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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.
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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===
*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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=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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