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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.


===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.
Line 44: Line 44:


==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
|-
|-
| 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 ?
|-
|-
| 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 ?
|-
|-
| 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 ?
|-
|-
| 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 ?
|- <!--
|- <!--
| name ?
| name ?
| description ?
| description ?
| DDx ?
| DDx ?
| image ?
| image ? -->
| other ? -->
|}
|}


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*[[epithelioid sarcoma|'''E'''pitheliod sarcoma]].
*[[epithelioid sarcoma|'''E'''pitheliod sarcoma]].
*[[fibrosarcoma|'''F'''ibrosarcoma]].
*[[fibrosarcoma|'''F'''ibrosarcoma]].
*[[pleomorphic undifferentiated sarcoma|'''M'''alignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma]].
*[[pleomorphic undifferentiated sarcoma|'''M'''alignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma)]].
*[[synovial sarcoma|'''S'''ynovial cell sarcoma]].
*[[synovial sarcoma|'''S'''ynovial cell sarcoma]].


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*[[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===
*Common sarcoma.
*Usually deep tissue of the trunk and extremities.
*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.
 
===Microscopic===
Features:<ref name=Ref_WMSP_613>{{Ref WMSP|613}}</ref>
*Storiform pattern ([[AKA]] ''patternless pattern'') - '''key feature'''.
*Marked [[nuclear pleomorphism]] '''key feature'''.
**Variation is nuclear size, nuclear shape and nuclear staining (esp. hyperchromasia).
*Mitoses - abundant; atypical mitoses common.
*Necrosis (common).
*Mix of spindle cells and epithelioid cells.
*Deep to skin  - '''important'''.
 
Other findings:
*+/-Giant cells (see subclassification).
*+/-Inflammation (see subclassification).
**Neutrophils.
**Eosinophils.
 
Notes:
*Superficial lesions with the morphology of ''PUS'' are called by some ''[[atypical fibroxanthoma]]s'' (AFXs).
 
DDx:
*[[Atypical fibroxanthoma]] (AFX) - superficial skin.
*[[Dedifferentiated liposarcoma]].
*[[Leiomyosarcoma]].
*[[Metaplastic carcinoma]].
*[[Malignant melanoma]].
*[[Rhabdomyosarcoma]].
*[[Synovial sarcoma]].
*Others.
 
====Images====
<gallery>
Image:Pleomorphic_undifferentiated_sarcoma_-_very_high_mag.jpg | PUS - high mag. (WC/Nephron)
Image:Pleomorphic_undifferentiated_sarcoma_-_intermed_mag.jpg | PUS - intermed. mag. (WC/Nephron)
</gallery>
====Subclassification====
Pleomorphic sarcoma (PS) is subclassified the following way:<ref name=Ref_WMSP_613-4>{{Ref WMSP|613-4}}</ref>
*PS with giant cells.
*PS with inflammation.
*PUS (not otherwise specified) - wastebasket diagnosis; if neither of the above two apply.
 
===IHC===
Exclusionary stains - should be negative:
*AE1/AE3.
*p63.
*Myogenin.
*S-100.
*HMB-45.
 
Usually negative, may be positive:<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
*Desmin.
*SMA.
 
Commonly positive:
*CD68.<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
*Vimentin.


=Fibroblastic/myofibroblastic tumours=
=Fibroblastic/myofibroblastic tumours=
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=Tumours of uncertain differentiation=
=Tumours of uncertain differentiation=
==Angiomatoid fibrous histiocytoma==
==Angiomatoid fibrous histiocytoma==
===General===
{{Main|Angiomatoid fibrous histiocytoma}}
*Rarely metastasizes.
*Children & young adults.
 
===Microscopic===
Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
*Cystic spaces with blood - simulates a vascular neoplasm.<ref name=pmid228836>{{Cite journal  | last1 = Enzinger | first1 = FM. | title = Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. | journal = Cancer | volume = 44 | issue = 6 | pages = 2147-57 | month = Dec | year = 1979 | doi =  | PMID = 228836 }}</ref>
*Epithelioid to spindle cells.
**May have a histiocytic appearance.<ref>URL: [http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html]. Accessed on: 15 November 2011.</ref>
*Inflammation.
**Lymphoid cuff<ref name=pmid20154033/> - lymphocytes around periphery of lesion.
*Hemorrhage.
 
