Congenital myofibroma of the skin mimicking a piloleiomyoma

Myofibroma is an uncommon benign soft tissue disorder, which is usually congenital or present in early infancy. Myofibroma usually manifests as a single mass. When there are multiple lesions, the term myofibromatosis is used. The characteristic histopathological feature of the myofibroma is the coexistence of two distinct areas. One area mainly contains plump spindle cells with thin blunt‐ended nuclei and eosinophilic cytoplasm, thus indicating myoid characteristics. The other area contains either round or polygonal cells with slightly pleomorphic, hyperchromatic nuclei or small spindle cells typically arranged around a distinct hemangiopericytoma‐like vascular pattern. In the present case, the majority of the tumor was composed of the plump myoid spindle cells. This led to an initial diagnosis of a piloleiomyoma. However, the tumor cells were not immunohistochemically positive for desmin. Moreover, careful examination revealed a hemangiopericytoma‐like vascular pattern characterized by the presence of high cellular areas with irregular vascular spaces. These features led to the final diagnosis of the myofibroma. It is therefore important to recognize the leiomyoma‐like variants of myofibromas. Inoue T, Sada A, Mori T, Misago N, Narisawa Y. Congenital myofibroma of the skin mimicking a piloleiomyoma.