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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.
*Pleomorphic [[liposarcoma]].
*S100 - neural marker, sensitive for malignant melanoma.
*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.
*MIB-1 - proliferation.
*MIB1 - proliferation.
*CD99 - small round cell tumours, considered "slutty", i.e. non-specific, by Dr. Fletcher; useful if membranous stain pattern = Ewing sarcoma/PNET.
*CD99 - immature T cells (thymus), [[small round cell tumours]], [[synovial sarcoma]], [[Merkel cell carcinoma]], [[osteosarcoma]].<ref name=Ref_DCHH108>{{Ref DCHH|108}}</ref>
*Bcl-2.
**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> [[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==
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*[[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==
Michael
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