Spindle cell lesions
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:
- Synovial sarcoma.
- Fibrosarcoma.
- MPNST.
- Metaplastic carcinoma, e.g. metaplastic breast carcinoma.
DDx - pleomorphic:
- Pleomorphic undifferentiated sarcoma.
- Pleomorphic liposarcoma.
- Pleomorphic rhabdomyosarcoma.
- Pleomorphic leiomyosarcoma.
- Metaplastic carcinoma.
- Malignant melanoma.
- Others - see pleomorphic tumours for an exhaustive DDx.
Grouping by pattern
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
- 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.
- MIB1 - proliferation.
- CD99 - immature T cells (thymus), small round cell tumours, synovial sarcoma, Merkel cell carcinoma, osteosarcoma.[12]
- 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,[13][14] solitary fibrous tumour.
- PGP 9.5.
- SMMS - smooth muscle.
- Caldesmon - muscle.
- PDGFR - GIST
- DOG1 - GIST (membranous, cytoplasmic),[15] normal pancreas (islets),[15] associated with BRCA1.
Others:
- Beta-catenin - a small subset of soft tissue lesions:
- Desmoid-type fibromatosis (~70%), solitary fibrous tumour & endometrial stromal sarcoma (~40%), synovial sarcoma (~30%).[16]
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
- Bone.
- Dermatopathology.
- Hematopathology.
- Soft tissue lesions.
- 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.[17]
References
- ↑ Miller RT. (Sep 2013). Atlantic Diagnostic Immunohistochemistry Symposium. St. John's, NL, Canada.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Montgomery, E.; Goldblum, JR.; Fisher, C. (Feb 2001). "Myofibrosarcoma: a clinicopathologic study.". Am J Surg Pathol 25 (2): 219-28. PMID 11176071.
- ↑ Ganesan, R.; McCluggage, WG.; Hirschowitz, L.; Rollason, TP. (Feb 2005). "Superficial myofibroblastoma of the lower female genital tract: report of a series including tumours with a vulval location.". Histopathology 46 (2): 137-43. doi:10.1111/j.1365-2559.2005.02063.x. PMID 15693885.
- ↑ Iwasa, Y.; Fletcher, CD. (Nov 2004). "Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases.". Am J Surg Pathol 28 (11): 1426-35. PMID 15489646.
- ↑ Oliva, E.; Clement, PB.; Young, RH.; Scully, RE. (Aug 1998). "Mixed endometrial stromal and smooth muscle tumors of the uterus: a clinicopathologic study of 15 cases.". Am J Surg Pathol 22 (8): 997-1005. PMID 9706980.
- ↑ Ulbright, TM.; Srigley, JR.; Hatzianastassiou, DK.; Young, RH. (Nov 2002). "Leydig cell tumors of the testis with unusual features: adipose differentiation, calcification with ossification, and spindle-shaped tumor cells.". Am J Surg Pathol 26 (11): 1424-33. PMID 12409718.
- ↑ BD. 1 April 2011.
- ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 108. ISBN 978-0470519035.
- ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 107. ISBN 978-0470519035.
- ↑ URL: http://ajp.amjpathol.org/cgi/content/full/160/3/759. Accessed on: 3 August 2010.
- ↑ 15.0 15.1 Ardeleanu C, Arsene D, Hinescu M, et al. (October 2009). "Pancreatic expression of DOG1: a novel gastrointestinal stromal tumor (GIST) biomarker". Appl. Immunohistochem. Mol. Morphol. 17 (5): 413–8. doi:10.1097/PAI.0b013e31819e4dc5. PMID 19417627.
- ↑ Ng TL, Gown AM, Barry TS, et al. (January 2005). "Nuclear beta-catenin in mesenchymal tumors". Mod. Pathol. 18 (1): 68–74. doi:10.1038/modpathol.3800272. PMID 15375433.
- ↑ Collini P, Sorensen PH, Patel S, et al. (August 2009). "Sarcomas with spindle cell morphology". Semin. Oncol. 36 (4): 324–37. doi:10.1053/j.seminoncol.2009.06.007. PMID 19664493.