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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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| | [[Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg |thumb|center|150px |Herring bone. (WC)]] | | | [[Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg |thumb|center|150px |Herring bone. (WC)]] |
| |- | | |- |
| | 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]] |
| | [[Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg |thumb|center|150px| Fasicular pattern. (WC)]] | | | [[Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg |thumb|center|150px| Fascicular pattern. (WC)]] |
| |- | | |- |
| | Biphasic | | | Biphasic |
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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===
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| *Common sarcoma.
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| *Usually 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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| *Deep to skin - '''important'''.
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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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| Notes:
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| *Superficial lesions with the morphology of ''PUS'' are called by some ''[[atypical fibroxanthoma]]s'' (AFXs).
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| DDx:
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| *[[Atypical fibroxanthoma]] (AFX) - superficial skin.
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| *[[Dedifferentiated liposarcoma]].
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| *[[Leiomyosarcoma]].
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| *[[Metaplastic carcinoma]].
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| *[[Malignant melanoma]].
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| *[[Rhabdomyosarcoma]].
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| *[[Synovial sarcoma]].
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| *Others.
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| ====Images====
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| <gallery>
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| Image:Pleomorphic_undifferentiated_sarcoma_-_very_high_mag.jpg | PUS - high mag. (WC/Nephron)
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| Image:Pleomorphic_undifferentiated_sarcoma_-_intermed_mag.jpg | PUS - intermed. mag. (WC/Nephron)
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| </gallery>
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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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| ===IHC===
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| Exclusionary stains - should be negative:
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| *AE1/AE3.
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| *p63.
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| *Myogenin.
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| *S-100.
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| *HMB-45.
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| Usually negative, may be positive:<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
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| *Desmin.
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| *SMA.
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| Commonly positive:
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| *CD68.<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
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| *Vimentin.
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| =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.
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| *Children & young adults.
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
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| *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>
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| *Epithelioid to spindle cells.
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| **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>
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| *Inflammation.
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| **Lymphoid cuff<ref name=pmid20154033/> - lymphocytes around periphery of lesion.
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| *Hemorrhage.
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| Note:
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| *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).
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| ====Images====
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| <gallery>
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| Image:Angiomatoid_fibrous_histiocytoma_-_intermed_mag.jpg | AFH - intermed. mag. (WC/Nephron)
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| Image:Angiomatoid_fibrous_histiocytoma_-_high_mag.jpg | AFH - high mag. (WC/Nephron)
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| </gallery>
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| www:
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| *[http://www.sarcomaimages.com/index.php?v=Angiomatoid-Fibrous-Histiocytoma AFH (sarcomaimages.com)].
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| *[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 }}
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| </ref>
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| *[http://path.upmc.edu/cases/case512.html AFH - several images (upmc.edu)].
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| ===IHC===
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| Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
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| *CD68 +ve.
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| *CD57 +ve.
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| *Desmin +ve.
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| *Vimentin +ve.
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| ===Molecular===
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| AFH has recurrent [[translocations]]:
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| *t(12;16) FUS/ATF1.
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| *t(12;22) EWS/ATF1.
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| ==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.
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| **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>
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| *Benign - no metastatic potential.
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| *"Aggressive" as it has a high recurrance.
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| ===Gross===
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| *Poorly circumscribed.
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| *"Rubbery" or "gelatinous".
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| Location:
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| *[[Vulva]] - classic location.
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| Clinical DDx:
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| *[[Bartholin cyst]].
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
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| #Thick blood vessels that meld into the surrounding stroma - '''key feature'''.
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| #[[Myxoid stroma]] - '''key feature'''.
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| #Small stellate cell/spindle cells without significant nuclear atypia.
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| DDx:
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| *[[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>
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| *[[Neurofibroma]].
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| *[[Myxoma]].
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| ====Images====
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| <gallery>
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| Image:Aggressive_angiomyxoma_-_intermed_mag.jpg | Aggressive angiomyxoma - intermed. mag. (WC/Nephron)
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| Image:Aggressive_angiomyxoma_-_very_high_mag.jpg | Aggressive angiomyxoma - very high mag. (WC/Nephron)
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| </gallery>
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| www:
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| *[http://www.webpathology.com/image.asp?n=1&Case=99 Aggressive angiomyxoma - low mag. (webpathology.com)].
