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| '''Soft tissue lesions''' strike fear in many pathologists as they are uncommon and may be difficult to diagnose. | | '''Soft tissue lesions''' strike fear in many pathologists as they are uncommon and may be difficult to diagnose. Malignant soft tissue lesions, i.e. [[cancer|cancerous]] soft tissue lesions, are usually '''sarcomas'''. Sarcomas are malignancies derived from mesenchymal tissue. |
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| =Introduction= | | =Introduction= |
| ==WHO classification of soft tissue lesions/tumours== | | ==WHO classification of soft tissue lesions/tumours== |
| ===Morphologic grouping<ref name=Ref_WMSP601-3>{{Ref WMSP|601-3}}</ref>=== | | ===Morphologic grouping=== |
| | These include:<ref name=Ref_WMSP601-3>{{Ref WMSP|601-3}}</ref> |
| #[[Adipocytic tumours]]. | | #[[Adipocytic tumours]]. |
| #[[Fibroblastic/myofibroblastic tumours]]. | | #[[Fibroblastic/myofibroblastic tumours]]. |
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| #[[Soft tissue lesions#Tumours of uncertain differentiation|Tumours of uncertain differentiation]]. | | #[[Soft tissue lesions#Tumours of uncertain differentiation|Tumours of uncertain differentiation]]. |
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| ===Biologic potential grouping<ref>{{Ref WMSP|598-604}}</ref>=== | | ===Biologic potential grouping=== |
| | These include:<ref>{{Ref WMSP|598-604}}</ref> |
| #Benign. | | #Benign. |
| #Intermediate (locally aggressive). | | #Intermediate (locally aggressive). |
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| *Once upon a time almost everything was called ''malignant fibrous histiocytoma''; thus, it is listed as a common entity in some publications. | | *Once upon a time almost everything was called ''malignant fibrous histiocytoma''; thus, it is listed as a common entity in some publications. |
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| ===Most common:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url= http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref>===
| | Most common:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url= http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref> |
| *Liposarcoma. | | *Liposarcoma. |
| *Leiomyosarcoma. | | *Leiomyosarcoma. |
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| ==Morphohistologic patterns== | | ==Morphohistologic patterns== |
| {{Main|Basics#Morphologic_patterns}} | | {{Main|Morphologic patterns}} |
| {| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | | {| class="wikitable sortable" style="margin-left:auto;margin-right:auto" |
| ! Name | | ! Name |
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| ! DDx | | ! DDx |
| ! Image(s) | | ! Image(s) |
| ! Other
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| |- | | |- |
| | Storiform, [[AKA]] patternless pattern<ref name=pmid9704618>{{cite journal |author=Mangano WE, Cagle PT, Churg A, Vollmer RT, Roggli VL |title=The diagnosis of desmoplastic malignant mesothelioma and its distinction from fibrous pleurisy: a histologic and immunohistochemical analysis of 31 cases including p53 immunostaining |journal=Am. J. Clin. Pathol. |volume=110 |issue=2 |pages=191–9 |year=1998 |month=August |pmid=9704618 |doi= |url=}}</ref> | | | Storiform, [[AKA]] patternless pattern<ref name=pmid9704618>{{cite journal |author=Mangano WE, Cagle PT, Churg A, Vollmer RT, Roggli VL |title=The diagnosis of desmoplastic malignant mesothelioma and its distinction from fibrous pleurisy: a histologic and immunohistochemical analysis of 31 cases including p53 immunostaining |journal=Am. J. Clin. Pathol. |volume=110 |issue=2 |pages=191–9 |year=1998 |month=August |pmid=9704618 |doi= |url=}}</ref> |
| | whorled, cartwheel-like arrangement | | | whorled, cartwheel-like arrangement |
| | [[pleomorphic undifferentiated sarcoma]], [[solitary fibrous tumour]], [[dermatofibrosarcoma protuberans]], [[dermatofibroma]]<ref name=pmid224569>{{cite journal |author=Meister P, Höhne N, Konrad E, Eder M |title=Fibrous histiocytoma: an analysis of the storiform pattern |journal=Virchows Arch A Pathol Anat Histol |volume=383 |issue=1 |pages=31–41 |year=1979 |month=July |pmid=224569 |doi= |url=}}</ref> | | | [[pleomorphic undifferentiated sarcoma]], [[solitary fibrous tumour]], [[dermatofibrosarcoma protuberans]], [[dermatofibroma]]<ref name=pmid224569>{{cite journal |author=Meister P, Höhne N, Konrad E, Eder M |title=Fibrous histiocytoma: an analysis of the storiform pattern |journal=Virchows Arch A Pathol Anat Histol |volume=383 |issue=1 |pages=31–41 |year=1979 |month=July |pmid=224569 |doi= |url=}}</ref> |
| | [http://commons.wikimedia.org/w/index.php?title=File:Storiform_pattern_-_intermed_mag.jpg intermed. mag.], [http://commons.wikimedia.org/wiki/File:Storiform_pattern_-_very_high_mag.jpg very high mag.] | | | [[Image:Storiform_pattern_-_intermed_mag.jpg |thumb|center|150px| Patternless pattern. (WC)]] |
| | other ?
