STBT5:Solitary fibrous tumour: Difference between revisions - Compendium of Cancer Genome Aberrations

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|Subtype(s)

|N/A

|}

|}

~~==Morphologic Features==~~

Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.

~~==Immunophenotype<ref name=":5">Goldblum, John R, et al. ''Enzinger & Weiss’s Soft Tissue Tumors''. 7th ed., Philadelphia, PA, Elsevier, 2020, pp. 1133–1147.</ref>==~~

~~Put your text here and fill in the table (''Instruction: Can include references in the table. Do not delete table.'')~~ </~~span~~>

~~{| class="wikitable sortable"~~

|-

~~!Finding!!Marker~~

|-

~~|Positive||CD34~~

|-

~~|Positive||STAT6 (nuclear)~~

|-

~~|Positive||BCL2 (30%)~~

|-

~~|Positive||CD99 (70%)~~

|-

~~|Positive~~

~~|EMA (30%)~~

|-

~~|Positive~~

~~|Actin (20%)~~

|-

~~|Negative~~

~~|S100~~

|-

~~|Negative~~

~~|Desmin~~

|-

~~|Negative~~

~~|Cytokeratins~~

|}

==Gene Rearrangements==

Put your text here and fill in the table (''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.'')

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This disease is defined/characterized as detailed below:

* Solitary fibrous tumor (SFT) is a mesenchymal spindle cell neoplasm that can develop at any site in the body, including soft tissue, visceral organs, bone, etc. It was first described by Klemperer and Rabin<ref>{{Cite journal|last=Klemperer|first=Paul|last2=Rabin|first2=Coleman B.|date=1992-01|title=Primary Neoplasms of the pleura. A report of five cases|url=https://onlinelibrary.wiley.com/doi/10.1002/ajim.4700220103|journal=American Journal of Industrial Medicine|language=en|volume=22|issue=1|pages=4–31|doi=10.1002/ajim.4700220103|issn=0271-3586}}</ref> in 1992 as a tumor of the pleura. However, since then this entity has been increasingly described from extrapleural sites. A morphologic clue to the diagnosis is the distinctive branching “staghorn” vessels. SFT is characterized by ''NAB2::STAT6'' fusion resulting from a small paracentric inversion at chromosome 12q13.

*Solitary fibrous tumor (SFT) is a mesenchymal spindle cell neoplasm that can develop at any site in the body, including soft tissue, visceral organs, bone, etc. It was first described by Klemperer and Rabin<ref>{{Cite journal|last=Klemperer|first=Paul|last2=Rabin|first2=Coleman B.|date=1992-01|title=Primary Neoplasms of the pleura. A report of five cases|url=https://onlinelibrary.wiley.com/doi/10.1002/ajim.4700220103|journal=American Journal of Industrial Medicine|language=en|volume=22|issue=1|pages=4–31|doi=10.1002/ajim.4700220103|issn=0271-3586}}</ref> in 1992 as a tumor of the pleura. However, since then this entity has been increasingly described from extrapleural sites. A morphologic clue to the diagnosis is the distinctive branching “staghorn” vessels. SFT is characterized by ''NAB2::STAT6'' fusion resulting from a small paracentric inversion at chromosome 12q13.

The epidemiology/prevalence of this disease is detailed below:

* SFT occurs most commonly in adults with no gender predilection. Incidence of the tumor is highest in the age group of 40-70 years.

*SFT occurs most commonly in adults with no gender predilection. Incidence of the tumor is highest in the age group of 40-70 years.

The clinical features of this disease are detailed below:

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The sites of involvement of this disease are detailed below:

* SFTs may occur at any site of the body. Involvement of head and neck, deep soft tissues, abdominal cavity, retroperitoneum, pelvis, bone, and visceral organs have been reported.

*SFTs may occur at any site of the body. Involvement of head and neck, deep soft tissues, abdominal cavity, retroperitoneum, pelvis, bone, and visceral organs have been reported.

The morphologic features of this disease are detailed below:

* Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.

*Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.

The immunophenotype of this disease is detailed below:

Positive - CD34, STAT6 (nuclear), BCL2 (30%), CD99 (70%), EMA (30%) and Actin (20%)<ref name=":5" />

Positive - CD34, STAT6 (nuclear), BCL2 (30%), CD99 (70%), EMA (30%) and Actin (20%)<ref name=":5">Goldblum, John R, et al. ''Enzinger & Weiss’s Soft Tissue Tumors''. 7th ed., Philadelphia, PA, Elsevier, 2020, pp. 1133–1147.</ref>

