV5''.
-|<span class="blue-text">EXAMPLE:</span> t(4;19)(q25;q13)
-|<span class="blue-text">EXAMPLE:</span> Common (CIC-rearranged sarcoma)
-|<span class="blue-text">EXAMPLE:</span> D
-|
-|<span class="blue-text">EXAMPLE:</span>
-''DUX4'' has many homologous genes; an alternate translocation in a minority of cases is t(10;19), but this is usually indistinguishable from t(4;19) by short-read sequencing (add references).
-|-
-|<span class="blue-text">EXAMPLE:</span> ''ALK''
-|<span class="blue-text">EXAMPLE:</span> ''ELM4::ALK''
-Other fusion partners include ''KIF5B, NPM1, STRN, TFG, TPM3, CLTC, KLC1''
-|<span class="blue-text">EXAMPLE:</span> Fusions result in constitutive activation of the ''ALK'' tyrosine kinase. The most common ''ALK'' fusion is ''EML4::ALK'', with breakpoints in intron 19 of ''ALK''. At the transcript level, a variable (5’) partner gene is fused to 3’ ''ALK'' at exon 20. Rarely, ''ALK'' fusions contain exon 19 due to breakpoints in intron 18.
-|<span class="blue-text">EXAMPLE:</span> N/A
-|<span class="blue-text">EXAMPLE:</span> Rare (Lung adenocarcinoma)
-|<span class="blue-text">EXAMPLE:</span> T
-|
-|<span class="blue-text">EXAMPLE:</span>
-Both balanced and unbalanced forms are observed by FISH (add references).
-|-
-|<span class="blue-text">EXAMPLE:</span> ''ABL1''
-|<span class="blue-text">EXAMPLE:</span> N/A
-|<span class="blue-text">EXAMPLE:</span> Intragenic deletion of exons 2–7 in ''EGFR'' removes the ligand-binding domain, resulting in a constitutively active tyrosine kinase with downstream activation of multiple oncogenic pathways.
-|<span class="blue-text">EXAMPLE:</span> N/A
-|<span class="blue-text">EXAMPLE:</span> Recurrent (IDH-wildtype Glioblastoma)
-|<span class="blue-text">EXAMPLE:</span> D, P, T
-|
-|
-|-
-|
-|
-|
-|
-|
-|
-|
-|
-|}
-==Individual Region Genomic Gain/Loss/LOH==
-Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene rearrangements. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Can refer to CGC workgroup tables as linked on the homepage if applicable. Please include references throughout the table. Do not delete the table.'') </span>
-{| class="wikitable"
-|'''Chr #'''
-|'''Gain / Loss / Amp / LOH'''
-|'''Minimal Region Cytoband and/or Approximate   Genomic Coordinates [Genome build GRCh38/hg38 if available]'''
-|'''Relevant Gene(s)'''
-|'''D / P / T (Diagnostic / Prognostic /   Therapeutic)'''
-|'''Established clinical significance per   guidelines? (Yes/No; source)'''
-|'''Clinical relevance / Notes'''
-|-
-|9p
-|Loss (deletion)
-|9p21.3 (~ chr9:21,900,000‑22,200,000)
-|CDKN2A, CDKN2B
-|P
-|No
-|High‐frequency  homozygous or biallelic deletion (~61% of cases; 45% biallelic) in PCGDTCL. (PMC)  Suggests aggressive biology, prognostic marker candidate.
-|-
-|18q
 |Loss
-|18q (arm level; no precise minimal region specified)
+|1p36.11
-|Putative tumour suppressors (unspecified)
+|ARID1A
 |P
 |No
-|Recurrent deletion ~22% in PCGDTCL cohort. (PMC)  May reflect genomic instability and poor outcome.
+|Deleted in ~28% of cases. Indicates epigenetic/chromatin modifier pathway involvement<ref name=":0" />
 |-
 |1q
@@ Line 282: / Line 104: @@
 |P / T
 |No
-|Amplification in ~33% of cases. (PMC)  Potential gene dosage effect; specific driver gene not yet defined.
+|Amplification in ~33% of cases. Potential gene dosage effect; specific driver gene not yet defined<ref name=":0" />
 |-
-|15q
+|2q
-|Gain (arm‐level)
+|Loss
-|15q (approx chr15:30,000,000‑102,000,000)
+|2q37.3
-|Multiple genes on 15q (unspecified)
+|PDCD1
 |P
 |No
-|Amplification in ~33% of cases. (PMC)  Likely reflects tumour evolution rather than diagnostic biomarker.
+|Deletion in ~22% of cases. Immune checkpoint gene loss; potential therapeutic‑escape mechanism<ref name=":0" />
