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Review
. 2026 Feb;53(2):169-174.
doi: 10.1111/cup.70010. Epub 2025 Nov 15.

TRPS1::PLAG1 Fusion in a Primary Cutaneous Myoepithelial Carcinoma: A Case Report and Literature Review

Timber Gillis  1 , Jenika Howell  1 , Fatemeh Jafarian  1
Affiliations
Review

TRPS1::PLAG1 Fusion in a Primary Cutaneous Myoepithelial Carcinoma: A Case Report and Literature Review

Timber Gillis et al. J Cutan Pathol. 2026 Feb.

Abstract

Myoepithelial neoplasms are rare tumors that are found in the salivary glands, the breast, soft tissue, and skin. Myoepithelial carcinomas typically have a poor prognosis with a high rate of local recurrence and metastasis in the cutaneous setting. There are fewer than 30 described cases of primary cutaneous myoepithelial carcinoma in the literature. We performed a literature review and identified 26 known cases of primary cutaneous myoepithelial carcinoma. Here, we describe a case of occipital primary cutaneous myoepithelial carcinoma occurring in a 56-year-old patient. RNA sequencing was performed on the tumor which identified a TRPS1::PLAG1 fusion event which is to our knowledge, the first time this has been reported in the literature for a primary cutaneous myoepithelial carcinoma.

Keywords: PLAG1; RNA; TRPS1; myoepithelial carcinoma; sequence analysis; skin neoplasms.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Clinical photograph of the occipital, ulcerated mass measuring 15 cm in maximum dimension.
FIGURE 2
FIGURE 2
(A) Hematoxylin and eosin (H&E) stain of a representative section of the resection specimen denoting cystic degeneration and infiltrative borders (40×). (B) CK7 immunohistochemical stain of a representative section (40×). (C) Area of ductal differentiation within the tumor (H&E, 200×). (D) p63 immunohistochemical stain of a representative section (40×). (E) Area of brisk mitotic activity and discohesive, epithelioid cells with atypia and atypical mitoses within the tumor (H&E, 400×). (F) S100 immunohistochemical stain of a representative section (40×).
See this image and copyright information in PMC

References

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