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| |- | | |- |
| | Herring bone | | | Herring bone |
| | like herring bone (technique) for climbing a hill in cross country skiing; books on a shelf, where they have partially fallen over -- on the one shelf to the left and the one below to the right | | | like herring bone (technique) for climbing a hill in cross country skiing; books on a shelf, where they have partially fallen over -- on the one shelf to the left and the one below to the right |
| | [[fibrosarcoma]], [[synovial sarcoma]], [[MPNST]] | | | [[fibrosarcoma]], [[synovial sarcoma]], [[MPNST]] |
| | [http://commons.wikimedia.org/wiki/File:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg intermed. mag.], [http://commons.wikimedia.org/wiki/File:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg high mag.] | | | [[Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg |thumb|center|150px |Herring bone. (WC)]] |
| | other ?
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| |- | | |- |
| | Fasicular | | | Fascicular |
| | the long axis of the (spindle) cells are perpendicular to one another in adjacent bundles of cells | | | the long axis of the (spindle) cells are perpendicular to one another in adjacent bundles of cells |
| | [[leiomyoma]], [[leiomyosarcoma]] | | | [[leiomyoma]], [[leiomyosarcoma]] |
| | [http://commons.wikimedia.org/wiki/File:Cutaneous_leiomyosarcoma_-_a_-_intermed_mag.jpg intermed. mag.], [http://commons.wikimedia.org/wiki/File:Cutaneous_leiomyosarcoma_-_high_mag.jpg high mag.] | | | [[Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg |thumb|center|150px| Fascicular pattern. (WC)]] |
| | other ?
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| |- | | |- |
| | Biphasic | | | Biphasic |
| | nests of cells and stroma | | | nests of cells and stroma |
| | [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]] | | | [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]] |
| | [http://commons.wikimedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg DSRCT - high mag. (WC)] | | | [[Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg|thumb|center|150px| DSRCT. (WC)]] |
| | other ?
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| |- <!-- | | |- <!-- |
| | name ? | | | name ? |
| | description ? | | | description ? |
| | DDx ? | | | DDx ? |
| | image ? | | | image ? --> |
| | other ? -->
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| |} | | |} |
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| ===French system=== | | ===French system=== |
| Overview:<ref name=pmid8996162/><ref name=uscap_stp>URL: [http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf]. Accessed on: 12 April 2011.</ref> | | *Formally known as the grading system from the ''French Federation of Cancer Centres Sarcoma Group'' (FNCLCC). |
| | |
| | ====Overview==== |
| | Components - overview:<ref name=pmid8996162/><ref name=uscap_stp>URL: [http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf]. Accessed on: 12 April 2011.</ref> |
| #Differentiation (score 1-3). | | #Differentiation (score 1-3). |
| #*De facto, this is mostly the ''histologic type''. | | #*De facto, this is mostly the ''histologic type''. |
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| *Well-differentiated liposarcoma = 1. | | *Well-differentiated liposarcoma = 1. |
| *Myxoid liposarcoma = 2. | | *Myxoid liposarcoma = 2. |
| *Conventional liposarcoma = 2. | | *Conventional [[liposarcoma]] = 2. |
| *Fibrosarcoma = 2. | | *Fibrosarcoma = 2. |
| *Myxofibrosarcoma =2. | | *[[Myxofibrosarcoma]] =2. |
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| A group of tumours is not graded:<ref name=uscap_stp/> | | A group of tumours is not graded:<ref name=uscap_stp/> |
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| *[[Alveolar soft part sarcoma]]. | | *[[Alveolar soft part sarcoma]]. |
| *[[Clear cell sarcoma]]. | | *[[Clear cell sarcoma]]. |
| *Extraskeletal myxoid chondrosarcoma. | | *[[Extraskeletal myxoid chondrosarcoma]]. |
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| =====Mitotic rate===== | | =====Mitotic rate===== |
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| *Cellularity (cells/matrix) > 50%. | | *Cellularity (cells/matrix) > 50%. |
| *Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly. | | *Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly. |