Note:
*The first impression may be that it is [[granuloma|granulomatous inflammation]]; however, the cytoplasm doesn't fit (it isn't bubbly and it isn't sheet-like), and the nuclei aren't quite right (few footprint shaped nuclei).
 
====Images====
<gallery>
Image:Angiomatoid_fibrous_histiocytoma_-_intermed_mag.jpg | AFH - intermed. mag. (WC/Nephron)
Image:Angiomatoid_fibrous_histiocytoma_-_high_mag.jpg | AFH - high mag. (WC/Nephron)
</gallery>
www:
*[http://www.sarcomaimages.com/index.php?v=Angiomatoid-Fibrous-Histiocytoma AFH (sarcomaimages.com)].
**[http://jcp.bmj.com/content/63/2/124/F1.large.jpg AFH (bmj.com)].<ref name=pmid20154033>{{Cite journal  | last1 = Matsumura | first1 = T. | last2 = Yamaguchi | first2 = T. | last3 = Tochigi | first3 = N. | last4 = Wada | first4 = T. | last5 = Yamashita | first5 = T. | last6 = Hasegawa | first6 = T. | title = Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation. | journal = J Clin Pathol | volume = 63 | issue = 2 | pages = 124-8 | month = Feb | year = 2010 | doi = 10.1136/jcp.2009.072256 | PMID = 20154033 | url = http://jcp.bmj.com/content/63/2/124.full }}
</ref>
*[http://path.upmc.edu/cases/case512.html AFH - several images (upmc.edu)].
 
===IHC===
Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
*CD68 +ve.
*CD57 +ve.
*Desmin +ve.
*Vimentin +ve.
 
===Molecular===
AFH has recurrent [[translocations]]:
*t(12;16) FUS/ATF1.
*t(12;22) EWS/ATF1.


==Aggressive angiomyxoma==
==Aggressive angiomyxoma==
*[[AKA]] deep aggressive angiomyxoma.
*[[AKA]] deep aggressive angiomyxoma.
===General===
{{Main|Aggressive angiomyxoma}}
*Classically a [[vulva]]r mass or (less commonly) a [[scrotum|scrotal]] mass.
**Case report of a thigh lesion.<ref name=pmid18338163>{{Cite journal  | last1 = Heffernan | first1 = EJ. | last2 = Hayes | first2 = MM. | last3 = Alkubaidan | first3 = FO. | last4 = Clarkson | first4 = PW. | last5 = Munk | first5 = PL. | title = Aggressive angiomyxoma of the thigh. | journal = Skeletal Radiol | volume = 37 | issue = 7 | pages = 673-8 | month = Jul | year = 2008 | doi = 10.1007/s00256-008-0465-0 | PMID = 18338163 }}</ref>
*Benign - no metastatic potential.
*"Aggressive" as it has a high recurrance.
 
===Gross===
*Poorly circumscribed.
*"Rubbery" or "gelatinous".
 
Location:
*[[Vulva]] - classic location.
 
Clinical DDx:
*[[Bartholin cyst]].
 
===Microscopic===
Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
#Thick blood vessels that meld into the surrounding stroma - '''key feature'''.
#[[Myxoid stroma]] - '''key feature'''.
#Small stellate cell/spindle cells without significant nuclear atypia.
 
DDx:
*[[Angiomyofibroblastoma]].<ref name=pmid1314521>{{Cite journal  | last1 = Fletcher | first1 = CD. | last2 = Tsang | first2 = WY. | last3 = Fisher | first3 = C. | last4 = Lee | first4 = KC. | last5 = Chan | first5 = JK. | title = Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. | journal = Am J Surg Pathol | volume = 16 | issue = 4 | pages = 373-82 | month = Apr | year = 1992 | doi =  | PMID = 1314521 }}</ref>
*[[Neurofibroma]].
*[[Myxoma]].
 
