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
LGFMS - low mag. (WC)
LGFMS - intermed. mag. (WC)
LGFMS - high mag. (WC)
www:
IHC
Features:[3]
- EMA +ve.
- CD99 +ve.
- BCL2 +ve.
Others:[3]
- SMA -ve.
- S100 -ve.
- Desmin -ve.
Molecular pathology
See also
References
- ↑ 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/.
- ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
- ↑ 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.
- ↑ 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.