Low-grade fibromyxoid sarcoma

From Libre Pathology
Jump to navigation Jump to search
The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.

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. https://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.