====Images====
<gallery>
Image:Aggressive_angiomyxoma_-_intermed_mag.jpg | Aggressive angiomyxoma - intermed. mag. (WC/Nephron)
Image:Aggressive_angiomyxoma_-_very_high_mag.jpg | Aggressive angiomyxoma - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.webpathology.com/image.asp?n=1&Case=99 Aggressive angiomyxoma - low mag. (webpathology.com)].
**[http://www.webpathology.com/image.asp?case=99&n=2 Aggressive angiomyxoma - high mag. (webpathology.com)].
*[http://path.upmc.edu/cases/case538.html Aggressive angiomyxoma - several images (upmc.edu)].
 
===IHC===
Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
*Desmin +ve.
*Vimentin +ve.
*ER +ve.
*PR +ve.


==Angiomyofibroblastoma==
==Angiomyofibroblastoma==
===General===
{{Main|Angiomyofibroblastoma}}
*Uncommon.
 
Clinical DDx:
*[[Bartholin cyst]].
 
===Microscopic===
Features:<ref name=pmid1314521>{{Cite journal  | last1 = Fletcher | first1 = CD. | last2 = Tsang | first2 = WY. | last3 = Fisher | first3 = C. | last4 = Lee | first4 = KC. | last5 = Chan | first5 = JK. | title = Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. | journal = Am J Surg Pathol | volume = 16 | issue = 4 | pages = 373-82 | month = Apr | year = 1992 | doi =  | PMID = 1314521 }}</ref>
*Hypercellular zones and hypocellular edematous zones.
*Small blood vessels (~20 micrometers) - no large blood vessels - '''key feature'''.
*[[Myxoid stroma]] - '''key feature'''.
*Small stellate cell/spindle cells without significant nuclear atypia.
 
DDx:
*[[Aggressive angiomyxoma]] - less cellular, large blood vessels.
 
Images:
*[http://www.webpathology.com/image.asp?case=544&n=4 Angiomyofibroblastoma (webpathology.com)]
*[http://www.webpathology.com/image.asp?n=5&Case=544 Angiomyofibroblastoma - high mag. (webpathology.com)].


==Extrarenal malignant rhabdoid tumour==
==Extrarenal malignant rhabdoid tumour==
*Essentially identical to ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Essentially identical to ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
===General===
{{Main|Extrarenal malignant rhabdoid tumour}}
*Usu. children < 2 years old.
*Very poor prognosis.
*In the CNS it is known as ''[[atypical teratoid-rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
 
===Microscopic===
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Variable architecture.
*Round cells.
*Eccentric vesicular nucleus.
*Prominent [[nucleolus]] -- '''key feature'''.
 
===IHC===
*INI1 (SMARCB1) -ve.
**AKA BAF47.


==Ewing sarcoma/PNET==
==Ewing sarcoma/PNET==
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==Epithelioid sarcoma==
==Epithelioid sarcoma==
:Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''.
:Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''.
===General===
{{Main|Epithelioid sarcoma}}
*Rare.
*Adolescents, young adults.
*Serum CA-125 may be useful for following clinically.<ref name=pmid19756736>{{Cite journal  | last1 = Hoshino | first1 = M. | last2 = Kawashima | first2 = H. | last3 = Ogose | first3 = A. | last4 = Kudo | first4 = N. | last5 = Ariizumi | first5 = T. | last6 = Hotta | first6 = T. | last7 = Umezu | first7 = H. | last8 = Hatano | first8 = H. | last9 = Morita | first9 = T. | title = Serum CA 125 expression as a tumor marker for diagnosis and monitoring the clinical course of epithelioid sarcoma. | journal = J Cancer Res Clin Oncol | volume = 136 | issue = 3 | pages = 457-64 | month = Mar | year = 2010 | doi = 10.1007/s00432-009-0678-1 | PMID = 19756736 }}</ref>
 
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>
*Proximal type:
**More aggressive.
*Distal type:
 
===Microscopic===
Features:<ref>{{Ref WHOSTAB|205}}</ref>
*Epithelioid morphology and spindle morphology - which predominates is dependent on location (see subclassification).
*+/-Prominent nucleolus - '''distinctive feature'''.
*Zonal necrosis with irregular border.
**Descriptors: "Garland necrosis", necrosis with "scalloped border" = necrotic regions with irregular border.
 