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| | ==Stage== |
| | {{Main|Cancer staging systems}} |
| | ===Lymph node metastases in sarcomas=== |
| | {{Main|Lymph node metastasis}} |
| | *[[Lymph node]] (LN) spread is uncommon in sarcomas; [[lymph node metastases]] are seen in <3% of cases.<ref name=pmid8424704>{{Cite journal | last1 = Fong | first1 = Y. | last2 = Coit | first2 = DG. | last3 = Woodruff | first3 = JM. | last4 = Brennan | first4 = MF. | title = Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. | journal = Ann Surg | volume = 217 | issue = 1 | pages = 72-7 | month = Jan | year = 1993 | doi = | PMID = 8424704 | PMC = 1242736}}</ref> |
| | **Many sarcomas are reported in LNs. |
| | ***According to the [[CAP checklist]] for soft tissue<ref>URL: [http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SoftTissue_11protocol.pdf]. Accessed on: 28 March 2012.</ref> the most common are: [[epithelioid sarcoma]] and [[clear cell sarcoma]]. |
| | ***According to Fong ''et al.''<ref name=pmid8424704/> the most commonly is: angiosarcoma. |
| | |
| | Sarcomas more likely to be found in the lymph nodes - mnemonic ''RACE For MS'':<ref>URL: [http://www.aippg.net/forum/f21/surgery-mnemonics-79897/ http://www.aippg.net/forum/f21/surgery-mnemonics-79897/]. Accessed on: 23 March 2012.</ref> |
| | *[[rhabdomyosarcoma|'''R'''habdomyosarcoma]] |
| | *[[Angiosarcoma|'''A'''ngiosarcoma]]. |
| | *[[clear cell sarcoma|'''C'''lear cell sarcoma]]. |
| | *[[epithelioid sarcoma|'''E'''pitheliod sarcoma]]. |
| | *[[fibrosarcoma|'''F'''ibrosarcoma]]. |
| | *[[pleomorphic undifferentiated sarcoma|'''M'''alignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma)]]. |
| | *[[synovial sarcoma|'''S'''ynovial cell sarcoma]]. |
| | |
| | ==DDx by history/site== |
| | ===Retroperiteum=== |
| | #[[Liposarcoma]]. |
| | #[[Undifferentiated pleomorphic sarcoma]]. |
| | #[[Leiomyosarcoma]]. |
| | #[[MPNST]]. |
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| | Note: |
| | [[Synovial sarcoma]] and [[fibrosarcoma]] are very rare in the retroperitoneum. |
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| | ===Young person - extremity sarcoma=== |
| | #[[Epithelioid sarcoma]]. |
| | #[[Synovial sarcoma]]. |
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| | ==Gross characteristics== |
| | *Usually non-specific. |
| | *Most sarcomas have a pushing border. |
| | **If there is an infiltrative border think: (1) fibromatosis, (2) carcinoma. |
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| =Adipocytic tumours= | | =Adipocytic tumours= |
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| =Smooth muscle tumours= | | =Smooth muscle tumours= |
| | {{Main|Smooth muscle tumours}} |
| | IHC markers: desmin, SMA, H-caldemsin (most specific). |
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| ==Leiomyosarcoma== | | ==Leiomyosarcoma== |
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| =Fibrohistiocytic tumours= | | =Fibrohistiocytic tumours= |
| | ''Fibrohistiocytic'' refers (only) to the histomorphologic appearance and therefore may be written in quotation marks; these tumours are not derived from histiocytes (or tissue macrophages), as the name implies.<ref name=pmid20055912>{{Cite journal | last1 = Luzar | first1 = B. | last2 = Calonje | first2 = E. | title = Cutaneous fibrohistiocytic tumours - an update. | journal = Histopathology | volume = 56 | issue = 1 | pages = 148-65 | month = Jan | year = 2010 | doi = 10.1111/j.1365-2559.2009.03447.x | PMID = 20055912 }}</ref> |
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| ==Pleomorphic undifferentiated sarcoma== | | ==Pleomorphic undifferentiated sarcoma== |
| *Abbreviated ''PUS''. | | *Abbreviated ''PUS''. |
| *[[AKA]] ''Undifferentiated pleomorphic sarcoma'', abbreviated ''UPS''. | | *[[AKA]] ''Undifferentiated pleomorphic sarcoma'', abbreviated ''UPS''. |
| *Previously known as ''malignant fibrous histiocytoma'', abbreviated ''MFH''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref> | | *Previously known as ''malignant fibrous histiocytoma'', abbreviated ''MFH''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref> |
| | | {{Main|Pleomorphic undifferentiated sarcoma}} |
| ===General===
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| *Common sarcoma.
