Low-grade fibromyxoid sarcoma

Low-grade fibromyxoid sarcoma
Micrograph of a low-grade fibromyxoid sarcoma. H&E stain.
SpecialtyPathology

Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of low-grade sarcoma first described by H. L. Evans in 1987.[1] LGFMS are soft tissue tumors of the mesenchyme-derived connective tissues; on microscopic examination, they are found to be composed of spindle-shaped cells that resemble fibroblasts.[2] These fibroblastic, spindle-shaped cells are neoplastic cells that in most cases of LGFMS express fusion genes, i.e. genes composed of parts of two different genes that form as a result of mutations.[2] The World Health Organization (2020) classified LGFMS as a specific type of tumor in the category of malignant fibroblastic and myofibroblastic tumors.[3]

LGFMS tumors occur in individuals of almost any age but up to 20% are less than 18 years/old. The tumors typically involve the proximal extremities but can occur virtually anywhere in the body.[4] The progression of these tumors commonly takes an indolent, prolonged course involving many years or decades. Over this time, however, the tumors often recur at the site of their surgical removal and/or metastasize usually to the lung or pleural tissues surrounding the lungs.[5] These metastasis can develop decades after the tumor's initial presentation and diagnosis.[6]

LGFMS can be difficult to distinguish from other mesenchymal tumors,[7] particularly from sclerosing epithelioid fibrosarcoma (SEF).[8] LGFMS tumors present with many clinical and pathological features that are similar to those in SEF. Indeed, current studies suggest that LGFMS may be an early form of SEF. For example, a tumor may present with features typical of LGFMS but over time progress to features typical of sclerosing epithelioid fibrosarcomas. This progression may be particularly evident in recurrent or metastatic "LGFMS" tumors.[8][9] Since the World Health Organization has classified LGFMS as one of the malignant fibroblastic and myofibroblastic tumors that is distinctly different than SEF,[3] SEF and LGFMS are here regarded as separate tumor forms.

  1. ^ Evans HL (November 1987). "Low-grade fibromyxoid sarcoma. A report of two metastasizing neoplasms having a deceptively benign appearance". American Journal of Clinical Pathology. 88 (5): 615–9. doi:10.1093/ajcp/88.5.615. PMID 3673943.
  2. ^ a b Flucke U, van Noesel MM, Siozopoulou V, Creytens D, Tops BB, van Gorp JM, Hiemcke-Jiwa LS (June 2021). "EWSR1-The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review". Diagnostics (Basel, Switzerland). 11 (6): 1093. doi:10.3390/diagnostics11061093. PMC 8232650. PMID 34203801.
  3. ^ a b Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC 8167394. PMID 33179614.
  4. ^ Porrino J, Al-Dasuqi K, Irshaid L, Wang A, Kani K, Haims A, Maloney E (June 2021). "Update of pediatric soft tissue tumors with review of conventional MRI appearance-part 1: tumor-like lesions, adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors". Skeletal Radiology. 51 (3): 477–504. doi:10.1007/s00256-021-03836-2. PMID 34191084. S2CID 235678096.
  5. ^ Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J (September 2021). "Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I". Cancer Treatment Reviews. 99: 102259. doi:10.1016/j.ctrv.2021.102259. PMID 34311246.
  6. ^ Evans, Harry L (2011). "Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 33 Cases With Long-Term Follow-Up". The American Journal of Surgical Pathology. 35 (10): 1450–1462. doi:10.1097/PAS.0b013e31822b3687. PMID 21921785. S2CID 21592343.
  7. ^ Yue Y, Liu Y, Song L, Chen X, Wang Y, Wang Z (February 2018). "MRI findings of low-grade fibromyxoid sarcoma: a case report and literature review". BMC Musculoskeletal Disorders. 19 (1): 65. doi:10.1186/s12891-018-1976-z. PMC 6389061. PMID 29482535.
  8. ^ a b Mustafa S, VandenBussche CJ, Ali SZ, Siddiqui MT, Wakely PE (2020). "Cytomorphologic findings of low-grade fibromyxoid sarcoma". Journal of the American Society of Cytopathology. 9 (3): 191–201. doi:10.1016/j.jasc.2020.01.006. PMID 32197967. S2CID 212810533.
  9. ^ Murshed KA, Al-Bozom I, Ammar A (August 2021). "Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor". APMIS. 129 (8): 455–460. doi:10.1111/apm.13157. PMID 34048081. S2CID 235232220.