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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. 


=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===
#Adipocytic tumours.
These include:<ref name=Ref_WMSP601-3>{{Ref WMSP|601-3}}</ref>
#Fibroblastic/myofibroblastic tumours.
#[[Adipocytic tumours]].
#"Fibrohistiocytic" tumours.
#[[Fibroblastic/myofibroblastic tumours]].
#Smooth muscle tumours.
#[[Fibrohistiocytic tumours|"Fibrohistiocytic" tumours]].
#Skeletal muscle tumours.
#[[Smooth muscle tumours|Smooth muscle tumours]].
#Vascular tumours.
#[[Soft tissue lesions#Skeletal muscle tumours|Skeletal muscle tumours]].
#Perivascular (pericytic) tumours.
#[[Vascular tumours]].
#Chondro-osseous tumours.
#[[Soft_tissue_lesions#Perivascular_tumours|Perivascular (pericytic) tumours]].
#Tumours of uncertain differentiation.
#[[Chondro-osseous tumours]].
#[[Soft tissue lesions#Tumours of uncertain differentiation|Tumours of uncertain differentiation]].


===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.


===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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*Many tumours in soft tissue pathology are diagnosed inconjunction with the finding of [[chromosomal translocations]].
*Many tumours in soft tissue pathology are diagnosed inconjunction with the finding of [[chromosomal translocations]].


==Histologic patterns==
==Morphohistologic 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
! Description
! Description
! DDx
! DDx
! Image
! 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
| [[Undifferentiated pleomorphic sarcoma]]
| [[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>
| image ?
| [[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]]
| image ?
| [[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]]
| [[leiomyoma]], [[leiomyosarcoma]]
| image ?
| [[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]]
| image ?
| [[Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg|thumb|center|150px| DSRCT. (WC)]]
| other ?
|- <!--
|- <!--
| name ?
| name ?
| description ?
| description ?
| DDx ?
| DDx ?
| image ?
| image ? -->
| other ? -->
|}
|}


=Adipocytic tumours=
Notes:
{{Main|Adipocytic tumours}}
*Memory device: herring bone DDx ''MSF'' = MPNST, Synovial sarcoma, Fibrosarcoma.


This category includes:
==Grading==
*Lipoma.
*Several systems exist.
*Liposarcoma.
*The US-CAP advocates the use of the French system over the NCI system.
*Hibernoma.
**The French system is a better predictor metastases and mortality.<ref name=pmid8996162>{{cite journal |author=Guillou L, Coindre JM, Bonichon F, ''et al.'' |title=Comparative study of the National Cancer Institute and French Federation of Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma |journal=J. Clin. Oncol. |volume=15 |issue=1 |pages=350–62 |year=1997 |month=January |pmid=8996162 |doi= |url=}}</ref>


=Smooth muscle tumours=
===French system===
==Leiomyosarcoma==
*Formally known as the grading system from the ''French Federation of Cancer Centres Sarcoma Group'' (FNCLCC).
See gyne notes.


===Microscopy===
====Overview====
Features:
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>
*Nuclear atypia.
#Differentiation (score 1-3).
*[[Necrosis]].
#*De facto, this is mostly the ''histologic type''.
*Mitoses.
#Mitotic rate (score 1-3).
#Necrosis (score 0-2)


=Fibrohistiocytic tumours=
Obtaining a score:
==Undifferentiated pleomorphic sarcoma==
*Add all the points from the three components.
*Abbreviated ''UPS''.
*Previously known as ''malignant fibrous histiocytoma''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref>


===General===
Scoring:
*Common sarcoma.
*Grade 1 = 2-3.
*Usu. deep tissue of the trunk and extremities.
*Grade 2 = 4-5.
*Grade 3 = 6-8.


===Microscopic===
=====Differentiation=====
Features:<ref name=Ref_WMSP_613>{{Ref WMSP|613}}</ref>
*Standardized for histologic types.
*Storiform pattern ([[AKA]] patternless pattern - '''key feature'''.
*Most tumours = 3/3.
*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.