 |-
 |7q
@@ Line 298: / Line 120: @@
 |P
 |No
-|Amplification in ~39% of cases. (PMC)  Suggests MAPK/other pathway involvement but specific gene not yet defined.
+|Amplification in ~39% of cases. Suggests MAPK/other pathway involvement but specific gene not yet defined.
 |-
-|Focal deletion: CDKN2A
+|9p
-|Loss (homozygous/biallelic)
+|Loss (deletion)
-|within 9p21.3, CDKN2A region
+|9p21.3 (~ chr9:21,900,000‑22,200,000)
-|CDKN2A
+|CDKN2A, CDKN2B
 |P
 |No
-|From GISTIC analysis: CDKN2A deletion in 61% of samples,  45% biallelic. (PMC)  Key focal region in PCGDTCL.
+|High‐frequency  homozygous or biallelic deletion (~61% of cases; 45% biallelic) in PCGDTCL. (PMC)  Suggests aggressive biology, prognostic marker candidate<ref name=":0" />
 |-
-|Focal deletion: ARID1A
+|10q
 |Loss
-|unspecified (del/trunc)
+|10q24.1
-|ARID1A
+|FAS
 |P
 |No
-|Deleted in ~28% of cases. (PMC)  Indicates epigenetic/chromatin modifier pathway involvement.
+|Deletion in ~22% of cases. Loss of apoptosis regulator; may contribute to immune‑escape<ref name=":0" />
 |-
-|Focal deletion: FAS
+|15q
-|Loss
+|Gain (arm‐level)
-|unspecified (biallelic)
+|15q (approx chr15:30,000,000‑102,000,000)
-|FAS
+|Multiple genes on 15q (unspecified)
 |P
 |No
-|Deletion in ~22% of cases. (PMC)  Loss of apoptosis regulator; may contribute to immune‑escape.
+|Amplification in ~33% of cases.  Likely reflects tumour evolution rather than diagnostic biomarker<ref name=":0" />
 |-
-|Focal deletion: PDCD1
+|18q
 |Loss
-|unspecified
+|18q (arm level; no precise minimal region specified)
-|PDCD1
+|Putative tumour suppressors (unspecified)
 |P
 |No
-|Deletion in ~22% of cases. (PMC)  Immune checkpoint gene loss; potential therapeutic‑escape mechanism.
+|Recurrent deletion ~22% in PCGDTCL cohort. May reflect genomic instability and poor outcome<ref name=":0" />
 |}
+==Characteristic Chromosomal or Other Global Mutational Patterns==
 {| class="wikitable sortable"
 |-
-!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
+!Chromosomal Pattern
+!Molecular Pathogenesis
+!Prevalence -
+Common >20%, Recurrent 5-20% or Rare <5% (Disease)
 !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
 !Established Clinical Significance Per Guidelines - Yes or No (Source)
 !Clinical Relevance Details/Other Notes
 |-
-|<span class="blue-text">EXAMPLE:</span>
+|'''Arm‑level somatic copy‑number variation  (SCNV)''' (average ~4 arm‑level events per case; median ~166.5  SCNVs per sample)<ref name=":0" />
-|<span class="blue-text">EXAMPLE:</span> Loss
-|<span class="blue-text">EXAMPLE:</span>
-chr7
-|<span class="blue-text">EXAMPLE:</span>
-Unknown
-|<span class="blue-text">EXAMPLE:</span> D, P
-|<span class="blue-text">EXAMPLE:</span> No
-|<span class="blue-text">EXAMPLE:</span>
-Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add references).
-|-
-|<span class="blue-text">EXAMPLE:</span>
-|<span class="blue-text">EXAMPLE:</span> Gain
-|<span class="blue-text">EXAMPLE:</span>
-chr8
-|<span class="blue-text">EXAMPLE:</span>
-Unknown
-|<span class="blue-text">EXAMPLE:</span> D, P
-|
-|<span class="blue-text">EXAMPLE:</span>
-Common recurrent secondary finding for t(8;21) (add references).
-|-
-|<span class="blue-text">EXAMPLE:</span>
-|<span class="blue-text">EXAMPLE:</span> Amp
-|<span class="blue-text">EXAMPLE:</span>
-q12; chr17:39,700,064-39,728,658 [hg38; 28.6 kb]
-|<span class="blue-text">EXAMPLE:</span>
-''ERBB2''
-|<span class="blue-text">EXAMPLE:</span> D, P, T
-|
-|<span class="blue-text">EXAMPLE:</span>
-Amplification of ''ERBB2'' is associated with HER2 overexpression in HER2 positive breast cancer (add references). Add criteria for how amplification is defined.
