Difference between revisions of "Spindle cell lesions"

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'''Spindle cell lesions''' are seen frequent enough that one ought to have a solid approach to 'em.
[[Image:Malignant_peripheral_nerve_sheath_tumour_-_very_high_mag.jpg|thumb|right|250px|A spindle cell lesion ([[MPNST]]). [[H&E stain]].]]
'''[[Spindle cell]] lesions''' are seen frequent enough that one ought to have a solid approach to 'em.
 
A general introduction to ''spindle cells'' is found in the ''[[spindle cell]]'' article.


==General DDx==
==General DDx==
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**Skeletal.
**Skeletal.
*Other.
*Other.
**Carcinoma, e.g. metaplastic carcinoma.
===A long differential diagnosis of spindle cell tumours===
Adapted from ''Miller'' with modifications:<ref name=miller>Miller RT. (Sep 2013). ''Atlantic Diagnostic Immunohistochemistry Symposium''. St. John's, NL, Canada.</ref>
{| class="wikitable sortable"
! Tumour
! Group
! Notes/comments
|-
| [[Malignant melanoma]]
| melanoma
|-
| [[PEComa]]
| mesenchymal lesion
|[[angiomyolipoma]], [[lymphangioleiomyomatosis]], other PEComas
|-
| [[Sarcomatoid carcinoma]]
| carcinoma
|-
| [[Desmoplastic mesothelioma]]
| carcinoma
|-
| [[Thymoma]]
| epithelial lesion
|-
| [[Spindle epithelial tumor with thymus-like differentiation]]
| epithelial lesion
|-
| [[Malignant phyllodes tumour]]
| sarcoma
|-
| [[Chondroid syringoma]]
| epithelial lesion
|-
| [[Malignant mixed Muellerian tumour]]
| carcinoma
|-
| [[Syphilis]]
| infection
|-
| [[Bacillary angiomatosis]]
| infection
|-
| [[Myocbacterial spindle cell pseudotumour]]
| infection
|-
| [[Cryotococcal pseudotumour]]
| infection
|-
| [[Anaplastic large cell lymphoma]]
| lymphoid
|-
| [[Lymphocyte-depleted Hodgkin lymphoma]]
| lymphoid
|-
| [[Follicular dendritic cell sarcoma]]
| sarcoma
|-
| [[Interdigitating reticulum cell sarcoma]]
| sarcoma
|-
| [[Sclerosing extramedullary hematopoetic tumours]]
| other
|seen in chronic [[myeloproliferative disorders]]<ref name=pmid10632487>{{Cite journal| last1 = Remstein| first1 = ED.| last2 = Kurtin| first2 = PJ.| last3 = Nascimento| first3 = AG.| title = Sclerosing extramedullary hematopoietic tumor in chronic myeloproliferative disorders.| journal = Am J Surg Pathol| volume = 24| issue = 1| pages = 51-5| month = Jan| year = 2000| doi =| PMID = 10632487 }}</ref>
|-
| [[Juvenile xanthogranuloma]]
| histiocytic lesion
|-
| [[Granuloma annulare]]
| histiocytic lesion
|-
| [[Crystal-storing histiocytosis]]
| histiocytic lesion
|-
| [[Schwannoma]]
| neural tumours
|-
| [[Neurofibroma]]
| neural tumours
|-
| [[Malignant peripheral nerve sheath tumour]]
| neural tumours
|-
| [[Perineurioma]]
| neural tumours
|-
| [[Sustentaculoma of adrenal gland]]
| neural tumours
|-
| [[Mucosal epithelioid nerve sheath tumour]]
| neural tumours
|-
| [[Neurothekeoma]]
| neural tumours
|-
| [[Angiosarcoma]]
| vascular tumours
|-
| [[Kaposi's sarcoma]]
| vascular tumours
|-
| [[Composite hemangioendothelioma]]
| vascular tumours
|-
| [[Pseudoangiomatous stromal hyperplasia]]
| vascular tumours
|-
| [[Angiolymphoid hyperplasia with eosinophilia]]
| vascular tumours
|-
| [[Kaposiform hemangioendothelioma]]
| vascular tumours
|-
| [[Leiomyosarcoma]]
| muscle tumours
|-
| [[Hyalinizing matrix-rich rhabdomyosarcoma]]
| muscle tumours
|DDx: [[osteosarcoma]], [[chondrosarcoma]]
|-
| [[Spindle cell rhabdomyosarcoma]]
| muscle tumours
|-
| [[Glomangiopericytoma]]
| muscle tumours
|-
| [[Myopericytoma]]
| muscle tumours
|-
| [[Fetal or genital rhabdomyoma]]
| muscle tumours
|-
| [[Spindle cell lipoma]]
| lipomatous tumours
|-
| [[Spindle cell liposarcoma]]
| lipomatous tumours
