Pediatric upper lip myopericytoma: a case report and comprehensive review. 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. A 7-year-old girl presented to oral
Intracranial malignant myopericytoma: case report and literature review. Malignant myopericytoma is an exceedingly rare soft tissue tumour with only 14 documented cases in the scientific literature. Myopericytomas can occur in association with HIV-AIDS and have a predilection for internal organs such as the bronchus, larynx, liver and brain. Ebstein-Barr virus encoded small RNA(EBER) was positive in three out of 14 cases. In this case report we present the second documented case of a malignant myopericytoma, with a positive EBER found intracranially - specifically in the supratentorial compartment - invading the superior sagittal sinus. Gross total excision of the tumour was achieved, and the patient responded well with no recurrence with only highly active antiretroviral therapy (HAART
Myopericytoma of the Base of the Finger: Radiological and Pathological Description of a Rare Benign Entity. A previously healthy 46-year-old woman presented with a mass lesion between the bases of the fourth and fifth fingers of the right hand. The mass had grown progressively over 2 years and started to cause practical difficulties in everyday life. Imaging depicted a hypervascular and well -circumscribed soft tissue tumor with imaging characteristics of a sarcoma. The lesion was treated surgically. The final diagnosis of the specimen upon pathology was a myopericytoma, a benign smooth-muscle cell neoplasm. Myopericytoma is a rare disease entity; however, it is important because it can mimic more ominous conditions.
Surgical Treatment of Primary Pulmonary MyopericytomaMyopericytoma is a benign tumor that occurs in soft tissues. Myopericytoma in the lungs is very rare. We report the case of a 63-year-old woman presenting with cavitary masses in the left lung and multiple tiny nodules in both lungs. She underwent surgery, and a histological examination revealed primary pulmonary myopericytoma.
Recurrent SRF-RELA Fusions Define a Novel Subset of Cellular Myofibroma/Myopericytoma: A Potential Diagnostic Pitfall With Sarcomas With Myogenic Differentiation. Cellular myofibroblastic tumors other than desmoid-type fibromatosis are often diagnostically challenging due to their relative rarity, lack of known genetic abnormalities, and expression of muscle markers which may be confused with sarcomas with myogenic differentiation. In this study we investigate the molecular alterations of a group of cellular myofibroblastic lesions with in the myofibroma and myopericytoma spectrum for better subclassification. Two index cases were studied by paired-end RNA sequencing for potential fusion gene discovery. One chest wall soft tissue tumor in a 3-month-old girl case showed a SRF-C3orf62 fusion
Myopericytoma occurrence in the liver and stomach space: imaging performance. Myopericytoma is a rare and usually benign tumor, which is even rarer if it occurs in the liver and stomach space. Previous reports of myopericytoma were mostly related to its pathological manifestations, while imaging reports were rare. Here, we report the computed tomography (CT), computed tomography angiography (CTA ) and magnetic resonance imaging (MRI) performance for one deep myopericytoma. In this study, one deep myopericytoma in the liver and stomach space is reported. A CT that was not contrast-enhanced showed a lobulated tumor with heterogeneous density, and a contrast-enhanced CT showed that the mass had progressive enhancement. CTA showed that the blood-supply of the tumor was supplied by the anterior superior
Myopericytomatosis: Clinicopathologic Analysis of 11 Cases With Molecular Identification of Recurrent PDGFRB Alterations in Myopericytomatosis and Myopericytoma. Myopericytoma is a benign tumor of concentrically distributed perivascular myoid cells. Its molecular basis and relationship with myofibroma/myofibromatosis and other pericytic tumors are not fully understood. In our consultation , ranging from 1.5 to 11.0 (median, 6.0) cm in size. Histologically, myopericytomatosis displayed diffuse infiltration by innumerable discrete myopericytoma/myofibroma-like nodules of bland spindled-to-ovoid cells (smooth muscle actin positive), in a mainly perivascular distribution. No mitoses, atypia, or necrosis was noted. All patients were treated by surgical excision (1 patient also received adjuvant
Primary malignant myopericytoma with cancer cachexia: Report of the first case and review of literature. Malignant myopericytoma is extremely rare, with a few cases described in the English literature. This novel study aimed to report a case of malignant myopericytoma with cancer cachexia arising in the left armpit. Also, it presented a review of the English literature regarding primary malignant myopericytoma, aiming to clarify the clinical features and potentially curative treatment. A 56-year-old male presented with an ulcerated and smelly mass involving her left armpit. The patient had obvious symptoms of cancer cachexia, including emaciation, anemia, and lower extremity edema. Computer tomography (CT) scan demonstrated a mass in the left armpit, with no evidence of metastasis
Myopericytoma/myopericytomatosis of the lower extremity in two young patients: a recently designated rare soft tissue neoplasm Myopericytomas are rare, slow-growing benign perivascular tumors most commonly arising within the superficial subcutaneous soft tissues of the lower extremity. They represent one of several related perivascular tumors of myoid lineage with similar morphology and shared immunohistochemical profile including positive staining for smooth muscle actin. Histologically, myopericytoma exhibit concentric, perivascular proliferation of spindled myoid cells with bland elongated nuclei and associated blood vessels. A solitary well-demarcated nodule or mass is typically referred to as myopericytoma, whereas an infiltrative multinodular lesion has more recently been termed myopericytomatosis
Foot soft tissue myopericytoma: Case-report and review Myopericytoma is a rare tumor of deep soft tissues, originating from pericytes and characterized by numerous thin walled blood vessels. We report a case of myopericytoma found at the level of the second toe of the right foot.A patient came to the Endocrinology Surgery Department of Catania Polyclinic because of a presence of a small swelling in the plantar region, between the 2nd and 3rd toe of the right foot. At the anatomopathological examination, the escalated lesion showed a neoformation of 0.6 cm in diameter, well circumscribed, capsulated, with myopericytoma diagnosis. Its histopathological appearance is similar to myofibromatic lesions from glomic and angiomyoma tumors. It is a rare tumor that affects all ages with a peak after 50 years 3
Myopericytoma in urinary bladder: a case report Myopericytoma is reported to occur mainly in the skin and superficial soft tissue of the extremities. In contrast, occurrence in the urinary bladder is extremely rare. We describe a 75-year-old Japanese man who developed a submucosal tumor at the right trigone of his bladder that led to interference with the discharge of right ureteral calculus . Cystoscopic and pathological findings were compatible with a diagnosis of myopericytoma of the urinary bladder.