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| *Usu. deep tissue of the trunk and extremities.
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| *A diagnosis of exclusion<ref name=pmid19671033>{{cite journal |author=Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C |title=MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century |journal=Expert Rev Anticancer Ther |volume=9 |issue=8 |pages=1135–44 |year=2009 |month=August |pmid=19671033 |pmc=3000413 |doi=10.1586/era.09.76 |url=}}</ref> / wastebasket for unclassifiable high grade sarcomas.
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP_613>{{Ref WMSP|613}}</ref>
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| *Storiform pattern ([[AKA]] ''patternless pattern'') - '''key feature'''.
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| *Marked [[nuclear pleomorphism]] '''key feature'''.
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| **Variation is nuclear size, nuclear shape and nuclear staining (esp. hyperchromasia).
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| *Mitoses - abundant; atypical mitoses common.
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| *Necrosis (common).
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| *Mix of spindle cells and epithelioid cells.
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| Other findings:
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| *+/-Giant cells (see subclassification).
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| *+/-Inflammation (see subclassification).
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| **Neutrophils.
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| **Eosinophils.
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| Image:
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| *[http://commons.wikimedia.org/w/index.php?title=File:Pleomorphic_undifferentiated_sarcoma_-_very_high_mag.jpg PUS - high mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Pleomorphic_undifferentiated_sarcoma_-_intermed_mag.jpg PUS - intermed. mag. (WC)].
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| ====Subclassification====
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| Pleomorphic sarcoma (PS) is subclassified the following way:<ref name=Ref_WMSP_613-4>{{Ref WMSP|613-4}}</ref>
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| *PS with giant cells.
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| *PS with inflammation.
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| *PUS (not otherwise specified) - wastebasket diagnosis; if neither of the above two apply.
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| =Fibroblastic/myofibroblastic tumours= | | =Fibroblastic/myofibroblastic tumours= |
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| =Tumours of uncertain differentiation= | | =Tumours of uncertain differentiation= |
| ==Extrarenal malignant rhabdoid tumour== | | ==Angiomatoid fibrous histiocytoma== |
| *Essentially identical to ''renal malignant rhabdoid tumour''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
| | {{Main|Angiomatoid fibrous histiocytoma}} |
| ===General=== | | |
| *Usu. children < 2 years old. | | ==Aggressive angiomyxoma== |
| *Very poor prognosis.
| | *[[AKA]] deep aggressive angiomyxoma. |
| *In the CNS it is known as ''[[atypical teratoid-rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
| | {{Main|Aggressive angiomyxoma}} |
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| ===Microscopic=== | | ==Angiomyofibroblastoma== |
| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
| | {{Main|Angiomyofibroblastoma}} |
| *Variable architecture.
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| *Round cells.
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| *Eccentric vesicular nucleus.
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| *Prominent [[nucleolus]] -- '''key feature'''.
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| ===IHC=== | | ==Extrarenal malignant rhabdoid tumour== |
| *INI1 (SMARCB1) -ve. | | *Essentially identical to ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref> |
| **AKA BAF47.
| | {{Main|Extrarenal malignant rhabdoid tumour}} |
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| ==Ewing sarcoma/PNET== | | ==Ewing sarcoma/PNET== |
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| ==Epithelioid sarcoma== | | ==Epithelioid sarcoma== |
| ===General===
| | :Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''. |
| *Rare.
| | {{Main|Epithelioid sarcoma}} |
| *Adolescents, young adults.