Other findings:
Exceptions:<ref name=uscap_stp/>
*+/-Giant cells (see subclassification).
*Well-differentiated liposarcoma = 1.
*+/-Inflammation (see subclassification).
*Myxoid liposarcoma = 2.
**Neutrophils.
*Conventional [[liposarcoma]] = 2.
**Eosinophils.
*Fibrosarcoma = 2.
*[[Myxofibrosarcoma]] =2.


====Subclassification====
A group of tumours is not graded:<ref name=uscap_stp/>
UPS is subclassified the following way:<ref name=Ref_WMSP_613-4>{{Ref WMSP|613-4}}</ref>
*[[MPNST]].
*UPS with giant cells.
*[[Rhabdomyosarcoma]].
*UPS with inflammation.
*[[Alveolar soft part sarcoma]].
*UPS not otherwise specified (NOS) - wastebasket diagnosis; if neither of the above two apply.
*[[Clear cell sarcoma]].
*[[Extraskeletal myxoid chondrosarcoma]].


=Fibroblastic/myofibroblastic tumours=
=====Mitotic rate=====
*0-9 mitoses/10 HPF.
*10-19 mitoses/10 HPF.
*>=20 mitoses/10 HPF.


==Proliferative fasciitis==
Notes:
*Need to write something here.
*1 HPF = 0.1734 mm^2.
**Most resident microscopes have a field of view = 0.2376 mm^2.
***Thus, ~7.3 HPFs on a resident microscope corresponds to 10 US-CAP HPFs.


==Solitary fibrous tumour==
=====Necrosis=====
===General===
*None = score 0.
*Grouped with ''hemangiopericytoma'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*<=50% of tumour = score 1.
*May be benign ''or'' malignant; more commonly benign.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9]. Accessed on: 25 June 2010.</ref><ref>URL: [http://wjso.com/content/6/1/86 http://wjso.com/content/6/1/86]. Accessed on: 25 June 2010.</ref>
*>50% of tumour = score 2.


===Microscopic===
===System used by some at MSH===
Features:
Some pathologists at [[MSH]] use the system advocated by Costa et al..<ref name=pmid6692258>{{cite journal |author=Costa J, Wesley RA, Glatstein E, Rosenberg SA |title=The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases |journal=Cancer |volume=53 |issue=3 |pages=530–41 |year=1984 |month=February |pmid=6692258 |doi= |url=}}</ref>
*Well-circumscribed.
*Fibroblast-like cells (spindle cells).
*Hemangiopericytoma-like area (staghorn vessels) - not seen on image.
*Keloid-like collagen bundles.


Images:
====Scoring====
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg SFT - low mag. (WC)].
*Grade 1 = 1 point.
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg SFT - intermed. mag. (WC)].
*Grade 2 = 2 points.
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg SFT - high mag. (WC)].
*Grade 3 = 3-4 points.


==Hemangiopericytoma==
====Components====
===General===
Points for each of the following:
*Grouped with ''solitary fibrous tumour'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Mitotic activity >= 6 / 10 HPF @ 40X - definition suffers from [[HPFitis]].
*Arises from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation.
*Pleomorphism present.
*Hematologic spread most common - to lungs.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*Cellularity (cells/matrix) > 50%.
*Oncogenic osteomalacia - assoc. with hemangiopericytoma.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly.


===Presentation===
==Stage==
*Usually painless mass, slow enlargement.
{{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.


====Radiology====
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>
*Intramedullary lytic mass.
*[[rhabdomyosarcoma|'''R'''habdomyosarcoma]]
*May be well-circumscribed.
*[[Angiosarcoma|'''A'''ngiosarcoma]].
*+/-Periosteal reaction.
*[[clear cell sarcoma|'''C'''lear cell sarcoma]].
*+/-Sclerotic border.
*[[epithelioid sarcoma|'''E'''pitheliod sarcoma]].
*[[fibrosarcoma|'''F'''ibrosarcoma]].
*[[pleomorphic undifferentiated sarcoma|'''M'''alignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma)]].
*[[synovial sarcoma|'''S'''ynovial cell sarcoma]].