miRNA expression profiling divides follicular dendritic cell sarcomas into two groups, related to fibroblasts and myopericytomas or Castleman's disease. Follicular dendritic cell (FDC) sarcomas are rare mesenchymal tumours, which are fatal in 20% of the patients and usually occur in secondary lymphoid organs or extranodal localizations. Due to the rareness of these tumours, only few studies have been conducted on molecular level. In the present study, we performed microRNA (miRNA) profiling of 31 FDC sarcomas and identified two subgroups, one with high miRNA expression and the other group with low miRNA expression levels. The first group showed a strong similarity to fibroblasts and myopericytomas, whereas the second group was more closely related to FDCs from Castleman's disease. Both
Recurrent Somatic PDGFRB Mutations in Sporadic Infantile/Solitary Adult Myofibromas But Not in Angioleiomyomas and Myopericytomas. Infantile myofibroma (MF) is an uncommon benign myofibroblastic tumor of infancy and childhood. Solitary adult MF shares similar features with infantile MF. The lesions occur in 3 clinicopathologic settings: solitary, multicentric, and generalized and can be either sporadic or familial. Traditionally, infantile MF has been included in the spectrum of infantile hemangiopericytoma. The recent World Health Organization classification listed MF, angioleiomyoma, and myopericytoma under the general heading of perivascular tumors in the sense of a morphologic spectrum of perivascular myoid cell neoplasms. Although activating germline PDGFRB mutations have recently been
Malignant Myopericytoma of Shoulder: A Rare Lesion Myopericytoma is a soft tissue tumor with perivascular myoid differentiation. It accounts for 1% of the vascular tumors and involves mostly cutaneous or subcutaneous tissue of the limbs in adults. Malignant myopericytoma is exceedingly rare. A 15-year old girl presented with slowly progressive mass over left shoulder region. Histopathology and immunohistochemistry after complete excision revealed it as malignant myopericytoma.
Case Report: A giant myopericytoma involving the occipital region of the scalp - a rare entity Herein we report a rare case of a giant myopericytoma presenting in a 16-year-old girl as a slowly progressive swelling involving the scalp in the occipital region. It was managed by complete excision. Histological examination of the lesion revealed spindle-shaped cells forming characteristic rosettes
Intravascular myopericytoma in the heel: case report and literature review. Intravascular myopericytoma (IVMP), regarded as a variant of myopericytoma, is a rare tumor. Very few cases have been described, none in the foot.The first case of IVMP located in the heel of the foot is described in this article. A literature review is reported of all cases of IVMP published in the English literature.A
Myopericytoma of the posterior cranial fossa. Myopericytoma is a soft tissue tumour believed to be derived from perivascular myoid cells. They are typically found in subcutaneous tissues in the extremities. Intracranial myopericytomas are exceptionally rare. Here we report a man with an asymptomatic posterior fossa myopericytoma with evidence of dural infiltration.
Role of BRAFV600E in the First Preclinical Model of Multifocal Infiltrating Myopericytoma Development and Microenvironment. Myopericytoma (MPC) is a rare tumor with perivascular proliferation of pluripotent stem-cell-like pericytes. Although indolent, MPC may be locally aggressive with recurrent disease. The pathogenesis and diagnostic biomarkers of MPC are poorly understood. We discovered
Myopericytoma around the knee: mimicking a neurogenic tumour. Myopericytoma is a rare, usually benign soft tissue tumour that arises most commonly in the dermis and subcutaneous soft tissue of the lower extremities. In this article, we present an unusual case of myopericytoma occurring in the deep soft tissue around the knee that mimicked a neurogenic tumour. Myopericytoma can be confused with neurogenic tumour when located in deep soft tissue and adjacent to nerve bundles. Preoperative MRI and colour Doppler ultrasound may help differentiate myopericytoma from neurogenic tumour by revealing the hypervascular nature of the tumour.
Myopericytoma of the external auditory canal and tragus. Myopericytoma is a relatively recently described skin and soft tissue tumour that demonstrates perivascular myoid cell or pericytic differentiation. Whilst the range of anatomical locations has expanded to include visceral locations, head and neck myopericytomas are rarely documented. There have been no previous reports of aural myopericytoma. This paper reports the clinicopathological features of a biopsy-proven, slow-growing, 20 × 20 mm, polypoid myopericytoma that involved the external auditory canal and tragus in an 18-year-old woman. Excision was curative. Heightened clinicopathological awareness of the expanding anatomical distribution of myopericytoma is critical to its diagnosis when it presents in unusual and novel locations