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| Subclassification:<ref name=pmid9042279>{{cite journal |author=Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD |title="Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series |journal=Am. J. Surg. Pathol. |volume=21 |issue=2 |pages=130–46 |year=1997 |month=February |pmid=9042279 |doi= |url=}}</ref>
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| *Proximal type:
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| **More aggressive.
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| *Distal type:
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| ===Microscopic===
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| Features:<ref>{{Ref WHOSTAB|205}}</ref>
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| *Epithelioid morphology and spindle morphology - which predominates is dependent on location (see subclassification).
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| *+/-Prominent nucleolus - '''distinctive feature'''.
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| *Zonal necrosis with irregular border.
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| **Descriptors: "Garland necrosis", necrosis with "scalloped border" = necrotic regions with irregular border.
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| Subclassification:<ref name=pmid9042279>{{cite journal |author=Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD |title="Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series |journal=Am. J. Surg. Pathol. |volume=21 |issue=2 |pages=130–46 |year=1997 |month=February |pmid=9042279 |doi= |url=}}</ref>
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| *Proximal-type (proximal location):
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| **More epithelioid.
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| *Distal-type (distal location):
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| **More spindled.
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| **Granuloma-like pattern.
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| DDx:
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| *Carcinoma.
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| *[[Rheumatoid nodule]].
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| *[[Granuloma annulare]].
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| ===IHC===
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| Features:<ref name=pmid10452506>{{cite journal |author=Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF |title=Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis |journal=Hum. Pathol. |volume=30 |issue=8 |pages=934–42 |year=1999 |month=August |pmid=10452506 |doi= |url=}}</ref>
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| *INI1 (SMARCB1<ref>{{OMIM|601607}}</ref>) -ve.<ref name=pmid19997734>{{cite journal |author=Mentzel T |title=[Epithelioid sarcoma: morphologic variants and differential diagnosis] |language=German |journal=Pathologe |volume=31 |issue=2 |pages=135–41 |year=2010 |month=March |pmid=19997734 |doi=10.1007/s00292-009-1250-0 |url=}}</ref>
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| *Vimentin +ve.
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| *Various keratins +ve.
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| **Keratin 8, Keratin 19 +ve.
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| **34betaE12 +ve/-ve.
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| *CD34 +ve.
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| **Malignant rhabdoid tumour -ve.
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| Others:
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| *S100 -ve (r/o melanoma).
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| *CK7 +ve / CK20 -ve.<ref name=pmid12680954>{{Cite journal | last1 = Humble | first1 = SD. | last2 = Prieto | first2 = VG. | last3 = Horenstein | first3 = MG. | title = Cytokeratin 7 and 20 expression in epithelioid sarcoma. | journal = J Cutan Pathol | volume = 30 | issue = 4 | pages = 242-6 | month = Apr | year = 2003 | doi = | PMID = 12680954 }}</ref>
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| ==Alveolar soft part sarcoma== | | ==Alveolar soft part sarcoma== |
| *Abbreviated ''ASPS''.
| | {{Main|Alveolar soft part sarcoma}} |
| ===General===
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| *Adolescents/young adults.
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| *Children -- classically location: base of tongue and orbit.
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
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| *Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power).
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| *Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
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| *An eccentric nucleus.
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| *+/-Nucleolus.
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-_low_mag.jpg ASPS - low mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-_intermed_mag.jpg ASPS - intermed. mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg ASPS - very high mag. (WC)].
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| ===Molecular===
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| *t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
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| ==Desmoplastic small round cell tumour== | | ==Desmoplastic small round cell tumour== |
| *Abbreviated ''DSRCT''.
| | {{Main|Desmoplastic small round cell tumour}} |
| ===General===
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| *Males > females.
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| *Usu. affects young adults.
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| *Typically retroperitoneal.
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| *Poor prognosis.
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| ===Microscopic===
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| Features:<ref name=pmid10207460>{{cite journal |author=Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC |title=Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation |journal=Radiology |volume=210 |issue=3 |pages=633–8 |year=1999 |month=March |pmid=10207460 |doi= |url=http://radiology.rsna.org/content/210/3/633.full}}</ref>
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| #Broad bands of paucicellular fibrous stroma with:
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| #Small round cells in nests with an undulating sharp border.
