Myxofibrosarcoma

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Myxofibrosarcoma is a malignant soft tissue tumour, in the fibroblastic/myofibroblastic tumours group. It is also known as myxoid malignant fibrous histiocytoma or myxoid MFH.[1]

It should not be confused with low-grade fibromyxoid sarcoma.[2]

General

  • Malignant.
  • Usually older people, superficial (skin/dermis) and extremities (arm, legs).[3][4]
  • Prognosis better than pleomorphic undifferentiated sarcoma.
    • Metastatic potential inversely related to component/portion that is myxoid.[5]

Microscopic

Features:[3]

  • Discontinuous fibrous septae.
  • Myxoid background.
  • Variable cellularity and nuclear pleomorphism.
  • Spindle cells or epithelioid cells.[4]
  • Curvilinear vessels.[4]

DDx:

Image:

IHC

  • Vimentin +ve -- otherwise non-distinctive.[3]

See also

References

  1. Fujimura, T.; Okuyama, R.; Terui, T.; Okuno, K.; Masu, A.; Masu, T.; Chiba, S.; Kunii, T. et al. (Aug 2005). "Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases.". J Cutan Pathol 32 (7): 512-5. doi:10.1111/j.0303-6987.2005.00368.x. PMID 16008697.
  2. 2.0 2.1 Mentzel, T.; Katenkamp, D.; Fletcher, CD. (Mar 1996). "[Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course].". Pathologe 17 (2): 116-21. PMID 8650138.
  3. 3.0 3.1 3.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 612-3. ISBN 978-0781765275.
  4. 4.0 4.1 4.2 Nascimento, AF.; Bertoni, F.; Fletcher, CD. (Jan 2007). "Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases.". Am J Surg Pathol 31 (1): 99-105. doi:10.1097/01.pas.0000213379.94547.e7. PMID 17197925.
  5. Weiss, SW.; Enzinger, FM. (Apr 1977). "Myxoid variant of malignant fibrous histiocytoma.". Cancer 39 (4): 1672-85. PMID 192434.