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>
*Proximal-type (proximal location):
**More epithelioid.
*Distal-type (distal location):
**More spindled.
**Granuloma-like pattern.
 
DDx:
*Carcinoma.
*[[Rheumatoid nodule]].
*[[Granuloma annulare]].
 
====Images====
<gallery>
Image:Epithelioid_sarcoma_-_intermed_mag.jpg | ES - intermed. mag. (WC/Nephron)
Image:Epithelioid_sarcoma_-_high_mag.jpg | ES - high mag. (WC/Nephron)
Image:Epithelioid_sarcoma_-_smarcb1_-_high_mag.jpg | ES - SMARCB1/INI1 - high mag. (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case594.html ES - several images (upmc.edu)].
 
===IHC===
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>
*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>
*Vimentin +ve.
*Various keratins +ve.
**Keratin 8, Keratin 19 +ve.
**34betaE12 +ve/-ve.
*CD34 +ve.
**Malignant rhabdoid tumour -ve.
 
Others:
*S100 -ve (r/o melanoma).
*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>
*CA-125 +ve.<ref name=pmid19756736/><ref name=pmid16740043>{{Cite journal  | last1 = Lee | first1 = HI. | last2 = Kang | first2 = KH. | last3 = Cho | first3 = YM. | last4 = Lee | first4 = OJ. | last5 = Ro | first5 = JY. | title = Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity. | journal = Arch Pathol Lab Med | volume = 130 | issue = 6 | pages = 871-4 | month = Jun | year = 2006 | doi = 10.1043/1543-2165(2006)130[871:PESWES]2.0.CO;2 | PMID = 16740043 }}</ref>


==Alveolar soft part sarcoma==
==Alveolar soft part sarcoma==
*Abbreviated ''ASPS''.
{{Main|Alveolar soft part sarcoma}}
===General===
*Adolescents/young adults.
*Children -- classically location: base of [[tongue]] and orbit.
*Typical indolent initially - ultimately a poor prognosis.<ref name=pmid17071801>{{Cite journal  | last1 = Folpe | first1 = AL. | last2 = Deyrup | first2 = AT. | title = Alveolar soft-part sarcoma: a review and update. | journal = J Clin Pathol | volume = 59 | issue = 11 | pages = 1127-32 | month = Nov | year = 2006 | doi = 10.1136/jcp.2005.031120 | PMID = 17071801 |PMC = 1860509 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/?tool=pubmed }}</ref>
 
===Microscopic===
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power).
*Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
*An eccentric nucleus.
*+/-Nucleolus.
 
DDx:
*[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells.
 
====Images====
<gallery>
Image:Alveolar_soft_part_sarcoma_-_low_mag.jpg | ASPS - low mag. (WC/Nephron)
Image:Alveolar_soft_part_sarcoma_-_intermed_mag.jpg | ASPS - intermed. mag. (WC/Nephron)
Image:Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg | ASPS - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.webpathology.com/image.asp?case=508&n=10 ASPS - PAS (webpathology.com)].
 
===Stains===
*PAS +ve (cytoplasmic) - considered the most useful.<ref name=pmid17516754>{{Cite journal  | last1 = Zarrin-Khameh | first1 = N. | last2 = Kaye | first2 = KS. | title = Alveolar soft part sarcoma. | journal = Arch Pathol Lab Med | volume = 131 | issue = 3 | pages = 488-91 | month = Mar | year = 2007 | doi = 10.1043/1543-2165(2007)131[488:ASPS]2.0.CO;2 | PMID = 17516754 }}</ref>
*PASD +ve (cytoplasmic).
 
===IHC===
*TFE3 +ve -- suggestive of characteristic translocation.
 
===Molecular===
*t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
 
Note:
*Same translocation may be seen in ''[[renal tumour with Xp11.2 translocation]]''.
 