-|-
-|
-|
-|
-|
-|
-|
-|
-|}
-==Characteristic Chromosomal or Other Global Mutational Patterns==
-Put your text here and fill in the table <span style="color:#0070C0">(I''nstructions: Included in this category are alterations such as hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis; microsatellite instability; homologous recombination deficiency; mutational signature pattern; etc. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>
-{| class="wikitable"
-|'''Chromosomal/Global Pattern'''
-|'''Molecular Pathogenesis / Explanation'''
-|'''Prevalence (common>20%, recurrent 5-20% or rare <5%)'''
-|'''D / P / T'''
-|'''Established Clinical Significance per   Guidelines? (Yes/No; Source)'''
-|'''Clinical Relevance / Other Notes'''
-|-
-|'''Arm‑level somatic copy‑number variation  (SCNV)''' (average ~4 arm‑level events per case; median ~166.5  SCNVs per sample) (PMC)
 |Reflects genomic instability; multiple gains and losses  of whole chromosome arms likely contribute to oncogenesis and progression by  altering gene dosage of multiple oncogenes/tumour suppressors simultaneously.  (PMC)
 |'''Common''' (>20%) — nearly all  cases show multiple arm‑level events (median 4 per sample) (PMC)
@@ Line 400: / Line 173: @@
 |High genomic complexity may explain aggressive behaviour  and poor response to therapy. Could impact prognosis or treatment resistance  but not yet in guidelines.
 |-
-|'''High burden of somatic copy‑number variants  (SCNVs) relative to single‐nucleotide  variants (SNVs)''' (e.g., median ~166.5 SCNVs per sample) (PMC)
+|'''High burden of somatic copy‑number variants  (SCNVs) relative to single‐nucleotide  variants (SNVs)''' (e.g., median ~166.5 SCNVs per sample) <ref name=":0" />
 |Suggests that structural genomic alterations dominate the  mutational landscape, perhaps more so than classical hotspot SNVs, indicating  a biology driven by large‑scale genomic disruption rather than just point  mutations.
 |'''Common''' (>20%)
@@ Line 407: / Line 180: @@
 |Recognising this pattern may guide expectation of  complexity, but this is not currently used clinically for diagnosis or  treatment.
 |-
-|'''Distinct cell‑of‑origin signature: Vδ1 vs Vδ2  subtype''' (epidermal/dermal Vδ1 vs panniculitic Vδ2) (PMC)
+|'''Distinct cell‑of‑origin signature: Vδ1 vs Vδ2  subtype''' (epidermal/dermal Vδ1 vs panniculitic Vδ2) <ref name=":0" />
-|Different tissue compartments (epidermis/dermis vs  subcutaneous) correspond to distinct γδ T‑cell subsets (Vδ1 vs Vδ2). The cell‑of‑origin  influences mutational signatures (eg UV signature in Vδ1) and clinical  phenotype (Vδ2 more aggressive). (PMC)
+|Different tissue compartments (epidermis/dermis vs  subcutaneous) correspond to distinct γδ T‑cell subsets (Vδ1 vs Vδ2). The cell‑of‑origin  influences mutational signatures (eg UV signature in Vδ1) and clinical  phenotype (Vδ2 more aggressive)<ref name=":0" />
 |'''Recurrent''' (5‑20%) — this  pattern applies in a subset of cases defined by tissue involvement and TCR  subtype.
 |D / P
@@ Line 414: / Line 187: @@
 |This dichotomy may help stratify patients clinically (Vδ2  subtype worse prognosis) but is not currently part of formal diagnostic or  therapeutic guidelines.
 |-
-|'''Ultraviolet (UV) mutational signature in Vδ1  subtype''' (PMC)
+|'''Ultraviolet (UV) mutational signature in Vδ1  subtype''' <ref name=":0" />
 |The epidermal/dermal Vδ1 γδ T‑cell lymphomas exhibit a UV  signature in their mutation spectrum, likely reflecting skin localization and  UV exposure contributing to oncogenesis.
 |'''Recurrent''' (5‑20%) — seen in  Vδ1 cases but not all.
@@ Line 421: / Line 194: @@
 |Could suggest etiology and may influence prognosis;  though not yet used for therapy selection.
