Difference between pages "Soft tissue lesions" and "Alveolar soft part sarcoma"

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'''Soft tissue lesions''' strike fear in many pathologists as they are uncommon and may be difficult to diagnose.  
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg
| Width      =
| Caption    = ASPS. [[H&E stain]].
| Micro      = large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nests/separated by thin septa - vaguely resembles alveoli (at low power)
| Subtypes  =
| LMDDx      = [[paraganglioma]], [[clear cell renal cell carcinoma]], [[alveolar rhabdomyosarcoma]]
| Stains    = PAS +ve (cytoplasm)
| IHC        = TFE3 +ve (suggestive of translocation)
| EM        = intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart
| Molecular  = t(X;17)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - usu. [[head and neck pathology|head and neck]]
| Assdx      =
| Syndromes  =
| Clinicalhx = usu. slow growing mass
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = ultimately poor
| Other      =
| ClinDDx    =
}}
'''Alveolar soft part sarcoma''', abbreviated '''ASPS''', is a rare [[malignant]] soft tissue lesion typically seen in younger individuals.


=Introduction=
==General==
==WHO classification of soft tissue lesions/tumours==
===Morphologic grouping<ref name=Ref_WMSP601-3>{{Ref WMSP|601-3}}</ref>===
#Adipocytic tumours.
#Fibroblastic/myofibroblastic tumours.
#"Fibrohistiocytic" tumours.
#Smooth muscle tumours.
#Skeletal muscle tumours.
#Vascular tumours.
#Perivascular (pericytic) tumours.
#Chondro-osseous tumours.
#Tumours of uncertain differentiation.
 
===Biologic potential grouping<ref>{{Ref WMSP|598-604}}</ref>===
#Benign.
#Intermediate (locally aggressive).
#Intermediate (rarely metastasizing).
#Malignant.
 
==Prevalence==
*All sarcomas are rare buggers. 
**As the classification has been changing over the past years (with more subtypes being recognized/identified) numbers are variable from study-to-study.
*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>===
*Liposarcoma.
*Leiomyosarcoma.
 
==Molecular testing==
{{Main|Molecular pathology}}
*Molecular testing plays an important role in soft tissue pathology.
*It is generally seen as an adjunct test that:<ref name=pmid11454050>{{cite journal |author=Fletcher CD, Fletcher JA, Dal Cin P, Ladanyi M, Woodruff JM |title=Diagnostic gold standard for soft tissue tumours: morphology or molecular genetics? |journal=Histopathology |volume=39 |issue=1 |pages=100–3 |year=2001 |month=July |pmid=11454050 |doi= |url=}}</ref>
**Often is used to confirm the histomorphologic impression/quality control.
**Frequently has some prognostic significance.
**May directly affect treatment.
 
===Translocations===
{{Main|Chromosomal translocations}}
*Many tumours in soft tissue pathology are diagnosed inconjunction with the finding of [[chromosomal translocations]].
 
==Histologic patterns==
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Name
! Description
! DDx
! Image
! 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>
| whorled, cartwheel-like arrangement
| [[Undifferentiated pleomorphic sarcoma]]
| image ?
| other ?
|-
| 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
| [[fibrosarcoma]], [[synovial sarcoma]], [[MPNST]]
| image ?
| other ?
|-
| Fasicular
| the long axis of the (spindle) cells are perpendicular to one another in adjacent bundles of cells
| [[leiomyoma]]
| image ?
| other ?
|-
| Biphasic
| nests of cells and stroma
| [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]]
| image ?
| other ?
|- <!--
| name ?
| description ?
| DDx ?
| image ?
| other ? -->
|}
 
=Adipocytic tumours=
{{Main|Adipocytic tumours}}
 
This category includes:
*Lipoma.
*Liposarcoma.
*Hibernoma.
 
=Smooth muscle tumours=
==Leiomyosarcoma==
See gyne notes.
 
===Microscopy===
Features:
*Nuclear atypia.
*[[Necrosis]].
*Mitoses.
 
=Fibrohistiocytic tumours=
==Pleomorphic undifferentiated sarcoma==
*Abbreviated ''PUS''.
*[[AKA]] ''Undifferentiated pleomorphic sarcoma''.
*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>
 
===General===
*Common sarcoma.
*Usu. deep tissue of the trunk and extremities.
 
===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.
 
Other findings:
*+/-Giant cells (see subclassification).
*+/-Inflammation (see subclassification).
**Neutrophils.
**Eosinophils.
 
====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.
 
=Fibroblastic/myofibroblastic tumours=
 
==Proliferative fasciitis==
*Need to write something here.
 
==Solitary fibrous tumour==
===General===
*Grouped with ''hemangiopericytoma'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*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>
 
===Microscopic===
Features:
*Well-circumscribed.
*Fibroblast-like cells (spindle cells).
*Hemangiopericytoma-like area (staghorn vessels) - not seen on image.
*Keloid-like collagen bundles.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg SFT - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg SFT - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg SFT - high mag. (WC)].
 