===EM===
*Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.<ref name=pmid17071801/>
 
Image:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig8/ Distinctive crystal lattice in ASPS (nlm.nih.gov)].<ref name=pmid17071801/>


==Desmoplastic small round cell tumour==
==Desmoplastic small round cell tumour==
*Abbreviated ''DSRCT''.
{{Main|Desmoplastic small round cell tumour}}
===General===
*Males > females.
*Usu. affects young adults.
*Typically retroperitoneal.
*Poor prognosis.
 
===Microscopic===
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>
#Broad bands of paucicellular fibrous stroma with:
#Small round cells in nests with an undulating sharp border.
#*The small round cells lack distinct nucleoli and have scant cytoplasm; they are ''[[small round cell tumour]]'' cells.
 
Notes:
*Usu. abundant mitoses.
*+/-Necrosis.
 
DDx:
*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.
 
====Images====
<gallery>
Image:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg | DSRCT - intermed. mag. (WC/Nephron)
Image:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg | DSRCT - very high mag. (WC/Nephron)
</gallery>
 
===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}}
*Not to be confused with ''[[clear cell sarcoma of the kidney]]''.
 
===General===
*Molecular changes and origin distinct from [[malignant melanoma]].
*Incidence: rare soft tissue tumour.
 
====Clinical====
*Usually - deep soft tissue ''or'' extremities.
**Classically associated with tendons and aponeuroses.<ref name=pmid17227118>{{Cite journal  | last1 = Dim | first1 = DC. | last2 = Cooley | first2 = LD. | last3 = Miranda | first3 = RN. | title = Clear cell sarcoma of tendons and aponeuroses: a review. | journal = Arch Pathol Lab Med | volume = 131 | issue = 1 | pages = 152-6 | month = Jan | year = 2007 | doi = 10.1043/1543-2165(2007)131[152:CCSOTA]2.0.CO;2 | PMID = 17227118 }}</ref>
*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>
 
===Microscopic===
Features:<ref name=pmid18300804/>
*Architecture: sheeting ''or'' fascicular (bundles) arrangement.
**Fibrous septae - between tumour cells.
*Tumour cells uniform (low pleomorphism) - spindle-shaped ''or'' epithelioid:
**Classically have clear cytoplasm.
**Prominent nucleoli - basophilic - '''key feature'''.
**+/-Binucleation.
 
DDx:
*[[Malignant melanoma]].
*[[PEComa]].
*Carcinoma.
 
====Images====
<gallery>
Image:Clear_cell_sarcoma_-_intermed_mag.jpg | Clear cell sarcoma - intermed. mag. (WC)
Image:Clear_cell_sarcoma_-_very_high_mag.jpg | Clear cell sarcoma - very high mag. (WC)
Image:Clear_cell_sarcoma.Image12.jpg | Clear cell sarcoma (WC/Gardner)
</gallery>
===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.
****Same translocation in: ''[[myxoid liposarcoma]]'',<ref name=pmid21115923>{{Cite journal  | last1 = Suzuki | first1 = K. | last2 = Matsui | first2 = Y. | last3 = Endo | first3 = K. | last4 = Kubo | first4 = T. | last5 = Hasegawa | first5 = T. | last6 = Kimura | first6 = T. | last7 = Ohtani | first7 = O. | last8 = Yasui | first8 = N. | title = Myxoid liposarcoma with EWS-CHOP type 1 fusion gene. | journal = Anticancer Res | volume = 30 | issue = 11 | pages = 4679-83 | month = Nov | year = 2010 | doi =  | PMID = 21115923 }}</ref> and ''[[hyalinizing clear cell carcinoma]]''.
***EWSR1-CREB1 (GI tract associated).


==Synovial sarcoma==
==Synovial sarcoma==
*Abbreviated ''SS''.
{{Main|Synovial sarcoma}}
 
===General===
*Does not arise from cartilage.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Young adults or adolescents.
**Classic age: 30s.
*Poor prognosis.
 
Clinical:<ref name=pmid16973781>{{Cite journal  | last1 = Murphey | first1 = MD. | last2 = Gibson | first2 = MS. | last3 = Jennings | first3 = BT. | last4 = Crespo-Rodríguez | first4 = AM. | last5 = Fanburg-Smith | first5 = J. | last6 = Gajewski | first6 = DA. | title = From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation. | journal = Radiographics | volume = 26 | issue = 5 | pages = 1543-65 | month =  | year =  | doi = 10.1148/rg.265065084 | PMID = 16973781 | URL = http://radiographics.rsna.org/content/26/5/1543.long }}</ref>
*Present with soft palpable mass - slow growing - often for years.
*May present with pain.
**Uncommon finding in sarcomas.
 
