|
|
(180 intermediate revisions by 2 users not shown) |
Line 1: |
Line 1: |
| '''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= |
| ==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). |
Line 24: |
Line 27: |
| *Once upon a time almost everything was called ''malignant fibrous histiocytoma''; thus, it is listed as a common entity in some publications. | | *Once upon a time almost everything was called ''malignant fibrous histiocytoma''; thus, it is listed as a common entity in some publications. |
|
| |
|
| ===Most common:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url= http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref>===
| | Most common:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url= http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref> |
| *Liposarcoma. | | *Liposarcoma. |
| *Leiomyosarcoma. | | *Leiomyosarcoma. |
Line 40: |
Line 43: |
| *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]]. |
|
| |
|
| =Adipocytic tumours= | | ==Morphohistologic patterns== |
| {{Main|Adipocytic tumours}} | | {{Main|Morphologic patterns}} |
| | {| class="wikitable sortable" style="margin-left:auto;margin-right:auto" |
| | ! Name |
| | ! Description |
| | ! DDx |
| | ! Image(s) |
| | |- |
| | | 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 |
| | | [[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:Storiform_pattern_-_intermed_mag.jpg |thumb|center|150px| Patternless pattern. (WC)]] |
| | |- |
| | | 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:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg |thumb|center|150px |Herring bone. (WC)]] |
| | |- |
| | | Fascicular |
| | | the long axis of the (spindle) cells are perpendicular to one another in adjacent bundles of cells |
| | | [[leiomyoma]], [[leiomyosarcoma]] |
| | | [[Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg |thumb|center|150px| Fascicular pattern. (WC)]] |
| | |- |
| | | Biphasic |
| | | nests of cells and stroma |
| | | [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]] |
| | | [[Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg|thumb|center|150px| DSRCT. (WC)]] |
| | |- <!-- |
| | | name ? |
| | | description ? |
| | | DDx ? |
| | | image ? --> |
| | |} |
|
| |
|
| This category includes:
| | Notes: |
| *Lipoma. | | *Memory device: herring bone DDx ''MSF'' = MPNST, Synovial sarcoma, Fibrosarcoma. |
| *Liposarcoma.
| |
| *Hibernoma.
| |
|
| |
|
| =Smooth muscle tumours= | | ==Grading== |
| ==Leiomyosarcoma== | | *Several systems exist. |
| See gyne notes.
| | *The US-CAP advocates the use of the French system over the NCI system. |
| | **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> |
|
| |
|
| ===Microscopy=== | | ===French system=== |
| Features:
| | *Formally known as the grading system from the ''French Federation of Cancer Centres Sarcoma Group'' (FNCLCC). |
| *Nuclear atypia.
| |
| *[[Necrosis]].
| |
| *Mitoses. | |
|
| |
|
| =Fibroblastic/myofibroblastic tumours= | | ====Overview==== |
| | 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> |
| | #Differentiation (score 1-3). |
| | #*De facto, this is mostly the ''histologic type''. |
| | #Mitotic rate (score 1-3). |
| | #Necrosis (score 0-2) |
|
| |
|
| ==Proliferative fasciitis==
| | Obtaining a score: |
| *Need to write something here. | | *Add all the points from the three components. |
|
| |
|
| ==Solitary fibrous tumour==
| | Scoring: |
| ===General=== | | *Grade 1 = 2-3. |
| *Grouped with ''hemangiopericytoma'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
| | *Grade 2 = 4-5. |
| *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> | | *Grade 3 = 6-8. |
|
| |
|
| ===Microscopic=== | | =====Differentiation===== |
| Features:
| | *Standardized for histologic types. |
| *Well-circumscribed. | | *Most tumours = 3/3. |
| *Fibroblast-like cells (spindle cells). | |
| *Hemangiopericytoma-like area (staghorn vessels) - not seen on image.
| |
| *Keloid-like collagen bundles.