|<ref name=pmid8067512>{{Cite journal| last1 = Dei Tos| first1 = AP.| last2 = Mentzel| first2 = T.| last3 = Newman| first3 = PL.| last4 = Fletcher| first4 = CD.| title = Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.| journal = Am J Surg Pathol| volume = 18| issue = 9| pages = 913-21| month = Sep| year = 1994| doi =| PMID = 8067512 }}</ref>
|-
| [[Massive localized lymphedema]]
| lipomatous tumours
|associated with [[obesity]]
|-
| [[Dedifferentiated liposarcoma]]
| lipomatous tumours
|-
| [[Lipoleiomyosarcoma]]
| lipomatous tumours
|<ref name=pmid12023578>{{Cite journal| last1 = Folpe| first1 = AL.| last2 = Weiss| first2 = SW.| title = Lipoleiomyosarcoma (well-differentiated liposarcoma with leiomyosarcomatous differentiation): a clinicopathologic study of nine cases including one with dedifferentiation.| journal = Am J Surg Pathol| volume = 26| issue = 6| pages = 742-9| month = Jun| year = 2002| doi =| PMID = 12023578 }}</ref>
|-
| [[Spindle cell osteosarcoma]]
| chondro-osseous tumours
|-
| [[Parosteal osteosarcoma]]
| chondro-osseous tumours
|-
| [[Extraskeletal myxoid chondrosarcoma ]]
| chondro-osseous tumours
|-
| [[Fibromatosis]]
| fibroblastic/myofibroblastic tumours
|-
| [[Desmoid tumour]]
| fibroblastic/myofibroblastic tumours
|-
| [[Hemorrhagic dermatomyofibroma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Sclerotic "plywood" fibroma]]
| fibroblastic/myofibroblastic tumours
| <ref>{{Cite journal  | last1 = Hanft | first1 = VN. | last2 = Shea | first2 = CR. | last3 = McNutt | first3 = NS. | last4 = Pullitzer | first4 = D. | last5 = Horenstein | first5 = MG. | last6 = Prieto | first6 = VG. | title = Expression of CD34 in sclerotic ("plywood") fibromas. | journal = Am J Dermatopathol | volume = 22 | issue = 1 | pages = 17-21 | month = Feb | year = 2000 | doi =  | PMID = 10698210 }}</ref>
|-
| [[Fibrosarcoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Dermatofibrosarcoma protuberans]]
| fibroblastic/myofibroblastic tumours
|-
| [[Angiomyofibroblastoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Myofibroblastic sarcoma]], low grade
| fibroblastic/myofibroblastic tumours
|-
| [[Solitary fibrous tumour]]
| fibroblastic/myofibroblastic tumours
|-
| [[Superficial acral fibromyxoma ]]
| fibroblastic/myofibroblastic tumours
|-
| [[Diffuse-type tenosynovial giant cell tumour]]
| fibroblastic/myofibroblastic tumours
|-
| [[Fasciitis-like reactive myofibroblastic proliferation]]
| fibroblastic/myofibroblastic tumours
|-
| [[Sclerosing epithelioid fibrosarcoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Myxoinflammatory fibroblastic sarcoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Dermatomyofibroma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Myofibrosarcoma]]
| fibroblastic/myofibroblastic tumours
|<ref name=pmid11176071>{{Cite journal| last1 = Montgomery| first1 = E.| last2 = Goldblum| first2 = JR.| last3 = Fisher| first3 = C.| title = Myofibrosarcoma: a clinicopathologic study.| journal = Am J Surg Pathol| volume = 25| issue = 2| pages = 219-28| month = Feb| year = 2001| doi =| PMID = 11176071 }}</ref>
|-
| [[Myofibromatosis-like perivascular myoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Myofibroblastoma]]
| fibroblastic/myofibroblastic tumours
| superficial cervicovaginal myofibroblastoma<ref name=pmid15693885>{{Cite journal  | last1 = Ganesan | first1 = R. | last2 = McCluggage | first2 = WG. | last3 = Hirschowitz | first3 = L. | last4 = Rollason | first4 = TP. | title = Superficial myofibroblastoma of the lower female genital tract: report of a series including tumours with a vulval location. | journal = Histopathology | volume = 46 | issue = 2 | pages = 137-43 | month = Feb | year = 2005 | doi = 10.1111/j.1365-2559.2005.02063.x | PMID = 15693885 }}</ref>
