Compendium of Cancer Genome Aberrations

Anaplastic Large Cell Lymphoma (ALK+/ALK−): Difference between revisions

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==Primary Author(s)*==
==Primary Author(s)*==
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==Cancer Category/Type==
==WHO Classification of Disease==
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{| class="wikitable"
==Cancer Sub-Classification / Subtype==
|+
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!Structure
!Disease
|-
|Book
|WHO Classification of Tumours Central Nervous System Tumours (5th ed.)
|-
|Category
|Lymphomas
|-
|Family
|Miscellaneous rare lymphomas in CNS
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|Type
|Anaplastic Large Cell Lymphoma (ALK+/ALK-)
|-
|Subtype
|None
|}
==Definition / Description of Disease==
==Definition / Description of Disease==
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Anaplastic large cell lymphoma (ALCL) is a distinctive CD30-positive peripheral T-cell lymphoma that is rare in the CNS and is separated into two distinct types: ALK-positive (ALK+ ALCL) and ALK-negative (ALK− ALCL).
==Synonyms / Terminology==
==Synonyms / Terminology==
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None
==Epidemiology / Prevalence==
==Epidemiology / Prevalence==
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ALK+ ALCL occurs from early childhood to young adulthood with a male preponderance.
 
ALK− ALCL affects adults (median age: 65 years), also with a male preponderance.
==Clinical Features==
==Clinical Features==
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{| class="wikitable"
{| class="wikitable"
|'''Signs and Symptoms'''
|'''Signs and Symptoms'''
|EXAMPLE Asymptomatic (incidental finding on complete blood counts)
|Headache, seizures, nausea, fever, or a combination
EXAMPLE B-symptoms (weight loss, fever, night sweats)
 
EXAMPLE Fatigue
 
EXAMPLE Lymphadenopathy (uncommon)
|-
|-
|'''Laboratory Findings'''
|'''Laboratory Findings'''
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|}
|}
==Sites of Involvement==
==Sites of Involvement==
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ALK+ ALCL occurs as single or multiple supratentorial parenchymal lesions with or without infratentorial involvement, and rarely with spinal cord involvement. Extension to involve the meninges and (rarely) the skull can occur.
 
ALK− ALCL occurs as single or multiple lesions, usually supratentorial
==Morphologic Features==
==Morphologic Features==
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ALK+ ALCL shows a diffuse proliferation of large atypical cells with abundant cytoplasm, including hallmark cells with bean-shaped nuclei and an eosinophilic paranuclear area
 
The cerebrospinal fluid may be involved. The large atypical neoplastic cells may have cytoplasmic azurophilic granules
==Immunophenotype==
==Immunophenotype==
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!Finding!!Marker
!Finding!!Marker
|-
|-
|Positive (universal)||EXAMPLE CD1
|Positive (universal)||CD30+, ALK+, and EMA+, may express one or more T-cell antigens
|-
|-
|Positive (subset)||EXAMPLE CD2
|Positive (subset)||EXAMPLE CD2
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|Negative (subset)||EXAMPLE CD4
|Negative (subset)||EXAMPLE CD4
|}
|}
==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions) ALK+ ALCL==
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{| class="wikitable sortable"
{| class="wikitable sortable"
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!Notes
!Notes
|-
|-
|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)
|t(2;5)(p23;q35)||''NPM1''::''ALK'' fusion
EXAMPLE 30% (add reference)
|5' NPM1::3' ALK on der(5). constitutive activation of the catalytic domain of ALK.  Kinase function activated by oligomerization of NPM1::ALK mediated by the NPM1 portion||EXAMPLE 30 to 50% of ALCL
(COSMIC)
(add reference)
|Yes
|Yes
|Yes
|No
|Yes
|Yes
|EXAMPLE
|EXAMPLE
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).
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).
Localized in both cytoplasm and nucleus.
In translocations other than the t(2;5), i.e. in t(2;Var) involving various partners and ALK, the fusion protein has a cytoplasmic localization; they are therefore called "cytoplasm only" ALK+ ALCL.
|-
|t(X;2)(q11;p23)
|
|5'MSN:: 3'ALK
|very rare, one case reported
|
|
|
|For the t(X;2) translocation, localization is restricted to the membrane.
|-
|t(1;2)(q25;p23)
|
|5'TPM3::3'ALK
|rare, four cases reported
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|
|
|TPM3::ALK is constitutively activated
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|inv(2)(p23q35)
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|5'ATIC::3'ALK
|rare
|
|
|
|ider(2)(q10)inv(2) has been found in some cases, carrying 2 additional copies of the ATIC::ALK hybrid gene; frequent complex karyotypes
|-
|t(2;3)(p23;q21)
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|5'TFG::3-ALK
|very rare, two cases reported
|
|
|
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|-
|t(2;17)(p23;q23)
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|5'CLTC::3'ALK
|very rare, one case reported
|
|
|
|
|-
|t(2;19)(p23; p13.1)
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|5'TPM4::3'ALK
|very rare, one case reported
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|
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|-
|t(2;22)(p23;q11.2)
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|5'CLTCL1::3'ALK
|very rare, one or two cases
|
|
|
|the localization is restricted to granules (vesicles) in the cytoplasm
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|}
==Individual Region Genomic Gain/Loss/LOH==
==Individual Region Genomic Gain/Loss/LOH==
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!Notes
!Notes
|-
|-
|EXAMPLE
|Complex Karyotype
Co-deletion of 1p and 18q
|
|Yes
|
|No
|
|No
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|-
| +7 found in 20% of cases
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|
|
|EXAMPLE:
|EXAMPLE:
See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
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|<nowiki>+9 in 5 to 10% of cases.</nowiki>
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|-
|<nowiki>+X in 5 to 10% of cases.</nowiki>
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==Gene Mutations (SNV/INDEL)==
==Gene Mutations (SNV/INDEL)==
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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==
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FISH w/ ALK BA probe
==Familial Forms==
==Familial Forms==
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