 |-
-|'''Frequent deletions of 9p21.3 (CDKN2A region)'''  (part of the SCNV pattern) (PMC)
+|'''Frequent deletions of 9p21.3 (CDKN2A region)'''  (part of the SCNV pattern) <ref name=":0" />
-|Loss of CDKN2A/p14^ARF leads to cell‑cycle deregulation,  loss of tumour suppressor control: a hallmark of many aggressive lymphomas.
+|Loss of CDKN2A/p14^ARF leads to cell‑cycle deregulation,  loss of tumour suppressor control: a hallmark of many aggressive lymphomas
-|'''Common''' (>20%) (approx 61% of  cases) (PubMed)
+|'''Common''' (>20%) (approx 61% of  cases)
 |P
 |No
 |Among the most prevalent genomic events in PCGDTCL —  potential prognostic marker though not yet guideline‑endorsed.
 |-
-|'''Multiple gains of oncogenic arms (e.g., 1q,  7q, 15q) and corresponding losses (eg 18q)''' (PMC)
+|'''Multiple gains of oncogenic arms (e.g., 1q,  7q, 15q) and corresponding losses (eg 18q)''' <ref name=":0" />
-|Gains may increase dosage of oncogenes; losses may reduce  tumour suppressor dosage—together contributing to malignant phenotype.
+|Gains may increase dosage of oncogenes; losses may reduce  tumour suppressor dosage—together contributing to malignant phenotype
-|'''Recurrent''' (5‑20%) for specific  arm‑level changes (e.g., 1q gain ~33%, 7q ~39%, 15q ~33%) (PubMed)
+|'''Recurrent''' (5‑20%) for specific  arm‑level changes (e.g., 1q gain ~33%, 7q ~39%, 15q ~33%)
 |P
 |No
 |These arm‑level events indicate complexity; may correlate  with poorer prognosis; not yet actionable in therapy.
 |-
-|'''TCR chain repertoire restriction / non‑random  Vγ or Vδ usage''' (eg Vγ3Vδ2 in panniculitic cases) (PMC)
+|'''TCR chain repertoire restriction / non‑random  Vγ or Vδ usage''' (eg Vγ3Vδ2 in panniculitic cases) <ref name=":0" />
-|Suggests antigen‑driven or tissue‐resident γδ T‑cell  proliferation; highlights non‑random selection of malignant clones.
+|Suggests antigen‑driven or tissue‐resident γδ T‑cell  proliferation; highlights non‑random selection of malignant clones
 |'''Recurrent''' (5‑20%) in defined  subtypes
 |D
@@ Line 442: / Line 215: @@
 |Might help refine subclassification of PCGDTCL; not  currently used in routine diagnostic algorithms.
 |}
+==Gene Mutations (SNV/INDEL)==
 {| class="wikitable sortable"
 |-
-!Chromosomal Pattern
+!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
-!Molecular Pathogenesis
-!Prevalence -
 Common >20%, Recurrent 5-20% or Rare <5% (Disease)
 !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
 !Established Clinical Significance Per Guidelines - Yes or No (Source)
 !Clinical Relevance Details/Other Notes
-|-
-|<span class="blue-text">EXAMPLE:</span>
-Co-deletion of 1p and 18q
-|<span class="blue-text">EXAMPLE:</span> See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
-|<span class="blue-text">EXAMPLE:</span> Common (Oligodendroglioma)
-|<span class="blue-text">EXAMPLE:</span> D, P
-|
-|
-|-
-|<span class="blue-text">EXAMPLE:</span>
-Microsatellite instability - hypermutated
-|
-|<span class="blue-text">EXAMPLE:</span> Common (Endometrial carcinoma)
-|<span class="blue-text">EXAMPLE:</span> P, T
-|
-|
-|-
-|
-|
-|
-|
-|
-|
-|}
-==Gene Mutations (SNV/INDEL)==
-Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent or common as well either disease defining and/or clinically significant. If a gene has multiple mechanisms depending on the type or site of the alteration, add multiple entries in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity. Details on clinical significance such as prognosis and other important information such as concomitant and mutually exclusive mutations can be provided in the notes section. Please include references throughout the table. Do not delete the table.'') </span>