|-
| [[Cellular Angiofibroma]]
| fibroblastic/myofibroblastic tumours
| <ref name=pmid15489646>{{Cite journal  | last1 = Iwasa | first1 = Y. | last2 = Fletcher | first2 = CD. | title = Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. | journal = Am J Surg Pathol | volume = 28 | issue = 11 | pages = 1426-35 | month = Nov | year = 2004 | doi =  | PMID = 15489646 }}</ref>
|-
| [[Giant cell angiofibroma ]]
| fibroblastic/myofibroblastic tumours
|-
| [[Myofibroma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Ossifying fibromyxoid tumour of soft parts]]
| fibroblastic/myofibroblastic tumours
| S-100 +ve
|-
| [[Myxofibrosarcoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Pleomorphic undifferentiated sarcoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Atypical fibroxanthoma]]
| fibroblastic/myofibroblastic tumours
|-
| [[Low grade fibromyxoid sarcoma ]]
| fibroblastic/myofibroblastic tumours
| hyalinizing spindle cell tumour with giant rosettes
|-
| [[Inflammatory myofibroblastic tumour]]
| fibroblastic/myofibroblastic tumours
|may be cytokeratin +ve, ALK +ve
|-
| [[Angiomatoid fibrous histiocytoma]]
| fibroblastic/myofibroblastic tumours
| younger individuals
|-
| [[Synovial sarcoma]]
| sarcoma
|-
| [[Gastrointestinal stromal tumour]]
| mesenchymal lesion
|-
| [[Meningioma]]
| other
| fibrous variant
|-
| [[Epithelioid sarcoma]]
| sarcoma
| pseudomyogenic "fibroma-like" variant
|-
| [[Anthracotic & anthracosilicotic spindle cell pseudotumour of mediastinal lymph node]]
| other
|-
| [[Adenosarcoma]]
| sarcoma
| under-sampled glandular component
|-
| [[Mixed Muellerian tumour]]
| sarcoma
|-
| [[Blastemal elements of Wilms' tumour]]
| other
|-
| [[Blastemal elements of pulmonary blastoma]]
| other
|-
| [[Ossifying desmoplastic nested epithelial-stromal tumour of liver]]
| other
|-
| [[Phosphaturic mesenchymal tumour]]
| other
|-
| [[Ectomesenchymoma]]
| other
| skeletal muscle and neural differentiation
|-
| [[Congenital pseudarthosis]] associated with [[neurofibromatosis]]
| other
|-
| [[Endometrial stromal sarcoma]]
| sarcoma
|-
| [[Mixed endometrial stromal and smooth muscle tumour of the uterus]]
| mesenchymal lesion
|<ref name=pmid9706980>{{Cite journal| last1 = Oliva| first1 = E.| last2 = Clement| first2 = PB.| last3 = Young| first3 = RH.| last4 = Scully| first4 = RE.| title = Mixed endometrial stromal and smooth muscle tumors of the uterus: a clinicopathologic study of 15 cases.| journal = Am J Surg Pathol| volume = 22| issue = 8| pages = 997-1005| month = Aug| year = 1998| doi =| PMID = 9706980 }}</ref>
|-
| [[Yolk sac tumour, spindle cell]]
| germ cell tumour
|-
| [[Granulosa cell tumour]]
| sex cord tumour
|-
| [[Thecoma]]
| sex cord tumour
|-
| [[Fibroma]]
| sex cord tumour
|-
| [[Leydig cell tumour, spindle cell]]
| sex cord tumour
|<ref>{{Cite journal| last1 = Ulbright| first1 = TM.| last2 = Srigley| first2 = JR.| last3 = Hatzianastassiou| first3 = DK.| last4 = Young| first4 = RH.| title = Leydig cell tumors of the testis with unusual features: adipose differentiation, calcification with ossification, and spindle-shaped tumor cells.| journal = Am J Surg Pathol| volume = 26| issue = 11| pages = 1424-33| month = Nov| year = 2002| doi =| PMID = 12409718 }}</ref>
|-
| [[Gynandroblastoma]]
| sex cord tumour
|[[Sertoli cell tumour]] & [[granulosa cell tumour]]
|-
|}
==Microscopic==
===Grouping by pleomorphism===
*Monomorphic - small variation of tumour cell size.
*Pleomorphic - large variation of tumour cell size.
DDx - monomorphic:
*[[Synovial sarcoma]].
*[[Fibrosarcoma]].
*[[MPNST]].
*Metaplastic carcinoma, e.g. [[metaplastic breast carcinoma]].


