Pediatric upper lip myopericytoma: a case report and comprehensive review.

BACKGROUND: Myopericytoma is a rare spindle cell tumor of mesenchymal origin, typically benign, characterized by concentric proliferation of tumor cells around blood vessels within subcutaneous tissue. It primarily occurs in middle-aged adults and is often located in distal extremities, although cases have been reported in proximal extremities and head-neck regions. However, occurrences within the oral cavity are exceedingly rare. To date, literature reviews have identified only two cases in children under 10 years old and reported only five cases of myopericytoma occurring in the lip region. We provide a comprehensive review and analysis of all documented cases to better understand this condition. CASE PRESENTATION: A 7-year-old girl presented to oral and maxillofacial surgery with the discovery of a painless mass on the inner aspect of the upper lip. The diagnosis of myopericytoma was confirmed by histological examination (HE staining), alcian blue staining, and immunohistochemistry. CONCLUSIONS: Following surgical excision, there were no signs of recurrence at a 3-month follow-up. The pathological diagnosis of myopericytoma is quite challenging, and immunohistochemical testing is necessary.

Thoracic myopericytoma in an older adult, rare but possible: A case report.

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. Here, we describe the case of a subpleural lesion incidentally diagnosed in an older adult affected by gastric cancer. Radiological features did not allow a differential diagnosis between a benign lesion, a primary tumor, or a metastasis. After resection, the histological examination showed histopathological features congruent with the diagnosis of myopericytoma. This unusual presentation reflects the need to share clinical, radiological, and histopathological data about this uncommon but frequently misdiagnosed disease.

Myopericytoma of the Parotid and Molecular Profiling: Report of a Rare Case and Review of the Literature.

Myopericytomas are uncommon tumors defined by their round to spindle shaped cells often arranged in a concentric pattern of perivascular growth. They are typically well-circumscribed, nodular, slow-growing lesions that occur in the soft tissue of the extremities. Here, we present a 30-year-old female with a 2.4 cm myopericytoma occurring in the deep lobe of the parotid gland. The diagnosis was made with detailed histopathologic and immunohistochemical findings and positive identification of the specific mutation for PDGFRß p.Asp666Lys by next generation sequencing (NGS). This is the first case report of a parotid myopericytoma with a genetic testing that shows a particular mutation that has been linked to myopericytomatosis.

Miopericitoma mitóticamente activo / Mitotically Active Myopericytoma

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Clinico-pathological features of gynecological myopericytoma: a challenging diagnosis in an exceptional location.

Myopericytomas (MPC) are rare mesenchymal tumors, originating from the perivascular myoid cells. They predominantly occur in the skin and superficial soft tissues of the extremities, while visceral involvement is rare. Histological features and clinical course are usually benign. To the best of our knowledge, MPC is still an uncharacterized tumor entity of the female internal genital tract. We describe three MPC cases involving the female internal genital tract: (1) a uterine wall MPC arising in a 49-year-old woman with progressive pelvic/abdominal pain; (2) a cervix MPC of a 49-year-old woman who presented with metrorrhagia, and (3) a MPC presenting as a simple ovarian cyst in a 26-year-old woman with pain located in the left iliac fossa. All patients were surgically treated, and recurrence occurred in two cases. The histological and immunohistochemical findings, supporting the diagnosis of MPC, are presented; in particular, one case showed characteristics pointing towards an uncertain biological behavior/low-grade malignancy. A literature search was conducted to identify previous reports of gynecological MPC and for possible alternative diagnoses. Leiomyoma, epithelioid leiomyoma, angioleiomyoma, perivascular epithelioid cell tumor, solitary fibrous tumor, and low-grade endometrial stromal sarcoma should be considered in the differential diagnosis. Awareness of possible occurrence of this rare neoplasm in the female genital tract is important to reach a correct diagnosis in the spectrum of mesenchymal tumors. Considering the risk of recurrence, we recommend careful evaluation of surgical margins and complete surgical removal whenever possible.

