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. 2022 Jun;16(2):434-443.
doi: 10.1007/s12105-021-01380-y. Epub 2021 Sep 24.

Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases

Sandra Gjorgova Gjeorgjievski  1 , Karen Fritchie  1 , Judith Jebastin Thangaiah  2 , Andrew L Folpe  2 , Nasir Ud Din  3
Affiliations

Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases

Sandra Gjorgova Gjeorgjievski et al. Head Neck Pathol. 2022 Jun.

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon mesenchymal tumor usually arising in the lower extremities and trunk. Only rare examples in the head and neck region have been reported. Fifteen cases of head and neck LGFMS were retrieved. MUC4 was performed on all cases. Results for smooth muscle actins, β-catenin, desmin, S100 protein, Epithelial membrane antigen (EMA) and STAT6 immunohistochemistry, as well as FUS rearrangement status, were recorded when available. Sites included neck (8), supraclavicular region (4) and orbit (1), parapharyngeal space (1) and lower lip (1). The age of the patients ranged from 3 to 97 years (median, 26 years). Tumors displayed classical morphologic features of LGFMS, as described. All cases (15/15) were positive for MUC4, and all cases tested (4/4) harbored FUS rearrangement. Variable positivity for EMA was identified in one case. Follow-up was available in 11 patients, ranging from 2 to 240 months (mean 71.4 months; median, 44 months). Three tumors recurred locally; none metastasized. In conclusion, although distinctly uncommon, LGFMS may arise in the head and neck region and should be distinguished from other more common spindle cell tumors in these locations. The morphologic, immunohistochemical and molecular genetic features of head/neck LGFMS are identical to those occurring elsewhere. The long-term metastatic risk of LGFMS in these locations remains to be fully elucidated.

Keywords: Head and neck; Lip; Low-grade fibromyxoid sarcoma; MUC4; Orbit; Parapharyngeal space; Supraclavicular.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Gross appearance of low-grade fibromyxoid sarcoma. The tumor is circumscribed with firm grey, white cut surface
Fig. 2
Fig. 2
LGFMS of Neck. A relatively circumscribed tumor in the dermis with unremarkable overlying epidermis (A H&E*, 10×). Tumor interface with adnexal structures (B H&E*, 20×). Transition between alternate myxoid and collagenized areas (C H&E*, 40×). Whorled areas containing arcade of vessels and short fascicles of relatively bland spindle cells (D H&E*, 40×) *Hematoxylin and eosin
Fig. 3
Fig. 3
Hematoxylin and eosin-stained section shows higher magnification of giant collagen rosettes (A H&E*, 40×). MUC positivity in tumor cells (B)
See this image and copyright information in PMC

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