low-grade fibromyxoid sarcoma

low-grade fibromyxoid sarcoma

low-grade malignant tumor of soft tissues of proximal extremities or trunk, composed of fibroblasts forming myxoid areas or surrounding giant collagen rosettes.

low-grade fibromyxoid sarcoma

A rare, deep soft-tissue sarcoma of young adults with a deceptively bland appearance, which is characterised by local recurrences and, eventually, metastases to lung and bone.

DiffDx Benign
Deep fibromatosis, nodular fasciitis.

DiffDx Malignant
Leiomyosarcoma, myofibrosarcoma, sclerosing epithelioid fibrosarcoma, low-grade fibrosarcoma, classic fibrosarcoma, inflammatory myxohyaline/myxoinflammatory fibroblastic sarcoma.

Prognosis
Local recurrence, 9%; metastases, 6%; death, 2%.
References in periodicals archive ?
The patient underwent left lower-lobe resection; pathology on a section of the lingula revealed a low-grade fibromyxoid sarcoma consistent with metastatic disease.
The most commonly used FISH probe set for this morphology was for the FUS gene region (16p11), which is split apart in either a t(7;16) or t(11;16) in low-grade fibromyxoid sarcoma (15,16) or in the t(12;16) in myxoid liposarcoma.
0/6 (0) Low-grade myxoid neoplasms (n = 34) Low-grade fibromyxoid sarcoma .
Examples included a case of perineurioma where FUS FISH was performed to exclude the possibility of low-grade fibromyxoid sarcoma.
Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene.
Cytogenetic and molecular genetic studies are still of limited value for the diagnosis of perineuriomas but may play a fundamental role in excluding important differential diagnostic considerations, such as DFSP and low-grade fibromyxoid sarcoma.
For deep-seated perineuriomas, the differential diagnosis mainly includes desmoid-type fibromatosis, low-grade fibromyxoid sarcoma (LGFS), solitary fibrous tumor, low-grade leiomyosarcoma, leiomyoma, and myxo-fibrosarcoma (Table).
Low-grade fibromyxoid sarcoma is a particularly difficult differential diagnosis since it may have overlapping histologic features with perineurioma.
Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma.
It appears that low-grade fibromyxoid sarcoma is a fibroblastic neoplasm in contrast to hyalinizing spindle cell tumor with giant rosettes whose spindle cells are composed of a mixture of histiocytes and fibroblasts.

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