Myopericytoma is a soft-tissue tumor of perivascular cells (pericytes). It is slow-growing, usually asymptomatic, and generally benign, although a malignant variant has been described. The etiology is unknown, but it has been associated with local trauma. The most common location is on the distal extremities. Histologically, it is characterized by a well-circumscribed, non-encapsulated proliferation of spindle shaped cells similar to myofibroblasts with oval nuclei and eosinophilic cytoplasm, arranged in perivascular concentric rings. There are few mitoses and no necrosis is reported. The immunohistochemical analysis is positive for smooth muscle actin and negative or weakly positive for desmin. A low Ki-67 proliferation index is typical. Treatment is surgical excision with free margins. Recurrences after adequate excision are uncommon. We describe a 48-year-old woman with a myopericytoma in an unusual location (next to the inner corner of her left eye) who was treated with surgical excision; there has been no recurrence after 5 years of follow up.
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J Cutan Pathol
August 2024
Division of Dermatology, Department of Internal Medicine, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA.
A neurofibroma with focal glomus-like body differentiation is an unusual phenomenon recently encountered in an excision specimen from the right lateral distal forearm of a 26-year-old man. Glomus cells are modified smooth muscle cells normally present in glomus-like bodies but can also be found in glomus tumors (GT) or lesions considered in the spectrum of GT, including myopericytoma, myofibroma, and angiolipoma. Neurofibromas are peripheral nerve sheath tumors derived from the neural crest cells.
View Article and Find Full Text PDFInt J Surg Pathol
April 2024
Department of Pathology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.
Myopericytoma, a perivascular myoid neoplasm, is commonly found in the dermis or subcutaneous tissues; however, its occurrence in visceral organs is unusual. Here, we present an extremely rare tumor of intravascular myopericytoma of the right renal vein. A 44-year-old man was incidentally diagnosed with a mass in the right kidney during a routine checkup.
View Article and Find Full Text PDFThorac Cancer
September 2022
Department of Experimental, Diagnostic, and Specialty Medicine, S.Orsola-Malpighi University Hospital, University of Bologna, Bologna, Italy.
Myopericytoma is a rare tumor generally arising from skin and soft tissues of extremities, trunk, head, and neck regions, rarely from visceral sites. An intrathoracic visceral localization may carry a broad differential diagnosis including primary lung, pleura and chest wall lesions, or metastatic lesions. To date, any radiological features have been recognized and diagnosis of myopericytoma with intrathoracic localization remains still challenging.
View Article and Find Full Text PDFDermatol Online J
March 2022
Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte, E.P.E., Lisbon, Portugal.
Myopericytoma is an uncommon benign neoplasm that arises from the perivascular myoid cells. It typically presents as a painless well-circumscribed cutaneous or soft-tissue nodule, most commonly on the extremities of adults. Histologically, it is characterized by spindle-shaped myoid-appearing cells with a concentric arrangement in vessel walls, that are immunoreactive to alpha-smooth muscle actin and often for h-caldesmon, but negative for other smooth muscle markers.
View Article and Find Full Text PDFAnn Pathol
November 2021
Département de pathologie, institut Claudius-Regaud, IUCT, 1, avenue Irène Joliot Curie, 31059 Toulouse, France.
Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization.
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