===IHC===
*CD34 ~90% +ve.
*CD99 ~70% +ve.
*BCL2 ~50% +ve.
 
==Hemangiopericytoma==
===General===
*Grouped with ''solitary fibrous tumour'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Arises from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation.
*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>
*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>
 
===Presentation===
*Usually painless mass, slow enlargement.
 
====Radiology====
*Intramedullary lytic mass.
*May be well-circumscribed.
*+/-Periosteal reaction.
*+/-Sclerotic border.
 
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>
===Location===
*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>
 
===Histology===
Features:<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
*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===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref name=enzinger/>
*Vimentin +ve (usually).
*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>
*EMA -ve.
*S100 -ve.
 
===DDx===
*Other vascular tumours.
*Vascular malformations.
*Synovial sarcoma.
 
==Desmoplastic fibroblastoma==
*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>
*Benign lesion.
*Classically found in shoulder region.
 
===IHC===
*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 }}
</ref>
**Significance ???
 
==Low-grade fibromyxoid sarcoma==
*[[AKA]] ''hyalinizing spindle cell tumour''.
===General===
*Deep soft tissue.
 
===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>
*Myoid stroma - '''key feature'''.
*Low cellularity.
*Spindle cells.
 
Notes:
*Few/absent mitoses.
 
===Molecular pathology===
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>
 
=Vascular lesions=
Vascular lesions are "too red"; they have too many RBCs.
 
==Hemangioma==
===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 - associated with [[POEMS syndrome]].
*Epithelioid hemangioma.
*Targetoid hemosideric hemangioma.
 
===Microscopic===
Features:
*Abundance of benign small blood vessels. (???)
 
==Kaposi sarcoma==
===General===
*Not really a sarcoma.
*Caused by HHV-8.
*Associated with immunodeficiency, e.g. [[HIV]]/AIDS.
 
===Stages===
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>
#Patch stage.
#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:
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
 
===IHC===
*CD31 +ve.
*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=
==Rhabdomyoma==
{{Main|Rhabdomyoma}}
 
==Rhabdomyosarcoma==
*Abbreviated ''RMS''.
 
{{Main|Rhabdomyosarcoma}}
Comes it two main flavours:
*Alveolar rhabdomyosarcoma.
*Embryonal rhabdomyosarcoma.
 
The histology may be that of a [[small round cell tumour]].
 
=Chondro-osseous tumours=
{{Main|Chondro-osseous tumours}}
 
This grouping includes tumours derived from [[cartilage]] and [[bone]].
 
=Tumours of uncertain differentiation=
==Alveolar soft part sarcoma==
*Abbreviated ''ASPS''.
===General===
*Adolescents/young adults.
*Adolescents/young adults.
*Children -- classically location: base of tongue and orbit.
*Children -- classically location: orbit and base of [[tongue]].<ref name=pmid21934227>{{Cite journal  | last1 = Anbarasi | first1 = K. | last2 = Sathasivasubramanian | first2 = S. | last3 = Kuruvilla | first3 = S. | title = Alveolar soft-part sarcoma of tongue. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 3 | pages = 581-3 | month =  | year =  | doi = 10.4103/0377-4929.85099 | PMID = 21934227 }}</ref>
*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===
==Microscopic==
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power).
*Arranged in nests/separated by thin septa; vaguely resembles alveoli (at low power).
*Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
*Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
**May be focally cleared.
*An eccentric nucleus.
*An eccentric nucleus.
*+/-Nucleolus.
*+/-Nucleolus, prominent.
*+/-Multi-nucleation (common).


Images:
DDx:
*[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-_low_mag.jpg ASPS - low mag. (WC)].
*[[Paraganglioma]].
*[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-_intermed_mag.jpg ASPS - intermed. mag. (WC)].
*[[Clear cell renal cell carcinoma]] - areas with cytoplasmic clearing.
*[http://commons.wikimedia.org/wiki/File:Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg ASPS - very high mag. (WC)].
*[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells, no cross striations.


===Molecular===
===Images===
*t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
<gallery>
Image:Alveolar soft part sarcoma - very low mag.jpg | ASPS - very low mag. (WC/Nephron)
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 - high mag.jpg | ASPS - high mag. (WC/Nephron)
Image:Alveolar soft part sarcoma - very high mag.jpg | ASPS - very high 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)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig1/ ASPS - low mag. (nih.gov)].<ref name=pmid17071801/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig5/ ASPS - high mag. (nih.gov)].<ref name=pmid17071801/>


==Desmoplastic small round cell tumour==
==Stains==
*Abbreviated ''DSRCT''.
*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>
===General===
*PASD +ve (cytoplasmic).
*Males > females.
*Usu. affects young adults.
*Typically retroperitoneal.
*Poor prognosis.