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| #*The small round cells lack distinct nucleoli and have scant cytoplasm; they are ''[[small round cell tumour]]'' cells.
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| Notes:
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| *Usu. abundant mitoses.
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| *+/-Necrosis.
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| Images:
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| *[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg DSRCT - intermed. mag. (WC)].
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| *[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg DSRCT - very high mag. (WC)].
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| DDx:
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| *Metastatic [[germ cell tumour]] (DDx of location and age).
| |
| *[[Embryonal RMS]].
| |
| **It should be noted that DSRCT, like embryonal RMS, is +ve for desmin!
| |
| *Solid variant of [[alveolar RMS]].
| |
| **Nests in alveolar RMS have round edges.
| |
| | |
| ===IHC===
| |
| Features:
| |
| *AE1/AE3 +ve.
| |
| *Desmin +ve.
| |
| *EMA +ve.
| |
| *Actin -ve.
| |
| *WT1 (N-terminal) -ve.
| |
| *WT1 (C-terminal) +ve.
| |
| *CD57 +ve.
| |
| | |
| ===Molecular===
| |
| *t(11;22)(p13;q12).<ref name=pmid17964965>{{cite journal |author=Lee YS, Hsiao CH |title=Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients |journal=J. Formos. Med. Assoc. |volume=106 |issue=10 |pages=854–60 |year=2007 |month=October |pmid=17964965 |doi=10.1016/S0929-6646(08)60051-0 |url=}}</ref><ref>{{cite journal |author=Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP |title=Results of multimodal treatment for desmoplastic small round cell tumors |journal=J. Pediatr. Surg. |volume=40 |issue=1 |pages=251–5 |year=2005 |month=January |pmid=15868593 |doi=10.1016/j.jpedsurg.2004.09.046 |url=http://www.dsrct.com/JPS%20Article.pdf}}</ref>
| |
|
| |
|
| ==Clear cell sarcoma== | | ==Clear cell sarcoma== |
| *Known among pathologists as "soft-tissue melanoma" and "melanoma of the soft parts", as it has a strong morphological resemblance.<ref name=pmid18300804>{{cite journal |author=Hisaoka M, Ishida T, Kuo TT, ''et al.'' |title=Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases |journal=Am. J. Surg. Pathol. |volume=32 |issue=3 |pages=452–60 |year=2008 |month=March |pmid=18300804 |doi=10.1097/PAS.0b013e31814b18fb |url=}}</ref>
| | {{Main|Clear cell sarcoma}} |
| **Molecular changes and origin distinct from melanoma.
| |
| *Incidence: rare soft tissue tumour.
| |
| | |
| ===Clinical===
| |
| *Usually - deep soft tissue ''or'' extremities.
| |
| *Guarded prognosis.
| |
| *First described in 1965.<ref>URL: [http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928 http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928]. Accessed on: 5 May 2010.</ref>
| |
| | |
| ===Microscopy===
| |
| Features:<ref name=pmid18300804/>
| |
| *Architecture: sheets or fascicular (bundles) arrangement.
| |
| *Cells: Spindle cells or epithelioid cells.
| |
| *Prominent nucleoli - basophilic - '''key feature'''.
| |
| *Fibrous septae.
| |
| *Uniform.
| |
| *+/-Binucleation.
| |
| | |
| DDx:
| |
| *[[Malignant melanoma]].
| |
| *[[PEComa]].
| |
| *Carcinoma.
| |
| | |
| Image:
| |
| *[http://commons.wikimedia.org/wiki/File:Clear_cell_sarcoma.Image12.jpg Clear cell sarcoma (WC)].
| |
| *[http://www.informaworld.com/ampp/image?path=/713690780/789166263/sonc_a_284443_o_f0003g.jpeg Clear cell sarcoma (informaworld.com)].
| |
| | |
| ===IHC===
| |
| Features:<ref name=pmid18300804/>
| |
| *S100 +ve.
| |
| *HMB-45 +ve.
| |
| *Melan A (MART-1) +ve; sometimes -ve.
| |
| *BCL2 +ve.
| |
| *CD57 +ve (usually).
| |
| | |
| Keratins:
| |
| *EMA may be +ve.
| |
| *CAM5.2 -ve.
| |
| *AE1/AE3 -ve.