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| *[http://www.webpathology.com/image.asp?case=99&n=2 Aggressive angiomyxoma - high mag. (webpathology.com)].
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| *[http://path.upmc.edu/cases/case538.html Aggressive angiomyxoma - several images (upmc.edu)].
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| ===IHC===
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| Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
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| *Desmin +ve.
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| *Vimentin +ve.
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| *ER +ve.
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| *PR +ve.
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| ==Angiomyofibroblastoma== | | ==Angiomyofibroblastoma== |
| ===General===
| | {{Main|Angiomyofibroblastoma}} |
| *Uncommon.
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| Clinical DDx:
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| *[[Bartholin cyst]].
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| ===Microscopic===
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| 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>
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| *Hypercellular zones and hypocellular edematous zones.
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| *Small blood vessels (~20 micrometers) - no large blood vessels - '''key feature'''.
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| *[[Myxoid stroma]] - '''key feature'''.
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| *Small stellate cell/spindle cells without significant nuclear atypia.
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| DDx:
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| *[[Aggressive angiomyxoma]] - less cellular, large blood vessels.
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| Images:
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| *[http://www.webpathology.com/image.asp?case=544&n=4 Angiomyofibroblastoma (webpathology.com)]
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| *[http://www.webpathology.com/image.asp?n=5&Case=544 Angiomyofibroblastoma - high mag. (webpathology.com)].
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| ==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.
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| *Very poor prognosis.
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| *In the CNS it is known as ''[[atypical teratoid-rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
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| *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===
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| *INI1 (SMARCB1) -ve.
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| **AKA BAF47.
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| ==Ewing sarcoma/PNET== | | ==Ewing sarcoma/PNET== |
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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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| *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>
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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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| DDx:
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| *[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells.
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| ====Images====
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| <gallery>
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| Image:Alveolar_soft_part_sarcoma_-_low_mag.jpg | ASPS - low mag. (WC/Nephron)
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| Image:Alveolar_soft_part_sarcoma_-_intermed_mag.jpg | ASPS - intermed. mag. (WC/Nephron)
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| Image:Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg | ASPS - very high mag. (WC/Nephron)
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| </gallery>
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| www:
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| *[http://www.webpathology.com/image.asp?case=508&n=10 ASPS - PAS (webpathology.com)].
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| ===Stains===
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| *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>
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| *PASD +ve (cytoplasmic).
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| ===IHC===
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| *TFE3 +ve -- suggestive of characteristic translocation.
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| ===Molecular===
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| *t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
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| Note:
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| *Same translocation may be seen in ''[[renal tumour with Xp11.2 translocation]]''.
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| ===EM===
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| *Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.<ref name=pmid17071801/>
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| Image:
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| *[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig8/ Distinctive crystal lattice in ASPS (nlm.nih.gov)].<ref name=pmid17071801/>
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| ==Desmoplastic small round cell tumour== | | ==Desmoplastic small round cell tumour== |
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| =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===
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| *Soft tissue manifestation of acute myeloid leukemia.<ref name=pmid21556238/>
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| ===Microscopic===
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| Features:
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| *Cluster of atypical small blue cells in [[soft tissue lesions|soft tissue]].
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| Note:
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| *May mimic [[small cell carcinoma]], large cell lymphomas ([[DLBCL]], [[ALCL]]), [[small round cell tumour]]s.
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| ====Images====
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| <gallery>
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| Image:Chloroma_-_high_mag.jpg | Chloroma - high mag. (WC)
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| Image:Chloroma_-_very_high_mag.jpg | Chloroma - very high mag. (WC)
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| </gallery>
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| www:
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| *[http://path.upmc.edu/cases/case306/micro.html Granulocytic sarcoma - several crappy images (upmc.edu)].
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| *[http://path.upmc.edu/cases/case379.html Myeloid sarcoma - several images (upmc.edu)].
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| =See also= | | =See also= |