May be worked-up with angiography to distinguish from a [[vascular malformation]].<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
==DDx by history/site==
===Location===
===Retroperiteum===
*Usually extremities - femur or prox. tibial.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
#[[Liposarcoma]].
#[[Undifferentiated pleomorphic sarcoma]].
#[[Leiomyosarcoma]].
#[[MPNST]].


===Histology===
Note:
Features:<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
[[Synovial sarcoma]] and [[fibrosarcoma]] are very rare in the retroperitoneum.
*Hypervascular lesion - '''key diagnostic feature'''.<ref name=enzinger>Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.</ref>
**Abundant thin-walled branching small vessels of variable size.
***May be described as "staghorn vessels" or "antler-like" vasculature.
***Cells may "onion-skin" around thin blood vessels.
*Spindle or ovoid shaped cells in nests or sheets.


===IHC===
===Young person - extremity sarcoma===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref name=enzinger/>
#[[Epithelioid sarcoma]].
*Vimentin +ve (usually).
#[[Synovial sarcoma]].
*Desmin -ve (typical).
*Factor VIII -ve (marks endothelium).
*CD34 +ve.
**CD34 usu. -ve in synovial sarcoma.
*CD31 -ve (marks benign endothelium).
*vWF (von Willebrand factor) -ve.


May be in the DDx for [[meningioma]]:<ref>Croul, SE. 8 November 2010.</ref>
==Gross characteristics==
*EMA -ve.
*Usually non-specific.
*S100 -ve.
*Most sarcomas have a pushing border.
**If there is an infiltrative border think: (1) fibromatosis, (2) carcinoma.


===DDx===
=Adipocytic tumours=
*Other vascular tumours.
{{Main|Adipocytic tumours}}
*Vascular malformations.
*Synovial sarcoma.


==Desmoplastic fibroblastoma==
This category includes:
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal  | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
*Lipoma.
*Benign lesion.
*Liposarcoma.
*Classically found in shoulder region.
*Hibernoma.


===IHC===
=Smooth muscle tumours=
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal  | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue =  | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
{{Main|Smooth muscle tumours}}
</ref>
IHC markers: desmin, SMA, H-caldemsin (most specific).
**Significance ???


==Low-grade fibromyxoid sarcoma==
==Leiomyosarcoma==
*[[AKA]] ''hyalinizing spindle cell tumour''.
{{Main|Leiomyosarcoma}}
===General===
*Deep soft tissue.


===Microscopic===
===Microscopic===
Features:<ref>{{cite journal |author=Vernon SE, Bejarano PA |title=Low-grade fibromyxoid sarcoma: a brief review |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=9 |pages=1358–60 |year=2006 |month=September |pmid=16948525 |doi= |url=}}</ref>
Features (summary):
*Myoid stroma - '''key feature'''.
*Fasicular cellular spindle cell lesion with:
*Low cellularity.
**Nuclear atypia.
*Spindle cells.
**[[Necrosis]].
**High mitotic rate.


Notes:
=Fibrohistiocytic tumours=
*Few/absent mitoses.
''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>


===Molecular pathology===
==Pleomorphic undifferentiated sarcoma==
t(7;16)(q33;p11)<ref>{{cite journal |author=Panagopoulos I, Storlazzi CT, Fletcher CD, ''et al.'' |title=The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma |journal=Genes Chromosomes Cancer |volume=40 |issue=3 |pages=218–28 |year=2004 |month=July |pmid=15139001 |doi=10.1002/gcc.20037 |url=}}</ref>
*Abbreviated ''PUS''.
*[[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>
{{Main|Pleomorphic undifferentiated sarcoma}}


=Vascular lesions=
=Fibroblastic/myofibroblastic tumours=
Vascular lesions are "too red"; they have too many RBCs.
{{Main|Fibroblastic/myofibroblastic tumours}}


==Hemangioma==
This is a very large and important group of soft tissue lesions. It is covered in a separate article.
===General===
Comes is various flavours:<ref name=pmid10410855>{{cite journal |author=Prieto VG, Shea CR |title=Selected cutaneous vascular neoplasms. A review |journal=Dermatol Clin |volume=17 |issue=3 |pages=507–20, viii |year=1999 |month=July |pmid=10410855 |doi= |url=}}</ref>
*Tufted.
**Small clusters of blood vessels.
*Microvenular hemangioma.
*Glomeruloid hemangioma.
*Epithelioid hemangioma.
*Targetoid hemosideric hemangioma.