-{| class="wikitable"
-|'''Gene'''
-|'''Genetic Alteration (SNV/INDEL) + presumed   mechanism'''
-|'''Tumour Suppressor / Oncogene / Other'''
-|'''Prevalence in PCGDTCL*'''
-|'''D / P / T (Diagnostic / Prognostic /   Therapeutic)'''
-|'''Established Clinical Significance per   Guidelines?'''
-|'''Clinical Relevance / Other Notes'''
 |-
 |'''STAT5B'''
 |Activating missense (e.g., p.N642H) → constitutive  downstream STAT5 signalling
 |Oncogene
-|Recurrent (~5‑20 %) — e.g., in the 2020 genomic study:  JAK/STAT mutations ~21 % of cases. (PMC)
+|Recurrent (~5‑20 %) — e.g., in the 2020 genomic study:  JAK/STAT mutations ~21 % of cases<ref name=":0" />
 |T / P: Therapeutic potential (JAK/STAT inhibition);  Prognostic implication (pathway addiction/resistance)
 |No
-|Mutant STAT5B (especially N642H) shown to induce T‑cell  neoplasia in models; in PCGDTCL JAK/STAT addiction shown clinically  (JCI 2025) (Ovid)
+|Mutant STAT5B (especially N642H) shown to induce T‑cell  neoplasia in models; in PCGDTCL JAK/STAT addiction shown clinically <ref name=":1">{{Cite journal|last=Küçük|first=Can|last2=Jiang|first2=Bei|last3=Hu|first3=Xiaozhou|last4=Zhang|first4=Wenyan|last5=Chan|first5=John K. C.|last6=Xiao|first6=Wenming|last7=Lack|first7=Nathan|last8=Alkan|first8=Can|last9=Williams|first9=John C.|date=2015-01-14|title=Activating mutations of STAT5B and STAT3 in lymphomas derived from γδ-T or NK cells|url=https://pubmed.ncbi.nlm.nih.gov/25586472|journal=Nature Communications|volume=6|pages=6025|doi=10.1038/ncomms7025|issn=2041-1723|pmc=7743911|pmid=25586472}}</ref><ref name=":3">{{Cite journal|last=Zhang|first=Yue|last2=Yescas|first2=Julia A.|last3=Tefft|first3=Kristy|last4=Ng|first4=Spencer|last5=Qiu|first5=Kevin|last6=Wang|first6=Erica B.|last7=Akhtar|first7=Shifa|last8=Walker|first8=Addie|last9=Welborn|first9=Macartney|date=2025-04-15|title=Addiction of primary cutaneous γδ T cell lymphomas to JAK/STAT signaling|url=https://pubmed.ncbi.nlm.nih.gov/40231467|journal=The Journal of Clinical Investigation|volume=135|issue=8|pages=e180417|doi=10.1172/JCI180417|issn=1558-8238|pmc=11996904|pmid=40231467}}</ref>
 |-
 |'''STAT3'''
 |Activating missense (SH2 domain) → constitutive STAT3  signalling
 |Oncogene
-|Rare (<5 %) to Recurrent (≈5‑10 %) (in NK/γδ‑T  lymphomas earlier) (PubMed)
+|Rare (<5 %) to Recurrent (≈5‑10 %) (in NK/γδ‑T  lymphomas earlier)
 |T / P
 |No
-|Less frequent than STAT5B in PCGDTCL; part of JAK/STAT  pathway involvement.
+|Less frequent than STAT5B in PCGDTCL; part of JAK/STAT pathway involvement<ref name=":0" /><ref name=":1" />
 |-
 |'''JAK3'''
 |Activating mutation (e.g., p.R657W) → JAK3 tyrosine  kinase activation
 |Oncogene
-|Rare (<5 %) (noted in the Daniels et al. cohort) (PMC)
+|Rare (<5 %) (noted in the Daniels et al. cohort)
 |T
 |No
-|Supports JAK/STAT involvement; one case report showed  response to JAK inhibition. (JCI)
+|Supports JAK/STAT involvement; one case report showed  response to JAK inhibition<ref name=":3" />
 |-
 |'''KRAS'''
 |Activating hotspot mutations (e.g., G12D, Q61H, D119N) →  RAS/MAPK activation
 |Oncogene
-|Recurrent (~5‑20 %) — “KRAS was the most frequently  mutated oncogene” in Daniels et al. (PMC)
+|Recurrent (~5‑20 %) — “KRAS was the most frequently  mutated oncogene” <ref name=":0" />
 |T / P
 |No
-|MAPK pathway appears relevant; patients with MAPK‑pathway  driver mutations had worse survival in the cohort. (PubMed)
+|MAPK pathway appears relevant; patients with MAPK‑pathway  driver mutations had worse survival in the cohort<ref name=":0" />
 |-
 |'''NRAS'''
 |Activating hotspot mutation → RAS/MAPK activation
 |Oncogene
-|Rare (<5 %) to Recurrent (~5‑10 %) (PMC)
+|Rare (<5 %) to Recurrent (~5‑10 %)
 |T / P
 |No
