Low-grade fibromyxoid sarcoma
Citation, DOI and article data
Low-grade fibromyxoid sarcomas are malignant fibroblastic soft tissue neoplasms characterized by alternating fibrous and myxoid elements and a propensity to late recurrence and metastasis.
An alternative term for low-grade fibromyxoid sarcoma is Evans tumor. The term hyalinizing spindle cell tumor with giant rosettes is now discouraged.
The incidence of low-grade fibromyxoid sarcomas might have been underestimated due to their propensity of mimicking other soft tissue tumors but is generally considered relatively rare. They can occur in a wide age range but occur most frequently in younger adults and about one-fifth of tumors are seen in adolescents. Men are slightly more commonly affected 1,2.
Typical manifestation is a painless slow-growing mass sometimes present over years 1.
Low-grade fibromyxoid sarcomas are malignant tumors with alternating collagenous and myxoid components with bland spindle cells arranged in a fascicular or whorling growth pattern 1.
The cause of low-grade fibromyxoid sarcomas is unknown.
In adults, low-grade fibromyxoid sarcomas usually arise from the subfascial soft tissues of the proximal extremities and the trunk. Less common sites of involvement are the abdominal cavity, the retroperitoneum or mediastinum. In children, the superficial soft tissues of the head and neck region are often involved 1-3.
The macroscopic appearance of low-grade fibromyxoid sarcomas is characterized by a firm well-demarcated fibrous tumor with mucoid areas 1,4.
Microscopic characteristics of low-grade fibromyxoid sarcomas include the following 1,2:
- an admixture of collagenous with few cells and more cellular myxoid zones
- abrupt transitions between the two patterns
- bland spindle cells growing in a fascicular whorling or storiform pattern
- arcades of small vessels with occasional staghorn pattern of vascularity
- giant hyaline collagen rosettes surrounded by epithelioid tumor cells in several cases
- sometimes sclerosing epithelioid fibrosarcoma-like patterns
Immunohistochemistry stains express MUC4 in a very high number of cases, which is highly sensitive and specific for low low-grade fibromyxoid sarcomas and sclerosing epithelioid fibrosarcoma. Other expressions include CD99, BCL2, EMA and vimentin in many cases as well as smooth muscle actin in a low number 1,2,4.
Low-grade fibromyxoid sarcomas regularly show either FUS-CREB3L2 or FUS-CREB3L1 gene fusions.
Imaging features of the tumor have been described as multinodular, heterogeneous, gyriform often with complex solid cystic components and mostly well-defined tumor margins 2-4.
Ultrasound findings of low-grade fibromyxoid sarcomas are described as solid soft tissue tumors with heterogeneous echogenicity 5.
Low-grade fibromyxoid sarcomas show the following imaging features 2,3,5:
- hypodense compared to skeletal muscle in most cases
- calcifications are uncommon
MR imaging findings have been described as tumors with a striated appearance or gyriform patterns of signal intensity and contrast enhancement 2-5. A substantial number of cases have been reported showing peritumoral edema and/or a split fat sign.
Signal characteristics were described as follows 2-5:
- mostly hypo- to isointense to muscle
- fibrous components: hypointense
- myxoid components: hypointense
- heterogeneous high-signal intensity
- fibrous components: hypointense
- myxoid components: hyperintense
- T2FS/PDFS: iso- to hyperintense
- DWI: diffusion restriction
- T1 C+ (Gd): inhomogeneous or alternating or gyriform patterns of enhancement
The radiological report should include a description of the following features:
- form, location and size
- tumor margins
- relation to adjacent bones and the muscular fascia
- relationship to local nerves and vessels
- relationship to other organs
Treatment and prognosis
Recurrences and metastasis are low within the first five years after excision but much higher in the long term with overall recurrence rates higher than 60% and a fatality rate of over 40%. Metastases have been observed even more than 40 years after primary resection. The most common sites for metastasis are lung, pleura, bone and liver 1,2.
History and etymology
Low-grade fibromyxoid sarcoma was first described by an American pathologist Harry L Evans in 1987, hence its alternate name of Evans tumor 3-6.
Conditions that can mimic the appearance of low-grade fibromyxoid sarcomas include 1-5:
- 1. W. H. O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020-04-17) ISBN: 9789283245025
- 2. Wang L, Li L, Chen D, Yang L, Li S, Cheng C. Low-Grade Myofibroblastic Sarcoma: Clinical and Imaging Findings. BMC Med Imaging. 2019;19(1):36. doi:10.1186/s12880-018-0287-z
- 3. Sargar K, Kao S, Spunt S et al. MRI and CT of Low-Grade Fibromyxoid Sarcoma in Children: A Report From Children's Oncology Group Study ARST0332. AJR Am J Roentgenol. 2015;205(2):414-20. doi:10.2214/AJR.14.13972
- 4. Yue Y, Liu Y, Song L, Chen X, Wang Y, Wang Z. MRI Findings of Low-Grade Fibromyxoid Sarcoma: A Case Report and Literature Review. BMC Musculoskelet Disord. 2018;19(1):65. doi:10.1186/s12891-018-1976-z
- 5. Hwang S, Kelliher E, Hameed M. Imaging Features of Low-Grade Fibromyxoid Sarcoma (Evans Tumor). Skeletal Radiol. 2012;41(10):1263-72. doi:10.1007/s00256-012-1417-2
- 6. Evans H. Low-Grade Fibromyxoid Sarcoma. A Report of Two Metastasizing Neoplasms Having a Deceptively Benign Appearance. Am J Clin Pathol. 1987;88(5):615-9. doi:10.1093/ajcp/88.5.615