===Gross===
Location:
*Usually close to a joint.
*Usually distal extremity ~65% of cases.<ref name=pmid16973781/>
**Upper extremity ~20% of cases.<ref name=pmid16973781/>
 
Appearance - often non-specific:
*Solid often lobulated +/- cystic component.
*Grey-yellow.
*Pushing border to ill-defined border.
 
Images:
*[http://www.sarcomaimages.com/images/db/synovial-sarcoma/synovial-sarcoma-01-gross-image,-sarcoma-images.jpg SS (sarcomaimages.com)].<ref>URL: [http://www.sarcomaimages.com/index.php?v=Synovial-Sarcoma http://www.sarcomaimages.com/index.php?v=Synovial-Sarcoma]. Accessed on: 2 April 2012.</ref>
*[http://www.tumorlibrary.com/case/detail.jsp?image_id=2462 SS (tumorlibrary.com)].
 
===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]] - '''key feature'''.
*Spindle cells.
*+/-Glandular component - typically more pink.
*+/-Calcification - uncommon.
**Extensive calcification = better prognosis.<ref name=pmid6282441>{{Cite journal  | last1 = Varela-Duran | first1 = J. | last2 = Enzinger | first2 = FM. | title = Calcifying synovial sarcoma. | journal = Cancer | volume = 50 | issue = 2 | pages = 345-52 | month = Jul | year = 1982 | doi =  | PMID = 6282441 }}</ref>
 
DDx:
*[[MPNST]].
**Can be difficult.
 
====Images====
<gallery>
*Image:Monophasic_synovial_sarcoma_-_intermed_mag.jpg | Monophasic synovial sarcoma with staghorn vessels - intermed. mag. (WC/Nephron)
</gallery>
www:
*[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)].
*[http://path.upmc.edu/cases/case292.html Synovial sarcoma - several images (upmc.edu)].
*[http://radiographics.rsna.org/content/26/5/1543/F14.expansion.html Biphasic SS (radiographics.rsna.org)].
*[http://radiographics.rsna.org/content/26/5/1543.long Monophasic SS (radiographics.rsna.org)].
 
===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>
 
Typically negative:<ref>URL: [http://path.upmc.edu/cases/case292/dx.html http://path.upmc.edu/cases/case292/dx.html]. Accessed on: 14 January 2012.</ref>
*CD34.
*S100 ~30% +ve.
*SMA.
 
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>
 
===EM===
Features:<ref name=pmid9930576>{{Cite journal  | last1 = Fisher | first1 = C. | title = Synovial sarcoma. | journal = Ann Diagn Pathol | volume = 2 | issue = 6 | pages = 401-21 | month = Dec | year = 1998 | doi =  | PMID = 9930576 }}</ref>
*Biphasic tumour have biphasic ultrastructural features (unlike spindle cell carcinoma and [[epithelioid sarcoma]]).
*Epithelioid component is adenocarcinoma-like - they have:
**Intermediate filaments.
**[[Tonofilament]]s.
**Microvilli.
*[[Spindle cell]] component - mostly features less.
**Poorly formed desmosomes.
**No intermediate filaments, no myofilaments.
 
===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 - cannot be differentiated with standard karyotyping:
*SSX1.
*SSX2 - better survival, rarely seen in biphasic tumours.<ref>{{Ref APBR|625 Q6}}</ref>
*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====
<gallery>
Image:Chloroma_-_high_mag.jpg | Chloroma - high mag. (WC)
Image:Chloroma_-_very_high_mag.jpg | Chloroma - very high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case306/micro.html Granulocytic sarcoma - several crappy images (upmc.edu)].
*[http://path.upmc.edu/cases/case379.html Myeloid sarcoma - several images (upmc.edu)].


=See also=
=See also=
Michael
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