Spindle cell lesions

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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]

  • 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

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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. BD. 1 April 2011.
  12. Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 108. ISBN 978-0470519035.
  13. Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 107. ISBN 978-0470519035.
  14. URL: http://ajp.amjpathol.org/cgi/content/full/160/3/759. Accessed on: 3 August 2010.
  15. 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.
  16. 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.
  17. 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.