==Malignant fibrous histiocytoma==
DDx - pleomorphic:
Work-up (MSH):
*[[Pleomorphic undifferentiated sarcoma]].
*CD34 - GIST, angiosarcoma, solitary fibrous tumour/hemangiopericytoma, Kaposi sarcoma.
*Pleomorphic [[liposarcoma]].
*S100 - neural marker, sensitive for malignant melanoma, Langerhans cells, clear cell sarcoma.
*Pleomorphic [[rhabdomyosarcoma]].
*Pleomorphic [[leiomyosarcoma]].
*Metaplastic carcinoma.
**[[Metaplastic breast carcinoma]].
**[[Spindle cell squamous carcinoma]].
*[[Malignant melanoma]].
*Others - see ''[[pleomorphic tumours]]'' for an exhaustive DDx.
 
===Grouping by pattern===
:See: ''[[Basics#Morphologic patterns]]''.
 
==IHC==
===Basic panel===
Dixon panel:<ref>BD. 1 April 2011.</ref>
*CD34.
*Desmin.
*SMA.
*[[S-100]].
*[[AE1/AE3]] (pankeratin).
 
Memory device ''SCADS'' or ''CAD SS'' = S-100, CD34, AE1/AE3, Desmin, SMA.
 
===Big panels===
====A MSH MFH panel====
*CD34 - [[GIST]], [[angiosarcoma]], [[solitary fibrous tumour]]/[[hemangiopericytoma]], [[Kaposi sarcoma]], [[DFSP]], [[PASH]].
*S-100 - neural marker, sensitive for [[malignant melanoma]], Langerhans cells, [[clear cell sarcoma]].
*Desmin - smooth muscle.
*Desmin - smooth muscle.
*MIB1 - proliferation.
*MIB1 - proliferation.
*CD99 - immature T cells (thymus), small round cell tumours, synovial sarcoma, merkel cell carcinoma, osteosarcoma.<ref name=Ref_DCHH108>{{Ref DCHH|108}}</ref>
*CD99 - immature T cells (thymus), [[small round cell tumours]], [[synovial sarcoma]], [[Merkel cell carcinoma]], [[osteosarcoma]].<ref name=Ref_DCHH108>{{Ref DCHH|108}}</ref>
**Considered "slutty", i.e. non-specific, by Dr. Fletcher; useful if membranous stain pattern = Ewing sarcoma/PNET.
**Considered "slutty", i.e. non-specific, by Dr. Fletcher; useful if membranous stain pattern = Ewing sarcoma/PNET.
*BCL2 - synovial sarcoma, [[small cell lymphomas]], spindle cell lipoma.<ref name=Ref_DCHH107>{{Ref DCHH|107}}</ref><ref>URL: [http://ajp.amjpathol.org/cgi/content/full/160/3/759 http://ajp.amjpathol.org/cgi/content/full/160/3/759]. Accessed on: 3 August 2010.</ref>
*BCL2 - [[synovial sarcoma]], [[small cell lymphomas]], spindle cell lipoma,<ref name=Ref_DCHH107>{{Ref DCHH|107}}</ref><ref>URL: [http://ajp.amjpathol.org/cgi/content/full/160/3/759 http://ajp.amjpathol.org/cgi/content/full/160/3/759]. Accessed on: 3 August 2010.</ref> [[solitary fibrous tumour]].
*PGP 9.5.
*PGP 9.5.
*SMMS - smooth muscle.
*SMMS - smooth muscle.
*Caldesmon - muscle.
*Caldesmon - muscle.
*PDGFR - GIST.
*PDGFR - [[GIST]]
*DOG-1 - GIST (membranous, cytoplasmic),<ref name=pmid19417627>{{cite journal |author=Ardeleanu C, Arsene D, Hinescu M, ''et al.'' |title=Pancreatic expression of DOG1: a novel gastrointestinal stromal tumor (GIST) biomarker |journal=Appl. Immunohistochem. Mol. Morphol. |volume=17 |issue=5 |pages=413–8 |year=2009 |month=October |pmid=19417627 |doi=10.1097/PAI.0b013e31819e4dc5 |url=}}</ref> normal pancreas (islets),<ref name=pmid19417627/> associated with BRCA1.
*[[DOG1]] - GIST (membranous, cytoplasmic),<ref name=pmid19417627>{{cite journal |author=Ardeleanu C, Arsene D, Hinescu M, ''et al.'' |title=Pancreatic expression of DOG1: a novel gastrointestinal stromal tumor (GIST) biomarker |journal=Appl. Immunohistochem. Mol. Morphol. |volume=17 |issue=5 |pages=413–8 |year=2009 |month=October |pmid=19417627 |doi=10.1097/PAI.0b013e31819e4dc5 |url=}}</ref> normal pancreas (islets),<ref name=pmid19417627/> associated with [[BRCA1]].