@@ Line 529: / Line 268: @@
 |Activating mutation → MAPK1 signalling activation
 |Oncogene
-|Rare (<5 %) (PMC)
+|Rare (<5 %)
 |T
 |No
-|Also in MAPK pathway; limited data in PCGDTCL.
+|Also in MAPK pathway; limited data in PCGDTCL<ref name=":0" /><ref name=":1" />
 |-
 |'''MYC'''
 |Activating missense mutation (e.g., p.P74L) → MYC pathway  up‑regulation
 |Oncogene
-|Rare (<5 %) (PMC)
+|Rare (<5 %)
 |P / T
 |No
-|MYC pathway involvement may contribute to the aggressive  phenotype; direct targeting not yet established.
+|MYC pathway involvement may contribute to the aggressive  phenotype; direct targeting not yet established<ref name=":0" />
 |-
 |'''MYCN'''
 |Activating mutation (e.g., p.G34R) → MYCN pathway  activation
 |Oncogene
-|Rare (<5 %) (PMC)
+|Rare (<5 %)
 |P / T
 |No
-|Highlights involvement of MYC‑family beyond MYC itself in  this disease.
+|Highlights involvement of MYC‑family beyond MYC itself in  this disease<ref name=":0" />
-|}
-{| class="wikitable sortable"
-|-
-!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
-Common >20%, Recurrent 5-20% or Rare <5% (Disease)
-!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
-!Established Clinical Significance Per Guidelines - Yes or No (Source)
-!Clinical Relevance Details/Other Notes
-|-
-|<span class="blue-text">EXAMPLE:</span>''EGFR''
-<br />
-|<span class="blue-text">EXAMPLE:</span> Exon 18-21 activating mutations
-|<span class="blue-text">EXAMPLE:</span> Oncogene
-|<span class="blue-text">EXAMPLE:</span> Common (lung cancer)
-|<span class="blue-text">EXAMPLE:</span> T
-|<span class="blue-text">EXAMPLE:</span> Yes (NCCN)
-|<span class="blue-text">EXAMPLE:</span> Exons 18, 19, and 21 mutations are targetable for therapy. Exon 20 T790M variants cause resistance to first generation TKI therapy and are targetable by second and third generation TKIs (add references).
-|-
-|<span class="blue-text">EXAMPLE:</span> ''TP53''; Variable LOF mutations
-<br />
-|<span class="blue-text">EXAMPLE:</span> Variable LOF mutations
-|<span class="blue-text">EXAMPLE:</span> Tumor Supressor Gene
-|<span class="blue-text">EXAMPLE:</span> Common (breast cancer)
-|<span class="blue-text">EXAMPLE:</span> P
-|
-|<span class="blue-text">EXAMPLE:</span> >90% are somatic; rare germline alterations associated with Li-Fraumeni syndrome (add reference). Denotes a poor prognosis in breast cancer.
-|-
-|<span class="blue-text">EXAMPLE:</span> ''BRAF''; Activating mutations
-|<span class="blue-text">EXAMPLE:</span> Activating mutations
-|<span class="blue-text">EXAMPLE:</span> Oncogene
-|<span class="blue-text">EXAMPLE:</span> Common (melanoma)
-|<span class="blue-text">EXAMPLE:</span> T
-|
-|
-|-
-|
-|
-|
-|
-|
-|
-|
 |}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
 ==Epigenomic Alterations==
-Put your text here
+N/A
 ==Genes and Main Pathways Involved==
-Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Please include references throughout the table. Do not delete the table.)''</span>
-{| class="wikitable"
+{| class="wikitable sortable"
-|'''Gene'''
+|-
-|'''Alteration / Pathway'''
+!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
-|'''Pathophysiologic Outcome'''
 |-
 |DNMT3A (DNA methyltransferase)
-|Loss‑of‑function mutations or deletions → reduced de novo  DNA methylation; “epigenetic writer” defect (DNA methylation pathway) (BioMed  Central)
+|Loss‑of‑function mutations or deletions → reduced de novo  DNA methylation; “epigenetic writer” defect (DNA methylation pathway)<ref name=":4">{{Cite journal|last=Zhang|first=Ping|last2=Zhang|first2=Mingzhi|date=2020-11-07|title=Epigenetic alterations and advancement of treatment in peripheral T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/33160401|journal=Clinical Epigenetics|volume=12|issue=1|pages=169|doi=10.1186/s13148-020-00962-x|issn=1868-7083|pmc=7648940|pmid=33160401}}</ref>
