Myopericytoma D21.9

Myopericytoma is a benign, perivascular, myoid soft tissue tumor of adulthood. Myopericytomas occur subcutaneously and cutaneously, particularly in the distal extremities. Myopericytoma is a tumor that has a hemangiopericytoma-like vascular pattern. The starting point is the myoid vessel wall cells (myopericytes).

m:w=2:1;

Unknown. Several times the tumor was observed post-traumatic.

Ubiquitous occurrence, especially in the subcutaneous tissue of the extremities (Datta Vet al. 2007)), also orally (gingiva, tongue - Akbulut S et al. 2013). Occasional cases have also been observed on the face. Rare is the primary infestation of internal organs (kidney - Li Jet al. 2015).

Clinically, they are often painless nodules and lumps that grow slowly over years and can become symptomatic at most by compression of adjacent structures.

Although myopericytoma shares morphologic features with angioleiomyoma, myofibroma, and glomus tumor, it is thought to be a distinct perivascular myoid neoplasm of the skin and soft tissues (Laga AC et al 2008).

The tumor is characterized by a radial and perivascular arrangement of ovoid, spindle-shaped to round neoplastic cells that are immunoreactive for alpha-smooth muscle actin, often also for h-caldesmon, and smooth muscle myosin heavy chain and usually negative for desmin antibodies. Ossification of the tumor parenchyma is possible.

Depending on the predominant morphology, several subtypes are distinguished:

• infantile and adult myofibroma
• myofibromatosis (with leiomyoma or hemangiopericytoma-like structure)
• glomangiopericytoma (similar to glomus tumor or hemangiopericytoma)

Most cases of myopericytomas are benign, although local recurrence and malignancy have been reported. Myopericytomas may be multifocal and involve a single or multiple anatomic regions.

1. Akbulut S et al. (2013) Myopericytoma of the tongue: a case report. Acta Medica (Hradec Kralove) 56:124-125.
2. Almeida LKY et al.(2020) Gingival ossifying myopericytoma in a pediatric patient: immunohistochemical analysis and literature review. Oral Oncol 107:104826.
3. Datta Vet al (2007) Myopericytoma of the oral cavity. Head Neck 29:605-608.
4. Kiyohara T et al. (2016) CD34-positive infantile myofibromatosis: Case report and review of hemangiopericytoma-like pattern tumors. J Dermatol 43:1088-1091.
5. Laga AC et al (2008) Myopericytoma: report of two cases associated with trauma. J Cutan Pathol 35:866-870.
6. Li Jet al (2015) Renal myopericytoma: a clinicopathologic study of six cases and review of the literature. Int J Clin Exp Pathol 8:4307-4320.
7. Mentzel T et al (2006) Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol 30: 104-113.
8. Porat Ben Amy D et al (2021) Oral myopericytoma: a rare pediatric case report and a review of the literature. BMC Oral Health 21:176.