Others:
Others:
*Beta-catenin - a small subset of soft tissue lesions:
*Beta-catenin - a small subset of soft tissue lesions:
**Desmoidtype fibromatosis (~70%), solitary fibrous tumour & endometrial stromal sarcoma (~40%), synovial sarcoma (~30%).<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>
**[[Desmoid-type fibromatosis]] (~70%), solitary fibrous tumour & endometrial stromal sarcoma (~40%), synovial sarcoma (~30%).<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>
 
====DJH panels====
=====MFH=====
*Vimentin.
*Keratin 5D3 - carcinomas.
*Keratin LP34 - carcinomas.
*[[EMA]].
*CD99.
*MSA.
*SMA.
*Caldesmon.
*CD34.
*S-100 - neural tumours.
*HMB45 - [[melanoma]]
*Mart-1 - [[melanoma]].
*Tyrosinase.
*MYF-4 - [[RMS]].
*Alpha-1-antichymotrypsin.
 
=====SRCT=====
Same as MFH panel plus the following:
*CD45.
*CD30.
*CD3.
*[[CD20]].
*Desmin.
*CD31 - [[angiosarcoma]].
*Factor VIII - [[angiosarcoma]].


==See also==
==See also==
Line 34: Line 499:
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Immunohistochemistry]].
*[[Immunohistochemistry]].
*[[Spindle cell]].
*[[Epithelioid cell lesions]].
*[[Spindle cell lesions of the skin]].
==Treatment==
*It depends on the subtype.
*Malignant lesions are usually sarcomas and treated with radiation and surgery.<ref name=pmid19664493>{{cite journal |author=Collini P, Sorensen PH, Patel S, ''et al.'' |title=Sarcomas with spindle cell morphology |journal=Semin. Oncol. |volume=36 |issue=4 |pages=324–37 |year=2009 |month=August |pmid=19664493 |doi=10.1053/j.seminoncol.2009.06.007 |url=}}</ref>


==References==
==References==

Latest revision as of 15:44, 18 June 2017

A spindle cell lesion (MPNST). H&E stain.

Spindle cell lesions are seen frequent enough that one ought to have a solid approach to 'em.

A general introduction to spindle cells is found in the spindle cell article.

General DDx

Spindle cells should make think:

  • Nerve.
  • Muscle.
    • Smooth.
    • Skeletal.
  • Other.
    • Carcinoma, e.g. metaplastic carcinoma.