===Microscopic===
The grouping includes:
Features:
*[[Inflammatory myofibroblastic tumour]].
*Abundance of benign small blood vessels. (???)
*[[Nodular fasciitis]].
*[[Desmoid-type fibromatosis]] (Desmoid tumour).
*[[Proliferative fasciitis]].
*[[Solitary fibrous tumour]] ([[Hemangiopericytoma]]).
*[[Desmoplastic fibroblastoma]].
*[[Low-grade fibromyxoid sarcoma]].
*Others.


==Kaposi sarcoma==
=Perivascular tumours=
===General===
This grouping includes only two:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref>
*Not really a sarcoma.
*[[Glomus tumour]] - both benign and malignant.
*Caused by HHV-8.
*[[Myopericytoma]].
*Associated with immunodeficiency, e.g. [[HIV]]/AIDS.


===Stages===
=Vascular lesions=
It is seen in different stages:<ref>URL: [http://www.histopathology-india.net/KS.htm http://www.histopathology-india.net/KS.htm]. Accessed on: 31 January 2010.</ref>
{{Main|Vascular lesions}}
#Patch stage.
Vascular lesions are "too red"; they have too many RBCs.
#Plaque stage.
#Nodular stage.
#Lymphangioma-like. (???)
 
===Microscopic===
Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>- '''key feature'''.
*+/-Nuclear atypia.
*Hyaline globules (intracytoplasmic)<ref name=pmid7528163>{{cite journal |author=del Rosario AD, Bui HX, Singh J, Ginsburg R, Ross JS |title=Intracytoplasmic eosinophilic hyaline globules in cartilaginous neoplasms: a surgical, pathological, ultrastructural, and electron probe x-ray microanalytic study |journal=Hum. Pathol. |volume=25 |issue=12 |pages=1283–9 |year=1994 |month=December |pmid=7528163 |doi= |url=}}</ref> - pale pink globs (that are paler than RBCs) - '''important feature'''.
*+/-Hemosiderin deposits.
 
DDx:
*Angiosarcoma (have many mitoses, nuclear atypia).
*Masson's hemangioma (Intravascular papillary endothelial hyperplasia).
 
Notes:
*Hyaline globules have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/>
*Promontory sign - small vessel protruding into an abnormal vascular space.<ref name=pmid19318797>{{cite journal |author=Lazova R, McNiff JM, Glusac EJ, Godic A |title=Promontory sign--present in patch and plaque stage of angiosarcoma! |journal=Am J Dermatopathol |volume=31 |issue=2 |pages=132–6 |year=2009 |month=April |pmid=19318797 |doi=10.1097/DAD.0b013e3181951045 |url=}}</ref>
**Not pathognomonic for KS.<ref name=pmid20577080>{{cite journal |author=Fernandez-Flores A, Rodriguez R |title=Promontory Sign in a Reactive Benign Vascular Proliferation |journal=Am J Dermatopathol |volume= |issue= |pages= |year=2010 |month=June |pmid=20577080 |doi=10.1097/DAD.0b013e3181cf0ae5 |url=}}</ref>
**Image: [http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].


Images:
They include:
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
*[[Hemangioma]].
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
*[[Kaposi sarcoma]].
 
*[[Masson hemangioma]].
===IHC===
*[[Angiosarcoma]]
*CD31 +ve.
*[[Epithelioid hemangioendothelioma]].
*CD34 +ve.
*HHV-8 +ve.
 
