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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> | |||
*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]]. | ||
== | ==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 | ||
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! DDx | ! DDx | ||
! Image(s) | ! 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> | | 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 | ||
| [[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> | | [[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 | | 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: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 | | the long axis of the (spindle) cells are perpendicular to one another in adjacent bundles of cells | ||
| [[leiomyoma]], [[leiomyosarcoma]] | | [[leiomyoma]], [[leiomyosarcoma]] | ||
| | | [[Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg |thumb|center|150px| Fascicular pattern. (WC)]] | ||
| | |||
|- | |- | ||
| Biphasic | | Biphasic | ||
| nests of cells and stroma | | nests of cells and stroma | ||
| [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]] | | [[synovial sarcoma]], [[DSRCT]], [[alveolar RMS]] | ||
| | | [[Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg|thumb|center|150px| DSRCT. (WC)]] | ||
| | |||
|- <!-- | |- <!-- | ||
| name ? | | name ? | ||
| description ? | | description ? | ||
| DDx ? | | DDx ? | ||
| image | | image ? --> | ||
|} | |} | ||
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===French system=== | ===French system=== | ||
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> | *Formally known as the grading system from the ''French Federation of Cancer Centres Sarcoma Group'' (FNCLCC). | ||
====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). | #Differentiation (score 1-3). | ||
#*De facto, this is mostly the ''histologic type''. | #*De facto, this is mostly the ''histologic type''. | ||
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*Well-differentiated liposarcoma = 1. | *Well-differentiated liposarcoma = 1. | ||
*Myxoid liposarcoma = 2. | *Myxoid liposarcoma = 2. | ||
*Conventional liposarcoma = 2. | *Conventional [[liposarcoma]] = 2. | ||
*Fibrosarcoma = 2. | *Fibrosarcoma = 2. | ||
*Myxofibrosarcoma =2. | *[[Myxofibrosarcoma]] =2. | ||
A group of tumours is not graded:<ref name=uscap_stp/> | A group of tumours is not graded:<ref name=uscap_stp/> | ||
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*[[Alveolar soft part sarcoma]]. | *[[Alveolar soft part sarcoma]]. | ||
*[[Clear cell sarcoma]]. | *[[Clear cell sarcoma]]. | ||
*Extraskeletal myxoid chondrosarcoma. | *[[Extraskeletal myxoid chondrosarcoma]]. | ||
=====Mitotic rate===== | =====Mitotic rate===== | ||
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*Cellularity (cells/matrix) > 50%. | *Cellularity (cells/matrix) > 50%. | ||
*Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly. | *Necrosis >15% - microscopic (without targeting necrosis grossly) ''or'' grossly. | ||
==Stage== | |||
{{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. | |||
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> | |||
*[[rhabdomyosarcoma|'''R'''habdomyosarcoma]] | |||
*[[Angiosarcoma|'''A'''ngiosarcoma]]. | |||
*[[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]]. | |||
==DDx by history/site== | |||
===Retroperiteum=== | |||
#[[Liposarcoma]]. | |||
#[[Undifferentiated pleomorphic sarcoma]]. | |||
#[[Leiomyosarcoma]]. | |||
#[[MPNST]]. | |||
Note: | |||
[[Synovial sarcoma]] and [[fibrosarcoma]] are very rare in the retroperitoneum. | |||
===Young person - extremity sarcoma=== | |||
#[[Epithelioid sarcoma]]. | |||
#[[Synovial sarcoma]]. | |||
==Gross characteristics== | |||
*Usually non-specific. | |||
*Most sarcomas have a pushing border. | |||
**If there is an infiltrative border think: (1) fibromatosis, (2) carcinoma. | |||
=Adipocytic tumours= | =Adipocytic tumours= | ||
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=Smooth muscle tumours= | =Smooth muscle tumours= | ||
{{Main|Smooth muscle tumours}} | |||
IHC markers: desmin, SMA, H-caldemsin (most specific). | |||
==Leiomyosarcoma== | ==Leiomyosarcoma== | ||
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=Fibrohistiocytic tumours= | =Fibrohistiocytic tumours= | ||
''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> | |||
==Pleomorphic undifferentiated sarcoma== | ==Pleomorphic undifferentiated sarcoma== | ||
*Abbreviated ''PUS''. | *Abbreviated ''PUS''. | ||
*[[AKA]] ''Undifferentiated pleomorphic sarcoma''. | *[[AKA]] ''Undifferentiated pleomorphic sarcoma'', abbreviated ''UPS''. | ||
*Previously known as ''malignant fibrous histiocytoma'', abbreviated ''MFH''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref> | *Previously known as ''malignant fibrous histiocytoma'', abbreviated ''MFH''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref> | ||
{{Main|Pleomorphic undifferentiated sarcoma}} | |||
=Fibroblastic/myofibroblastic tumours= | =Fibroblastic/myofibroblastic tumours= | ||
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*[[Desmoplastic fibroblastoma]]. | *[[Desmoplastic fibroblastoma]]. | ||
*[[Low-grade fibromyxoid sarcoma]]. | *[[Low-grade fibromyxoid sarcoma]]. | ||
*Others. | |||
=Perivascular tumours= | =Perivascular tumours= | ||
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=Tumours of uncertain differentiation= | =Tumours of uncertain differentiation= | ||
== | ==Angiomatoid fibrous histiocytoma== | ||
{{Main|Angiomatoid fibrous histiocytoma}} | |||
== | |||
* | ==Aggressive angiomyxoma== | ||
*[[AKA]] deep aggressive angiomyxoma. | |||
{{Main|Aggressive angiomyxoma}} | |||
== | ==Angiomyofibroblastoma== | ||
{{Main|Angiomyofibroblastoma}} | |||
== | ==Extrarenal malignant rhabdoid tumour== | ||
* | *Essentially identical to ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref> | ||
{{Main|Extrarenal malignant rhabdoid tumour}} | |||
==Ewing sarcoma/PNET== | ==Ewing sarcoma/PNET== | ||
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==Epithelioid sarcoma== | ==Epithelioid sarcoma== | ||
:Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''. | |||
{{Main|Epithelioid sarcoma}} | |||
==Alveolar soft part sarcoma== | ==Alveolar soft part sarcoma== | ||
{{Main|Alveolar soft part sarcoma}} | |||
==Desmoplastic small round cell tumour== | ==Desmoplastic small round cell tumour== | ||
{{Main|Desmoplastic small round cell tumour}} | |||
==Clear cell sarcoma== | ==Clear cell sarcoma== | ||
{{Main|Clear cell sarcoma}} | |||
==Synovial sarcoma== | ==Synovial sarcoma== | ||
{{Main|Synovial sarcoma}} | |||
=Other= | =Other= | ||
==Granulocytic sarcoma== | ==Granulocytic sarcoma== | ||
* | *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= |
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