A long differential diagnosis of spindle cell tumours

Adapted from Miller with modifications:[1]

Tumour Group Notes/comments
Malignant melanoma melanoma
PEComa mesenchymal lesion angiomyolipoma, lymphangioleiomyomatosis, other PEComas
Sarcomatoid carcinoma carcinoma
Desmoplastic mesothelioma carcinoma
Thymoma epithelial lesion
Spindle epithelial tumor with thymus-like differentiation epithelial lesion
Malignant phyllodes tumour sarcoma
Chondroid syringoma epithelial lesion
Malignant mixed Muellerian tumour carcinoma
Syphilis infection
Bacillary angiomatosis infection
Myocbacterial spindle cell pseudotumour infection
Cryotococcal pseudotumour infection
Anaplastic large cell lymphoma lymphoid
Lymphocyte-depleted Hodgkin lymphoma lymphoid
Follicular dendritic cell sarcoma sarcoma
Interdigitating reticulum cell sarcoma sarcoma
Sclerosing extramedullary hematopoetic tumours other seen in chronic myeloproliferative disorders[2]
Juvenile xanthogranuloma histiocytic lesion
Granuloma annulare histiocytic lesion
Crystal-storing histiocytosis histiocytic lesion
Schwannoma neural tumours
Neurofibroma neural tumours
Malignant peripheral nerve sheath tumour neural tumours
Perineurioma neural tumours
Sustentaculoma of adrenal gland neural tumours
Mucosal epithelioid nerve sheath tumour neural tumours
Neurothekeoma neural tumours
Angiosarcoma vascular tumours
Kaposi's sarcoma vascular tumours
Composite hemangioendothelioma vascular tumours
Pseudoangiomatous stromal hyperplasia vascular tumours
Angiolymphoid hyperplasia with eosinophilia vascular tumours
Kaposiform hemangioendothelioma vascular tumours
Leiomyosarcoma muscle tumours
Hyalinizing matrix-rich rhabdomyosarcoma muscle tumours DDx: osteosarcoma, chondrosarcoma
Spindle cell rhabdomyosarcoma muscle tumours
Glomangiopericytoma muscle tumours
Myopericytoma muscle tumours
Fetal or genital rhabdomyoma muscle tumours
Spindle cell lipoma lipomatous tumours
Spindle cell liposarcoma lipomatous tumours [3]
Massive localized lymphedema lipomatous tumours associated with obesity
Dedifferentiated liposarcoma lipomatous tumours
Lipoleiomyosarcoma lipomatous tumours [4]
Spindle cell osteosarcoma chondro-osseous tumours
Parosteal osteosarcoma chondro-osseous tumours
Extraskeletal myxoid chondrosarcoma chondro-osseous tumours
Fibromatosis fibroblastic/myofibroblastic tumours
Desmoid tumour fibroblastic/myofibroblastic tumours
Hemorrhagic dermatomyofibroma fibroblastic/myofibroblastic tumours
Sclerotic "plywood" fibroma fibroblastic/myofibroblastic tumours [5]
Fibrosarcoma fibroblastic/myofibroblastic tumours
Dermatofibrosarcoma protuberans fibroblastic/myofibroblastic tumours
Angiomyofibroblastoma fibroblastic/myofibroblastic tumours
Myofibroblastic sarcoma, low grade fibroblastic/myofibroblastic tumours
Solitary fibrous tumour fibroblastic/myofibroblastic tumours
Superficial acral fibromyxoma fibroblastic/myofibroblastic tumours
Diffuse-type tenosynovial giant cell tumour fibroblastic/myofibroblastic tumours
Fasciitis-like reactive myofibroblastic proliferation fibroblastic/myofibroblastic tumours
Sclerosing epithelioid fibrosarcoma fibroblastic/myofibroblastic tumours
Myxoinflammatory fibroblastic sarcoma fibroblastic/myofibroblastic tumours
Dermatomyofibroma fibroblastic/myofibroblastic tumours
Myofibrosarcoma fibroblastic/myofibroblastic tumours [6]
Myofibromatosis-like perivascular myoma fibroblastic/myofibroblastic tumours
Myofibroblastoma fibroblastic/myofibroblastic tumours superficial cervicovaginal myofibroblastoma[7]
Cellular Angiofibroma fibroblastic/myofibroblastic tumours [8]
Giant cell angiofibroma fibroblastic/myofibroblastic tumours
Myofibroma fibroblastic/myofibroblastic tumours
Ossifying fibromyxoid tumour of soft parts fibroblastic/myofibroblastic tumours S-100 +ve
Myxofibrosarcoma fibroblastic/myofibroblastic tumours
Pleomorphic undifferentiated sarcoma fibroblastic/myofibroblastic tumours
Atypical fibroxanthoma fibroblastic/myofibroblastic tumours
Low grade fibromyxoid sarcoma fibroblastic/myofibroblastic tumours hyalinizing spindle cell tumour with giant rosettes
Inflammatory myofibroblastic tumour fibroblastic/myofibroblastic tumours may be cytokeratin +ve, ALK +ve
Angiomatoid fibrous histiocytoma fibroblastic/myofibroblastic tumours younger individuals
Synovial sarcoma sarcoma
Gastrointestinal stromal tumour mesenchymal lesion
Meningioma other fibrous variant
Epithelioid sarcoma sarcoma pseudomyogenic "fibroma-like" variant
Anthracotic & anthracosilicotic spindle cell pseudotumour of mediastinal lymph node other
Adenosarcoma sarcoma under-sampled glandular component
Mixed Muellerian tumour sarcoma
Blastemal elements of Wilms' tumour other
Blastemal elements of pulmonary blastoma other
Ossifying desmoplastic nested epithelial-stromal tumour of liver other
Phosphaturic mesenchymal tumour other
Ectomesenchymoma other skeletal muscle and neural differentiation
Congenital pseudarthosis associated with neurofibromatosis other
Endometrial stromal sarcoma sarcoma
Mixed endometrial stromal and smooth muscle tumour of the uterus mesenchymal lesion [9]
Yolk sac tumour, spindle cell germ cell tumour
Granulosa cell tumour sex cord tumour
Thecoma sex cord tumour
Fibroma sex cord tumour
Leydig cell tumour, spindle cell sex cord tumour [10]
Gynandroblastoma sex cord tumour Sertoli cell tumour & granulosa cell tumour

