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Case Reports
. 2021 May-Aug;12(2):271-275.
doi: 10.4103/njms.NJMS_54_15. Epub 2021 Jul 15.

Low-grade fibromyxoid sarcoma: A rare case report

Vinayak Gourish Naik  1 , Kirthi Kumar Rai  1 , H R Shivakumar  1
Affiliations
Case Reports

Low-grade fibromyxoid sarcoma: A rare case report

Vinayak Gourish Naik et al. Natl J Maxillofac Surg. 2021 May-Aug.

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of sarcoma that is characterized by benign-appearing histologic features but a paradoxically aggressive clinical course. Recognition of this lesion is important because of its indolent but metastasizing nature. These tumors generally occur in young to middle-aged adults, sometimes in children, but rarely in the high-aged adults. LGFMS typically affects the deep soft tissues of the trunk or lower extremities: however, it is rarely seen on the maxillofacial region. Here, we describe a case of LGFMS on the left lower one-third region of the face of a 35-year-old male patient with a 6-month history. On gross examination, the resected specimen consisted of an open ovoid mass of 2 cm × 2 cm × 1 cm. Light microscopy revealed well-circumscribed myxoid tumor with hypocellular areas in nodules merging to collagenized areas. Immunohistochemical examination revealed diffuse positivity to vimentin, whereas tests for desmin, S-100 protein were negative, thus confirming the diagnosis. After the initial healing of the surgical wound, the patient was advised 30 cycles of radiotherapy. Recurrence and metastasis have not been observed for 1 year of surgical excision now. Due to the notably indolent nature of LGFMS, long-term follow-up is necessary to evaluate its clinical course.

Keywords: Low-grade fibromyxoid sarcoma; maxillofacial region; myxoid tumor; recurrence; wide excision.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Preoperative profile photograph
Figure 2
Figure 2
Extraoral incision
Figure 3
Figure 3
Intraoperative dissection
Figure 4
Figure 4
Closure
Figure 5
Figure 5
Excised lesion
Figure 6
Figure 6
Photomicrograph of excisional biopsy. (a) Atrophic muscle and tumor interface. (b) Cellular areas and myxoid stroma. (c) Collagenous areas and cellular areas. (d) Cellular areas
Figure 7
Figure 7
High-power electron microscopy showing spindle cells
See this image and copyright information in PMC

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