==Masson hemangioma==
===General===
*Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
*[[AKA]] ''intravascular papillary endothelial hyperplasia''.<ref name=pmid15865098>{{cite journal |author=Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma |journal=Anticancer Res. |volume=25 |issue=2B |pages=1409–12 |year=2005 |pmid=15865098 |doi= |url=}}</ref>
*Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.<ref name=pmid15865098/>
===Microscopic===
Features:
*Well-circumscribed - '''key (low power) feature'''.
*Abundant small vascular channels with benign endothelium.
 
Notes:
*Looks like Kaposi sarcoma at high power.
 
==Angiosarcoma==
===General===
*Malignant tumour - with a horrible prognosis.<ref name=pmid20537949>{{cite journal |author=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol |volume= |issue= |pages= |year=2010 |month=May |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Classically on the scalp or head & neck.
*May arise secondary to therapeutic radiation or chronic lymphoedema related to breast carcinoma.
 
===Microscopic===
Features:
*Very many small capillaries of irregular shape lined with:
**Pleomorphic nuclei.
***May have hobnail morphology.
*Mitoses.
*Cytoplasmic vacuoles.
**Cells trying to form lumina - embryologic.
 
Notes:
*Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble ''[[melanoma]]'' or ''[[hepatocellular carcinoma]]''.
 
===IHC===
*CD34 +ve.
*D2-40 +ve. (???)
*CD31 +ve.
 
==Hemangioendothelioma==
===General===
*Usually benign.
 
===Microscopic===
Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>
*Well-formed thin vascular channels on a fibrous stroma - '''key feature'''.
*+/-Thrombosis.
*+/-Calcification.
*+/-Fibrosis.
*+/-Myxoid change.
 
===IHC===
*Factor VIII +ve.


=Skeletal muscle tumours=
=Skeletal muscle tumours=
Line 350: Line 270:


=Tumours of uncertain differentiation=
=Tumours of uncertain differentiation=
==Desmoplastic small round cell tumour==
==Angiomatoid fibrous histiocytoma==
*Abbreviated ''DSRCT''.
{{Main|Angiomatoid fibrous histiocytoma}}
===General===
 
*Males > females.
==Aggressive angiomyxoma==
*Usu. affects young adults.
*[[AKA]] deep aggressive angiomyxoma.
*Typically retroperitoneal.
{{Main|Aggressive angiomyxoma}}
*Poor prognosis.


===Microscopic===
==Angiomyofibroblastoma==
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>
{{Main|Angiomyofibroblastoma}}
#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:
==Extrarenal malignant rhabdoid tumour==
*Usu. abundant mitoses.
*Essentially identical to ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*+/-Necrosis.
{{Main|Extrarenal malignant rhabdoid tumour}}


Images:
==Ewing sarcoma/PNET==
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg DSRCT - intermed. mag. (WC)].
{{Main|Ewing sarcoma}}
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg DSRCT - very high mag. (WC)].
*A [[small round blue cell tumour]] that may be seen in [[bone]]. It is discussed in the context of [[bone tumours]].


DDx:
==Epithelioid sarcoma==
*Metastatic [[germ cell tumour]] (DDx of location and age).
:Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''.
*[[Embryonal RMS]].
{{Main|Epithelioid sarcoma}}
**It should be noted that DSRCT, like embryonal RMS, is +ve for desmin!


===IHC===
==Alveolar soft part sarcoma==
Features:
{{Main|Alveolar soft part sarcoma}}
*AE1/AE3 +ve.
*Desmin +ve.
*EMA +ve.


===Molecular===
==Desmoplastic small round cell tumour==
*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>
{{Main|Desmoplastic small round cell tumour}}


==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.
*Fibrous septae.
*Uniform
 
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>
*Young adults or adolescents.
 
===Microscopic===
Comes in three 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.
#Primative round cell type.
 
Images:
*[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 + cytokeratin and/or EMA +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>


===Molecular pathology===
=Other=
Unique translocation:
==Granulocytic sarcoma==
*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>
*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.
{{Main|Granulocytic sarcoma}}


=See also=
=See also=
Michael
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