Microscopic

Grouping by pleomorphism

  • Monomorphic - small variation of tumour cell size.
  • Pleomorphic - large variation of tumour cell size.

DDx - monomorphic:

DDx - pleomorphic:

Grouping by pattern

See: Basics#Morphologic patterns.

IHC

Basic panel

Dixon panel:[11]

Memory device SCADS or CAD SS = S-100, CD34, AE1/AE3, Desmin, SMA.

Big panels

A MSH MFH panel

Others:

  • Beta-catenin - a small subset of soft tissue lesions:

DJH panels

MFH
  • Vimentin.
  • Keratin 5D3 - carcinomas.
  • Keratin LP34 - carcinomas.
  • EMA.
  • CD99.
  • MSA.
  • SMA.
  • Caldesmon.
  • CD34.
  • S-100 - neural tumours.
  • HMB45 - melanoma
  • Mart-1 - melanoma.
  • Tyrosinase.
  • MYF-4 - RMS.
  • Alpha-1-antichymotrypsin.
SRCT

Same as MFH panel plus the following:

See also

Treatment

  • It depends on the subtype.
  • Malignant lesions are usually sarcomas and treated with radiation and surgery.[17]

References

  1. Miller RT. (Sep 2013). Atlantic Diagnostic Immunohistochemistry Symposium. St. John's, NL, Canada.
  2. Remstein, ED.; Kurtin, PJ.; Nascimento, AG. (Jan 2000). "Sclerosing extramedullary hematopoietic tumor in chronic myeloproliferative disorders.". Am J Surg Pathol 24 (1): 51-5. PMID 10632487.
  3. Dei Tos, AP.; Mentzel, T.; Newman, PL.; Fletcher, CD. (Sep 1994). "Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.". Am J Surg Pathol 18 (9): 913-21. PMID 8067512.
  4. Folpe, AL.; Weiss, SW. (Jun 2002). "Lipoleiomyosarcoma (well-differentiated liposarcoma with leiomyosarcomatous differentiation): a clinicopathologic study of nine cases including one with dedifferentiation.". Am J Surg Pathol 26 (6): 742-9. PMID 12023578.
  5. Hanft, VN.; Shea, CR.; McNutt, NS.; Pullitzer, D.; Horenstein, MG.; Prieto, VG. (Feb 2000). "Expression of CD34 in sclerotic ("plywood") fibromas.". Am J Dermatopathol 22 (1): 17-21. PMID 10698210.
  6. Montgomery, E.; Goldblum, JR.; Fisher, C. (Feb 2001). "Myofibrosarcoma: a clinicopathologic study.". Am J Surg Pathol 25 (2): 219-28. PMID 11176071.
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