| |
|
| |
|
| Images:
| | Exceptions:<ref name=uscap_stp/> |
| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg SFT - low mag. (WC)]. | | *Well-differentiated liposarcoma = 1. |
| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg SFT - intermed. mag. (WC)]. | | *Myxoid liposarcoma = 2. |
| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg SFT - high mag. (WC)]. | | *Conventional [[liposarcoma]] = 2. |
| | *Fibrosarcoma = 2. |
| | *[[Myxofibrosarcoma]] =2. |
|
| |
|
| ==Hemangiopericytoma==
| | A group of tumours is not graded:<ref name=uscap_stp/> |
| ===General===
| | *[[MPNST]]. |
| *Grouped with ''solitary fibrous tumour'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
| | *[[Rhabdomyosarcoma]]. |
| *Arises from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation. | | *[[Alveolar soft part sarcoma]]. |
| *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> | | *[[Clear cell sarcoma]]. |
| *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> | | *[[Extraskeletal myxoid chondrosarcoma]]. |
|
| |
|
| ===Presentation=== | | =====Mitotic rate===== |
| *Usually painless mass, slow enlargement. | | *0-9 mitoses/10 HPF. |
| | *10-19 mitoses/10 HPF. |
| | *>=20 mitoses/10 HPF. |
|
| |
|
| ====Radiology====
| | Notes: |
| *Intramedullary lytic mass. | | *1 HPF = 0.1734 mm^2. |
| *May be well-circumscribed. | | **Most resident microscopes have a field of view = 0.2376 mm^2. |
| *+/-Periosteal reaction. | | ***Thus, ~7.3 HPFs on a resident microscope corresponds to 10 US-CAP HPFs. |
| *+/-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>
| | =====Necrosis===== |
| ===Location=== | | *None = score 0. |
| *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> | | *<=50% of tumour = score 1. |
| | *>50% of tumour = score 2. |
|
| |
|
| ===Histology=== | | ===System used by some at MSH=== |
| Features:<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
| | 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> |
| *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=== | | ====Scoring==== |
| Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref name=enzinger/>
| | *Grade 1 = 1 point. |
| *Vimentin +ve (usually). | | *Grade 2 = 2 points. |
| *Desmin -ve (typical). | | *Grade 3 = 3-4 points. |
| *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>
| | ====Components==== |
| *EMA -ve.
| | Points for each of the following: |
| *S100 -ve. | | *Mitotic activity >= 6 / 10 HPF @ 40X - definition suffers from [[HPFitis]]. |
| | *Pleomorphism present. |
| | *Cellularity (cells/matrix) > 50%. |
| | *Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly. |
|
| |
|
| ===DDx=== | | ==Stage== |
| *Other vascular tumours. | | {{Main|Cancer staging systems}} |
| *Vascular malformations. | | ===Lymph node metastases in sarcomas=== |
| *Synovial sarcoma. | | {{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. |
|
| |
|
| ==Desmoplastic fibroblastoma==
| | 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> |
| *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>
| | *[[rhabdomyosarcoma|'''R'''habdomyosarcoma]] |
| *Benign lesion. | | *[[Angiosarcoma|'''A'''ngiosarcoma]]. |
| *Classically found in shoulder region. | | *[[clear cell sarcoma|'''C'''lear cell sarcoma]]. |
| | *[[epithelioid sarcoma|'''E'''pitheliod sarcoma]]. |
| | *[[fibrosarcoma|'''F'''ibrosarcoma]]. |
| | *[[pleomorphic undifferentiated sarcoma|'''M'''alignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma)]]. |
| | *[[synovial sarcoma|'''S'''ynovial cell sarcoma]]. |
|
| |
|
| ===IHC=== | | ==DDx by history/site== |
| *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 }}
| | ===Retroperiteum=== |
| </ref>
| | #[[Liposarcoma]]. |
| **Significance ???
| | #[[Undifferentiated pleomorphic sarcoma]]. |
| | #[[Leiomyosarcoma]]. |
| | #[[MPNST]]. |
|
| |
|
| ==Low-grade fibromyxoid sarcoma==
| | Note: |
| *[[AKA]] ''hyalinizing spindle cell tumour''.
| | [[Synovial sarcoma]] and [[fibrosarcoma]] are very rare in the retroperitoneum. |
| ===General===
| |
| *Deep soft tissue.
| |
|
| |
|
| ===Microscopic=== | | ===Young person - extremity sarcoma=== |
| 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>
| | #[[Epithelioid sarcoma]]. |
| *Myoid stroma - '''key feature'''.
| | #[[Synovial sarcoma]]. |
| *Low cellularity.
| |
| *Spindle cells.
| |
|
| |
|
| Notes:
| | ==Gross characteristics== |
| *Few/absent mitoses. | | *Usually non-specific. |
| | *Most sarcomas have a pushing border. |
| | **If there is an infiltrative border think: (1) fibromatosis, (2) carcinoma. |
|
| |
|
| ===Molecular pathology=== | | =Adipocytic tumours= |
| 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>
| | {{Main|Adipocytic tumours}} |
|
| |
|
| =Vascular lesions=
| | This category includes: |
| Vascular lesions are "too red"; they have too many RBCs.