Negative - S100, Desmin and Cytokeratins<ref name=":5" />

@@ Line 28: / Line 28: @@
 |Subtype(s)
 |N/A
-|}
+|}<br />
-==Morphologic Features==
-Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.
-==Immunophenotype<ref name=":5">Goldblum, John R, et al. ''Enzinger & Weiss’s Soft Tissue Tumors''. 7th ed., Philadelphia, PA, Elsevier, 2020, pp. 1133–1147.</ref>==
-Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
-{| class="wikitable sortable"
-|-
-!Finding!!Marker
-|-
-|Positive||CD34
-|-
-|Positive||STAT6 (nuclear)
-|-
-|Positive||BCL2 (30%)
-|-
-|Positive||CD99 (70%)
-|-
-|Positive
-|EMA (30%)
-|-
-|Positive
-|Actin (20%)
-|-
-|Negative
-|S100
-|-
-|Negative
-|Desmin
-|-
-|Negative
-|Cytokeratins
-|}
 ==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>
@@ Line 416: / Line 385: @@
 This disease is <u>defined/characterized</u> as detailed below:
-* Solitary fibrous tumor (SFT) is a mesenchymal spindle cell neoplasm that can develop at any site in the body, including soft tissue, visceral organs, bone, etc.  It was first described by Klemperer and Rabin<ref>{{Cite journal|last=Klemperer|first=Paul|last2=Rabin|first2=Coleman B.|date=1992-01|title=Primary Neoplasms of the pleura. A report of five cases|url=https://onlinelibrary.wiley.com/doi/10.1002/ajim.4700220103|journal=American Journal of Industrial Medicine|language=en|volume=22|issue=1|pages=4–31|doi=10.1002/ajim.4700220103|issn=0271-3586}}</ref> in 1992 as a tumor of the pleura. However, since then this entity has been increasingly described from extrapleural sites. A morphologic clue to the diagnosis is the distinctive branching “staghorn” vessels. SFT is characterized by ''NAB2::STAT6'' fusion resulting from a small paracentric inversion at chromosome 12q13.
+*Solitary fibrous tumor (SFT) is a mesenchymal spindle cell neoplasm that can develop at any site in the body, including soft tissue, visceral organs, bone, etc.  It was first described by Klemperer and Rabin<ref>{{Cite journal|last=Klemperer|first=Paul|last2=Rabin|first2=Coleman B.|date=1992-01|title=Primary Neoplasms of the pleura. A report of five cases|url=https://onlinelibrary.wiley.com/doi/10.1002/ajim.4700220103|journal=American Journal of Industrial Medicine|language=en|volume=22|issue=1|pages=4–31|doi=10.1002/ajim.4700220103|issn=0271-3586}}</ref> in 1992 as a tumor of the pleura. However, since then this entity has been increasingly described from extrapleural sites. A morphologic clue to the diagnosis is the distinctive branching “staghorn” vessels. SFT is characterized by ''NAB2::STAT6'' fusion resulting from a small paracentric inversion at chromosome 12q13.
 The <u>epidemiology/prevalence</u> of this disease is detailed below:
-* SFT occurs most commonly in adults with no gender predilection. Incidence of the tumor is highest in the age group of 40-70 years.
+*SFT occurs most commonly in adults with no gender predilection. Incidence of the tumor is highest in the age group of 40-70 years.
 The <u>clinical features</u> of this disease are detailed below:
@@ Line 430: / Line 399: @@
 The <u>sites of involvement</u> of this disease are detailed below:
-* SFTs may occur at any site of the body. Involvement of head and neck, deep soft tissues, abdominal cavity, retroperitoneum, pelvis, bone, and visceral organs have been reported.
+*SFTs may occur at any site of the body. Involvement of head and neck, deep soft tissues, abdominal cavity, retroperitoneum, pelvis, bone, and visceral organs have been reported.
 The <u>morphologic features</u> of this disease are detailed below:
-* Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.
+*Morphologically, biopsy or resection specimens will show a bland spindle cell proliferation arranged haphazardly in a background of collagenous stroma. Cells contain ovoid nuclei within eosinophilic cytoplasm with indistinct cell borders. Tumors can vary in cellularity and stromal collagen. On low power, branching, “staghorn” shaped vessels can often be appreciated. Mitotic counts are usually low. Myxoid and lipomatous change have been described. SFT with adipocytic component are referred to as fat-forming (lipomatous) SFTs. There is also a variant known as giant cell rich SFT, which has the classic patternless spindle cell proliferation admixed with multinucleated giant cells. Dedifferentiated SFTs will show conventional SFT transitioning to a high-grade pleomorphic variant. Heterologous elements may be present. Standard immunophenotypic expression of CD34 and STAT6 is often lost in the dedifferentiated component.
 The <u>immunophenotype</u> of this disease is detailed below:
-Positive - CD34, STAT6 (nuclear), BCL2 (30%), CD99 (70%), EMA (30%) and Actin (20%)<ref name=":5" />
+Positive - CD34, STAT6 (nuclear), BCL2 (30%), CD99 (70%), EMA (30%) and Actin (20%)<ref name=":5">Goldblum, John R, et al. ''Enzinger & Weiss’s Soft Tissue Tumors''. 7th ed., Philadelphia, PA, Elsevier, 2020, pp. 1133–1147.</ref>
 Negative - S100, Desmin and Cytokeratins<ref name=":5" />