 |Deregulation of gene silencing; tumour suppressor genes  may remain unmethylated or aberrantly methylated → genomic instability,  aberrant T‑cell differentiation/activation
 |-
 |TET2 (methylcytosine dioxygenase)
-|Loss‑of‑function mutations → failure of DNA 5‑mC → 5‑hmC  demethylation (“epigenetic eraser” defect) (BioMed  Central)
+|Loss‑of‑function mutations → failure of DNA 5‑mC → 5‑hmC  demethylation (“epigenetic eraser” defect)<ref name=":4" />
 |Aberrant hypermethylation or demethylation patterns;  influences T‑cell development and malignant transformation (e.g., in T‑fh  lymphomas)
 |-
 |IDH2 (metabolic enzyme altering epigenome)
-|Gain‑of‑function mutation (e.g., R172) → produces 2‑hydroxyglutarate  → inhibits TET family → epigenetic dysregulation (BioMed  Central)
+|Gain‑of‑function mutation (e.g., R172) → produces 2‑hydroxyglutarate  → inhibits TET family → epigenetic dysregulation<ref name=":4" />
 |Oncometabolite‑driven methylation changes, impaired  differentiation, proliferation of malignant T cells
 |-
 |ARID1A (SWI/SNF chromatin‑remodeller)
-|Loss‑of‑function mutation/deletion → impaired nucleosome  remodelling, altered chromatin accessibility (“chromatin remodeller”) (PMC)
+|Loss‑of‑function mutation/deletion → impaired nucleosome  remodelling, altered chromatin accessibility (“chromatin remodeller”)<ref name=":4" />
 |Reduced tumour‑suppressor gene expression due to  chromatin compaction; may influence immune microenvironment and genomic  instability
 |-
 |KMT2D / KMT2A (H3K4 methyltransferases)
-|Loss‑of‑function mutations (“histone‑writer” defect) →  decreased H3K4 methylation (activating mark) (PMC)
+|Loss‑of‑function mutations (“histone‑writer” defect) →  decreased H3K4 methylation (activating mark)<ref name=":5">{{Cite journal|last=Ahmed|first=Nada|last2=Feldman|first2=Andrew L.|date=2020-02|title=Targeting epigenetic regulators in the treatment of T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/31903826|journal=Expert Review of Hematology|volume=13|issue=2|pages=127–139|doi=10.1080/17474086.2020.1711732|issn=1747-4094|pmc=7110907|pmid=31903826}}</ref>
 |Impaired activation of gene expression programs  (differentiation, apoptosis) → contributes to malignant transformation
 |-
 |KDM6A (H3K27 demethylase)
-|Loss‑of‑function → accumulation of H3K27me3 (repressive  histone mark) (“histone‑eraser” defect) (PMC)
+|Loss‑of‑function → accumulation of H3K27me3 (repressive  histone mark) (“histone‑eraser” defect)<ref name=":5" />
 |Further chromatin repression of tumour‑suppressor genes;  may enhance survival of malignant T cells
 |-
 |EZH2 (PRC2 complex methyltransferase)
-|Overexpression/gain of function → increased H3K27me3  (“histone‑writer” overactivity) (PMC)
+|Overexpression/gain of function → increased H3K27me3  (“histone‑writer” overactivity) <ref name=":4" />
 |Enhanced silencing of differentiation/apoptosis genes;  contributes to aggressive lymphoma phenotypes
 |-
 |CREBBP / EP300 (histone acetyl‑transferases)
-|Loss‑of‑function mutations (“histone‑writer” defect) →  reduced histone acetylation and gene activation (PMC)
+|Loss‑of‑function mutations (“histone‑writer” defect) →  reduced histone acetylation and gene activation<ref name=":5" />
 |Diminished transcriptional activation of tumour‑suppressor/immune  genes; may drive malignant progression
 |-
 |DNA methylation of specific tumour‑suppressor loci (e.g.,  CDKN2A promoter; FAS promoter)
-|Hypermethylation of promoter CpG islands → silencing of  tumour suppressor / apoptosis‑initiator genes (PMC)