| | *Lipoma. |
| | *Liposarcoma. |
| | *Hibernoma. |
|
| |
|
| ==Hemangioma== | | =Smooth muscle tumours= |
| ===General===
| | {{Main|Smooth muscle tumours}} |
| 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>
| | IHC markers: desmin, SMA, H-caldemsin (most specific). |
| *Tufted.
| |
| **Small clusters of blood vessels.
| |
| *Microvenular hemangioma.
| |
| *Glomeruloid hemangioma.
| |
| *Epithelioid hemangioma.
| |
| *Targetoid hemosideric hemangioma.
| |
|
| |
|
| ===Microscopic=== | | ==Leiomyosarcoma== |
| Features:
| | {{Main|Leiomyosarcoma}} |
| *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=== | | ===Microscopic=== |
| Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>- '''key feature'''. | | Features (summary): |
| *+/-Nuclear atypia. | | *Fasicular cellular spindle cell lesion with: |
| *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'''. | | **Nuclear atypia. |
| *+/-Hemosiderin deposits. | | **[[Necrosis]]. |
| | **High mitotic rate. |
|
| |
|
| DDx:
| | =Fibrohistiocytic tumours= |
| *Angiosarcoma (have many mitoses, nuclear atypia).
| | ''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> |
| *Masson's hemangioma (Intravascular papillary endothelial hyperplasia).
| |
|
| |
|
| Notes:
| | ==Pleomorphic undifferentiated sarcoma== |
| *Hyaline globules have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/> | | *Abbreviated ''PUS''. |
| *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>
| | *[[AKA]] ''Undifferentiated pleomorphic sarcoma'', abbreviated ''UPS''. |
| **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> | | *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> |
| **Image: [http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].
| | {{Main|Pleomorphic undifferentiated sarcoma}} |
|
| |
|
| Images:
| | =Fibroblastic/myofibroblastic tumours= |
| *[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
| | {{Main|Fibroblastic/myofibroblastic tumours}} |
| *[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
| |
|
| |
|
| ===IHC===
| | This is a very large and important group of soft tissue lesions. It is covered in a separate article. |
| *CD31 +ve.
| |
| *CD34 +ve.
| |
| *HHV-8 +ve.
| |
|
| |
|
| ==Masson hemangioma==
| | The grouping includes: |
| ===General===
| | *[[Inflammatory myofibroblastic tumour]]. |
| *Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized. | | *[[Nodular fasciitis]]. |
| *[[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> | | *[[Desmoid-type fibromatosis]] (Desmoid tumour). |
| *Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.<ref name=pmid15865098/> | | *[[Proliferative fasciitis]]. |
| ===Microscopic===
| | *[[Solitary fibrous tumour]] ([[Hemangiopericytoma]]). |
| Features:
| | *[[Desmoplastic fibroblastoma]]. |
| *Well-circumscribed - '''key (low power) feature'''. | | *[[Low-grade fibromyxoid sarcoma]]. |
| *Abundant small vascular channels with benign endothelium. | | *Others. |
|
| |
|
| Notes:
| | =Perivascular tumours= |
| *Looks like Kaposi sarcoma at high power. | | This grouping includes only two:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref> |
| | *[[Glomus tumour]] - both benign and malignant. |
| | *[[Myopericytoma]]. |
|
| |
|
| ==Angiosarcoma== | | =Vascular lesions= |
| ===General===
| | {{Main|Vascular lesions}} |
| *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>
| | Vascular lesions are "too red"; they have too many RBCs. |
| *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===
| | They include: |
| Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>
| | *[[Hemangioma]]. |
| *Well-formed thin vascular channels on a fibrous stroma - '''key feature'''.
| | *[[Kaposi sarcoma]]. |
| *+/-Thrombosis. | | *[[Masson hemangioma]]. |
| *+/-Calcification. | | *[[Angiosarcoma]] |
| *+/-Fibrosis. | | *[[Epithelioid hemangioendothelioma]]. |
| *+/-Myxoid change. | |
| | |
| ===IHC===
| |
| *Factor VIII +ve. | |
|
| |
|
| =Skeletal muscle tumours= | | =Skeletal muscle tumours= |
Line 280: |
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]].
| | :Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''. |
| | {{Main|Epithelioid sarcoma}} |
|
| |
|
| ===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= |