Clinicopathologic features in a case of intermuscular myopericitoma of thigh.

Myopericytoma is a benign tumor with the most common presentation as a well-circumscribed, slow-growing mass. It is frequently misdiagnosed as a sarcoma. We presented a 23-year-old patient with a history of a sciatic pain of the right leg. A careful physical examination discovered tumor-like mass in the posterior part of the thigh. Neurological finding showed a reduction of myotatic reflexes on the right leg with a weaker muscle strength on the right leg. The right leg musculature was slightly hypotrophic in the range of 2-3 cm comparing to left leg. Initially electrophysiological and radiological diagnostic with magnetic resonance imaging (MRI) of the lumbar spine, pelvis and thighs were normal. Magnetic resonance imaging of the right thigh discovered a slow growing 2.1 × 3.8 cm sized mass that was initially described by radiologist as a neurinoma. Patient was admitted to department of neurosurgery and operated on for a tumor removal. Tumor was located intimately to femur and sciatic nerve and after careful dissection completely removed. Patient was doing well after surgery and discharge after three days from the hospital. In the postoperative period the symptoms disappeared. Histopathology showed a myopericitoma. Postoperative MRI after three months of follow up showed no tumor residues, and after 6 and 12 months there was no tumor recurrence. Myopericytoma behave in a benign fashion, but, because local recurrences and rarely metastases may occur in atypical and malignant neoplasms, a careful follow-up after radical resection is recommended.

Glomangiopericytoma-type glomus tumour/myopericytoma of the lip.

We describe the case of a 46-year-old man who presented with a four-month history of a reddish, otherwise asymptomatic, nodular lesion that involved the mucosal side of the upper lip. The lesion consisted of myoid glomus-like cells arranged around branching and thin-walled haemangiopericytoma-like vascular structures, and was classified as glomangiopericytoma. No recurrences occurred during an eight-month follow-up. To the best of our knowledge, this tumour has never previously been described in the mucosa of the lip.

Clinical and pathologic analysis of myopericytoma in the oral and maxillofacial region.

OBJECTIVE: The aim of this study was to analyze myopericytoma in the oral and maxillofacial region in terms of clinical appearance, diagnosis, treatment, and outcomes. STUDY DESIGN: Data on 5 new patients with myopericytoma in the oral and maxillofacial region treated at our department were collected and analyzed. RESULTS: There were 2 males and 3 females (age range 10-62 years; mean age 43.8 years). All of the 5 patients presented with masses showing benign biologic behavior. Imaging examinations with use of computed tomography or magnetic resonance imaging showed heterogeneous regions with internal contrast-enhancement or cystic change in 3 cases. All of the patients underwent surgery. Histologic examination showed a broad morphologic spectrum characterized by concentric and perivascular growth of ovoid, plump spindled, and/or round myoid tumor cells. Immunohistochemical examination showed positive staining for vimentin and smooth muscle actin, and negative for CD34 and desmin. During the follow-up period (8-56 months), there was no tumor recurrence. CONCLUSIONS: Myopericytoma in the oral and maxillofacial region always exhibits benign biologic behavior and a heterogeneous region with internal contrast-enhancement or cystic change on imaging examinations. Surgery is the first choice of treatment and results in good clinical outcomes.

Myopericytoma of the Lip: A Case Report of This Rare Lesion.

Myopericytomas are rare benign tumors that show a distinctive, concentric perivascular proliferation of oval to spindle-shaped myoid-appearing cells that are predominantly arranged concentrically around thin-walled vascular channels. These lesions are similar in histologic appearance to hemangiopericytomas, myofibromas, and glomus tumors. The myopericytoma is usually found in the distal extremities, but a handful of reports have shown the lesion to be present in the oral cavity. A review of the literature to date shows only 4 other reported cases of myopericytomas occurring in the lips. We describe a case of myopericytoma in the lower lip of a 42-year-old woman.