| |
| | |
| ===Molecular studies===
| |
| *Chromosomal translocation t(12;22)(q13;q12).<ref name=pmid18300804/>
| |
| **Fusion transcripts:
| |
| ***EWSR1-ATF1.
| |
| ***EWSR1-CREB1 (GI tract associated).
| |
|
| |
|
| ==Synovial sarcoma== | | ==Synovial sarcoma== |
| ===General===
| | {{Main|Synovial sarcoma}} |
| *Does not arise from cartilage.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
| |
| **Usually close to a joint.
| |
| **Usually distal extremity.
| |
| *Young adults or adolescents.
| |
| *Poor prognosis.
| |
| | |
| ===Microscopic===
| |
| Comes in three (histologic) flavours:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref><ref>{{cite journal |author=Schaal CH, Navarro FC, Moraes Neto FA |title=Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma |journal=Int Braz J Urol |volume=30 |issue=3 |pages=210–3 |year=2004 |pmid=15689250 |doi= |url=http://www.brazjurol.com.br/may_june_2004/Schaal_ing_210_213.htm}}</ref>
| |
| #Spindle cell sarcoma with features of hemangiopericytoma, i.e. staghorn vessels.
| |
| #Biphasic synovial sarcoma:
| |
| ##Spindle cells with features of hemangiopericytoma.
| |
| ##Epitheliod glands or nests.
| |
| #Primitive round cell type.
| |
| | |
| Features:
| |
| *[[Herring bone]] or [[vesicular pattern]].
| |
| *Spindle cells.
| |
| *+/-Glandular component.
| |
| | |
| DDx:
| |
| *[[MPNST]].
| |
| **Can be difficult.
| |
| | |
| Images:
| |
| *[http://commons.wikimedia.org/wiki/File:Monophasic_synovial_sarcoma_-_intermed_mag.jpg Monophasic synovial sarcoma with staghorn vessels - intermed. mag. (WC)].
| |
| *[http://www.scielo.br/img/revistas/ibju/v30n3/3a06f03.jpg Synovial sarcoma (scielo.br)].
| |
| *[http://www.humpath.com/spip.php?page=article&id_article=1965 Synovial sarcoma - collection of images (humpath.com)].
| |
| | |
| ===IHC===
| |
| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
| |
| *Vimentin +ve.
| |
| *EMA +ve.
| |
| *BCL2 +ve.
| |
| *CD99 +ve.
| |
| | |
| Others:
| |
| *Beta-catenin +ve ~30-70%.<ref name=pmid16740029>{{cite journal |author=Horvai AE, Kramer MJ, O'Donnell R |title=Beta-catenin nuclear expression correlates with cyclin D1 expression in primary and metastatic synovial sarcoma: a tissue microarray study |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=6 |pages=792–8 |year=2006 |month=June |pmid=16740029 |doi= |url=}}</ref>
| |
| *Cyclin D1 ~50%.<ref name=pmid16740029/><ref name=pmid15375433>{{cite journal |author=Ng TL, Gown AM, Barry TS, ''et al.'' |title=Nuclear beta-catenin in mesenchymal tumors |journal=Mod. Pathol. |volume=18 |issue=1 |pages=68–74 |year=2005 |month=January |pmid=15375433 |doi=10.1038/modpathol.3800272 |url=}}</ref>
| |
| *TLE1 +ve nuclear staining; not specific for synovial sarcoma.<ref name=pmid19363472>{{cite journal |author=Kosemehmetoglu K, Vrana JA, Folpe AL |title=TLE1 expression is not specific for synovial sarcoma: a whole section study of 163 soft tissue and bone neoplasms |journal=Mod. Pathol. |volume=22 |issue=7 |pages=872–8 |year=2009 |month=July |pmid=19363472 |doi=10.1038/modpathol.2009.47 |url=http://www.nature.com/modpathol/journal/v22/n7/full/modpathol200947a.html}}</ref><ref>{{cite journal |author=Seo SW, Lee H, Lee HI, Kim HS |title=The role of TLE1 in synovial sarcoma |journal=J Orthop Res |volume= |issue= |pages= |year=2011 |month=February |pmid=21319215 |doi=10.1002/jor.21318 |url=}}</ref>
| |
| | |
| Notes:
| |
| *Synovial sarcoma & MPNST:
| |
| **Both +ve: PGP9.5 (UCHL1<ref>{{OMIM|191342}}</ref>), S100, NGFR, CD56, CD99, vimentin.
