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Case Reports
. 2024 Feb 29:16:20363613241234201.
doi: 10.1177/20363613241234201. eCollection 2024.

Primary lung low-grade fibromyxoid sarcoma: A rare case with A diagnostic dilemma

Aida Ayadi  1 , Yoldez Houcine  1 , Chirine Moussa  2 , Houda Rouis  2 , Tarek Abid  3 , Begueret Hugues  4 , François Le Loarer  5
Affiliations
Case Reports

Primary lung low-grade fibromyxoid sarcoma: A rare case with A diagnostic dilemma

Aida Ayadi et al. Rare Tumors. .

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a rare, low-grade malignant soft tissue tumor that is often mistaken for benign or more rarely malignant tumor types. Commonly, this tumor affects young adults and typically arises in the deep proximal extremities or trunk with frequent recurrences and can metastasize to the lungs many years late. Visceral LGFMS is extremely rare. Only a few cases of primary LGFMS of the lung have been reported. Here, we present the clinical, gross, microscopic, and immunohistochemical characteristics of Evans tumor occurring in the lung with a review of the literature and discuss the differential diagnosis in this exceptional localization.

Keywords: Evans tumor; Muc 4; low-grade fibromyxoid sarcoma; lung soft tissue sarcomas.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
The tumor was endobronchial with an extension into the surrounding lung parenchyma and no attachment to the visceral pleura. The cut surface had a whorled white tan appearance with focal myxoid and hemorrhage changes.
Figure 2.
Figure 2.
Crisscrossing fascicles of bland spindle tumor cells within hyaline to myxoid stroma that tends to vary in different areas of the tumor.
Figure 3.
Figure 3.
The neoplastic cells showed strong nuclear immunoreactivity for MUC4.
See this image and copyright information in PMC

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