===Microscopic===
==IHC==
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>
*TFE3 +ve -- suggestive of characteristic translocation.
#Broad bands of paucicellular fibrous stroma with:
*Desmin ~ 50% of cases.<ref name=pmid17071801/>
#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:
Others:<ref name=pmid17071801/>
*Usu. abundant mitoses.
*EMA -ve.
*+/-Necrosis.
*Cytokeratins -ve.
*HMB45 -ve.
*Melan‐A -ve.
*Chromogranin A -ve.
*Synaptophysin -ve.


Images:
==Molecular==
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg DSRCT - intermed. mag. (WC)].
*t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg DSRCT - very high mag. (WC)].
 
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!
 
===IHC===
Features:
*AE1/AE3 +ve.
*Desmin +ve.
*EMA +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==
*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>
**Molecular changes and origin distinct from melanoma.
*Incidence: rare soft tissue tumour.


===Clinical===
Note:
*Usually - deep soft tissue ''or'' extremities.
*Same translocation may be seen in ''[[renal tumour with Xp11.2 translocation]]''.
*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===
==EM==
Features:<ref name=pmid18300804/>
*Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.<ref name=pmid17071801/>
*Architecture: sheets or fascicular (bundles) arrangement.
*Cells: Spindle cells or epithelioid cells.
*Prominent nucleoli - basophilic.
*Fibrous septae.
*Uniform


Image:
Image:
*[http://commons.wikimedia.org/wiki/File:Clear_cell_sarcoma.Image12.jpg Clear cell sarcoma (WC)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig8/ Distinctive crystal lattice in ASPS (nlm.nih.gov)].<ref name=pmid17071801/>
*[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==
===General===
*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===
Unique 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>


=See also=
==See also==
*[[Bone]].
*[[Soft tissue lesions]].
*[[Dermatopathology]].
*[[Head and neck pathology]].
*[[Hematopathology]].
*[[Spindle cell lesion]].
*[[Neurofibromatosis]].
*[[Small round cell tumours]].


=References=
==References==
{{reflist|2}}
{{Reflist|2}}


[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
[[Category:Diagnosis]]

Latest revision as of 16:40, 25 December 2014

Alveolar soft part sarcoma
Diagnosis in short

ASPS. H&E stain.
LM large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nests/separated by thin septa - vaguely resembles alveoli (at low power)
LM DDx paraganglioma, clear cell renal cell carcinoma, alveolar rhabdomyosarcoma
Stains PAS +ve (cytoplasm)
IHC TFE3 +ve (suggestive of translocation)
EM intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart
Molecular t(X;17)
Site soft tissue - usu. head and neck
Clinical history usu. slow growing mass
Prevalence rare
Prognosis ultimately poor

Alveolar soft part sarcoma, abbreviated ASPS, is a rare malignant soft tissue lesion typically seen in younger individuals.

General

  • Adolescents/young adults.
  • Children -- classically location: orbit and base of tongue.[1]
  • Typical indolent initially - ultimately a poor prognosis.[2]

Microscopic

Features:[3]

  • Arranged in nests/separated by thin septa; vaguely resembles alveoli (at low power).
  • Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
    • May be focally cleared.
  • An eccentric nucleus.
  • +/-Nucleolus, prominent.
  • +/-Multi-nucleation (common).

DDx:

Images

  • ASPS - very low mag. (WC/Nephron)

  • ASPS - low mag. (WC/Nephron)

  • ASPS - intermed. mag. (WC/Nephron)

  • ASPS - high mag. (WC/Nephron)

  • ASPS - very high mag. (WC/Nephron)

  • ASPS - very high mag. (WC/Nephron)

www:

Stains

  • PAS +ve (cytoplasmic) - considered the most useful.[4]
  • PASD +ve (cytoplasmic).

IHC

  • TFE3 +ve -- suggestive of characteristic translocation.
  • Desmin ~ 50% of cases.[2]

Others:[2]

  • EMA -ve.
  • Cytokeratins -ve.
  • HMB45 -ve.
  • Melan‐A -ve.
  • Chromogranin A -ve.
  • Synaptophysin -ve.

Molecular

  • t(X;17)(p11.2;q25).[5]

Note:

EM

  • Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.[2]

Image:

See also

References

  1. ↑ Anbarasi, K.; Sathasivasubramanian, S.; Kuruvilla, S.. "Alveolar soft-part sarcoma of tongue.". Indian J Pathol Microbiol 54 (3): 581-3. doi:10.4103/0377-4929.85099. PMID 21934227.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Folpe, AL.; Deyrup, AT. (Nov 2006). "Alveolar soft-part sarcoma: a review and update.". J Clin Pathol 59 (11): 1127-32. doi:10.1136/jcp.2005.031120. PMC 1860509. PMID 17071801. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/.
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 627. ISBN 978-0781765275.
  4. ↑ Zarrin-Khameh, N.; Kaye, KS. (Mar 2007). "Alveolar soft part sarcoma.". Arch Pathol Lab Med 131 (3): 488-91. doi:10.1043/1543-2165(2007)131[488:ASPS]2.0.CO;2. PMID 17516754.
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606243
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