Haemorrhagic low-grade fibromyxoid sarcoma: MR findings in two young women

Abstract

ABSTRACT. Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma characterised by a deceptively bland histological appearance and a paradoxically aggressive behaviour.LGFMS usually presents in young-to-middle-aged adults as a painless, slow-growing mass with the potential for local recurrence and metastasis despite low-grade histology. Several case reports have described variable MR findings of LGFMS without haemorrhage or necrosis. We report here on the MR findings in two young women with haemorrhagic LGFMS in the thigh.

Recurrent Low-Grade Fibromyxoid Sarcoma of the Neck: Report of a Case and Review of the Literature

“…LGFMS is most prevalent in young patients and it can be found in the lower extremities and deep soft tissue, with even sex distribution [3]. Nonetheless, Folpe et al demonstrate a male predominance in accordance with other smaller series.…”

“…MR findings are in absolute agreement with the pathological features. In general, fibrous tissue components appear hypointense on T1/T2-weighted images [3]. Additional slight enhancement is commonly encountered.…”

“…Alternatively, reverse transcriptase polymerase chain reaction (RT-PCR) can also be used to detect either fusion product; such assays have only rarely been proposed to analyze formalin-fixed, paraffin-embedded neoplasm samples [11]. It is obvious that diagnosis is challenging; both long latency periods between diagnosis and recurrence, as well as slow tumor growth have been assessed, underlying the importance of a long-term clinical follow-up and imaging surveillance [3].…”

“…Among them, lowgrade fibromyxoid sarcoma (LGFMS) represents a rare, indolent, benign and distinctive sarcoma first reported by Evans in 1987 [2]. Most patients range in age from 25 to 46 years with an equal incidence in men and women [3]. LGFMS proliferates at various anatomical locations, especially in subcutaneous or superficial soft tissue on the proximal extremities, as a painless, slow-growing, non-tender, firm mass.…”

“…Local recurrence and metastasis, usually to the lungs, have been detected at variable but significant rates [5]. Typically, LGFMS appears as a well-described encapsulated mass, without necrosis or hemorrhage [3]. Histologically, the tumor consists of bland, deceptively benign-appearing spindle cells in a whirling pattern, as well as myxoid and collagenized stroma.…”