Low-grade fibromyxoid sarcoma

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Low-grade fibromyxoid sarcoma, abbreviated LGFMS, is an uncommon malignant fibroblastic/myofibroblastic tumour.

It is also known as hyalinizing spindle cell tumour. It should not be confused with myxofibrosarcoma.

General

  • Deep soft tissue.
  • Related to hyalinizing spindle cell tumour with giant collagen rosettes.[1]

Microscopic

Features:[2]

  • Myxoid stroma alternating with fibrogenic areas - key feature.
  • Low cellularity.
  • Spindle cells.
  • +/-Rosette of collagen with central hyaline core.[3]

Notes:

  • Few/absent mitoses.

DDx:

Images

www:

IHC

Features:[3]

  • EMA +ve.
  • CD99 +ve.
  • BCL2 +ve.

Others:[3]

  • SMA -ve.
  • S100 -ve.
  • Desmin -ve.

Molecular pathology

  • t(7;16)(q33;p11).[4]
  • t(11;16).[3]

See also

References

  1. Ramaswamy, AS.; Chatura, KR.. "Hyalinizing spindle cell tumor with giant rosettes.". Ann Saudi Med 31 (1): 83-6. PMC 3101732. PMID 20864787. //www.ncbi.nlm.nih.gov/pmc/articles/PMC3101732/.
  2. Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
  3. 3.0 3.1 3.2 3.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 611. ISBN 978-0781765275.
  4. Panagopoulos I, Storlazzi CT, Fletcher CD, et al. (July 2004). "The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma". Genes Chromosomes Cancer 40 (3): 218–28. doi:10.1002/gcc.20037. PMID 15139001.