| |
| **Synovial +ve: EMA, keratin, BCL2, TLE1.
| |
| **MPNST +ve: nestin, CD34.
| |
| | |
| Trivia:
| |
| *PGP in ''PGP9.5'' = protein gene product.<ref name=pmid6343558>{{Cite journal | last1 = Doran | first1 = JF. | last2 = Jackson | first2 = P. | last3 = Kynoch | first3 = PA. | last4 = Thompson | first4 = RJ. | title = Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis. | journal = J Neurochem | volume = 40 | issue = 6 | pages = 1542-7 | month = Jun | year = 1983 | doi = | PMID = 6343558 }}</ref>
| |
| | |
| ===Molecular pathology===
| |
| Associated [[translocation]]:
| |
| *t(X;18)(p11.2;q11.2).<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/300813 http://www.ncbi.nlm.nih.gov/omim/300813]. Accessed on: 30 May 2010.</ref>
| |
| **SYT/SSX fusion gene.
| |
| | |
| Several SSX genes:
| |
| *SSX1.
| |
| *SSX2 - better survival
| |
| *SSX4 - uncommon.
| |
| | |
| Notes:
| |
| *At HSC t(X,18) = synovial sarcoma.
| |
|
| |
|
| =Other= | | =Other= |
| ==Granulocytic sarcoma== | | ==Granulocytic sarcoma== |
| *Common alternate terms: myeloid sarcoma, chloroma. | | *Common alternate terms: extramedullary leukemia,<ref name=pmid21795742>{{Cite journal | last1 = Bakst | first1 = RL. | last2 = Tallman | first2 = MS. | last3 = Douer | first3 = D. | last4 = Yahalom | first4 = J. | title = How I treat extramedullary acute myeloid leukemia. | journal = Blood | volume = 118 | issue = 14 | pages = 3785-93 | month = Oct | year = 2011 | doi = 10.1182/blood-2011-04-347229 | PMID = 21795742 }}</ref> myeloid sarcoma, chloroma. |
| *Other terms:<ref name=pmid21556238>{{Cite journal | last1 = Eom | first1 = KS. | last2 = Kim | first2 = TY. | title = Intraparenchymal myeloid sarcoma and subsequent spinal myeloid sarcoma for acute myeloblastic leukemia. | journal = J Korean Neurosurg Soc | volume = 49 | issue = 3 | pages = 171-4 | month = Mar | year = 2011 | doi = 10.3340/jkns.2011.49.3.171 | PMID = 21556238 | PMC = 3085814 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3085814/ }}</ref> myeloblastoma, chloromyeloma, chloromyelosarcoma, granulocytic leukosarcoma, or myelosarcoma. | | *Other terms:<ref name=pmid21556238>{{Cite journal | last1 = Eom | first1 = KS. | last2 = Kim | first2 = TY. | title = Intraparenchymal myeloid sarcoma and subsequent spinal myeloid sarcoma for acute myeloblastic leukemia. | journal = J Korean Neurosurg Soc | volume = 49 | issue = 3 | pages = 171-4 | month = Mar | year = 2011 | doi = 10.3340/jkns.2011.49.3.171 | PMID = 21556238 | PMC = 3085814 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3085814/ }}</ref> myeloblastoma, chloromyeloma, chloromyelosarcoma, granulocytic leukosarcoma, or myelosarcoma. |
| | | {{Main|Granulocytic sarcoma}} |
| ===General===
| |
| *Soft tissue manifestation of acute myeloid leukemia.<ref name=pmid21556238/>
| |
| | |
| ===Microscopic===
| |
| Features:
| |
| *Cluster of atypical small blue cells in [[soft tissue lesions|soft tissue]].
| |
| | |
| Note:
| |
| *May mimic [[small cell carcinoma]], large cell lymphomas ([[DLBCL]], [[ALCL]]), [[small round cell tumour]]s.
| |
| | |
| Images:
| |
| *[http://commons.wikimedia.org/wiki/File:Chloroma_-_high_mag.jpg Chloroma - high mag. (WC)].
| |
| *[http://commons.wikimedia.org/wiki/File:Chloroma_-_very_high_mag.jpg Chloroma - very high mag. (WC)].
| |
|
| |
|
| =See also= | | =See also= |