+|Hypermethylation of promoter CpG islands → silencing of tumor suppressor / apoptosis‑initiator genes<ref>{{Cite journal|last=Hara|first=Natsumi|last2=Sawada|first2=Yu|date=2022-03-24|title=Epigenetics of Cutaneous T-Cell Lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/35408897|journal=International Journal of Molecular Sciences|volume=23|issue=7|pages=3538|doi=10.3390/ijms23073538|issn=1422-0067|pmc=8998216|pmid=35408897}}</ref>
 |Loss of cell‑cycle control or apoptosis leads to  malignant T‑cell survival/proliferation
-|}
-{| class="wikitable sortable"
-|-
-!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
-|-
-|<span class="blue-text">EXAMPLE:</span> ''BRAF'' and ''MAP2K1''; Activating mutations
-|<span class="blue-text">EXAMPLE:</span> MAPK signaling
-|<span class="blue-text">EXAMPLE:</span> Increased cell growth and proliferation
-|-
-|<span class="blue-text">EXAMPLE:</span> ''CDKN2A''; Inactivating mutations
-|<span class="blue-text">EXAMPLE:</span> Cell cycle regulation
-|<span class="blue-text">EXAMPLE:</span> Unregulated cell division
-|-
-|<span class="blue-text">EXAMPLE:</span> ''KMT2C'' and ''ARID1A''; Inactivating mutations
-|<span class="blue-text">EXAMPLE:</span> Histone modification, chromatin remodeling
-|<span class="blue-text">EXAMPLE:</span> Abnormal gene expression program
-|-
-|
-|
-|
 |}
 ==Genetic Diagnostic Testing Methods==
-Put your text here <span style="color:#0070C0">(''Instructions: Include recommended testing type(s) to identify the clinically significant genetic alterations.'')</span>
 {| class="wikitable"
 |'''Method'''
@@ Line 726: / Line 401: @@
 ==Familial Forms==
-Put your text here <span style="color:#0070C0">(''Instructions: Include associated hereditary conditions/syndromes that cause this entity or are caused by this entity.'') </span>
 There are currently '''no well-established familial or hereditary forms''' described in the literature.
 ==Additional Information==
-Put your text here
+N/A
 ==Links==
-Put a link here or anywhere appropriate in this page <span style="color:#0070C0">(''Instructions: Highlight the text to which you want to add a link in this section or elsewhere, select the "Link" icon at the top of the wiki page, and search the name of the internal page to which you want to link this text, or enter an external internet address by including the "<nowiki>http://www</nowiki>." portion.'')</span>
+N/A
 ==References==
-(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span>
+<references />
+==Notes==
+<nowiki>*</nowiki>''Citation of this Page'': Azimpouran M, Kitahara S. “Primary cutaneous gamma/delta T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_gamma/delta_T-cell_lymphoma</nowiki>.
-==Notes==
 <nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the [[Leadership|''<u>Associate Editor</u>'']] or other CCGA representative.  When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.
-Prior Author(s):
+Prior Author(s): N/A
-<nowiki>*</nowiki>''Citation of this Page'': “Primary cutaneous gamma/delta T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_gamma/delta_T-cell_lymphoma</nowiki>.
 [[Category:HAEM5]]
 [[Category:DISEASE]]
 [[Category:Diseases P]]

HAEM5:Primary cutaneous gamma/delta T-cell lymphoma: Difference between revisions

Latest revision as of 22:48, 6 January 2026

Primary Author(s)*

WHO Classification of Disease

Related Terminology

Gene Rearrangements

Individual Region Genomic Gain/Loss/LOH

Characteristic Chromosomal or Other Global Mutational Patterns

Gene Mutations (SNV/INDEL)

Epigenomic Alterations

Genes and Main Pathways Involved

Genetic Diagnostic Testing Methods

Familial Forms

Additional Information

Links

References

Notes

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