Intrathoracic Myopericytoma: A Scoping Review of Clinical, Radiological, and Histopathological Features

Background: Myopericytoma is a rare perivascular soft tissue tumor that predominantly arises in the distal extremities. Primary involvement of the thoracic cavity is exceptionally uncommon, and existing evidence remains limited to isolated case reports, hindering diagnostic recognition and management guidance. Method: A scoping review was conducted in accordance with PRISMA ScR guidelines. PubMed, Scopus, Web of Science, ProQuest, ScienceDirect, and Google Scholar were systematically searched from inception to December 2025. Studies reporting primary intrathoracic myopericytoma were included, and clinical, radiological, pathological, management, and outcome data were synthesized. Result: Ten studies encompassing 11 patients were identified. Tumors involved the lung (45.5%), pleura or subpleural region (27.3%), heart, mediastinum, and chest wall (each 9.1%). Most lesions were incidentally detected (63.6%) and demonstrated benign histopathological features (63.6%). Radiologically, benign tumors appeared as well circumscribed hypervascular masses, whereas malignant cases showed infiltrative growth, necrosis, or metastatic disease. All tumors exhibited characteristic concentric perivascular myoid growth and uniform smooth muscle actin positivity, with consistent negativity for STAT6 and desmin when reported. Complete surgical excision resulted in durable local control in benign disease, while malignant or diffuse variants were associated with rapid progression and poor survival despite intervention. Conclusion: Intrathoracic myopericytoma represents a distinct but underrecognized thoracic neoplasm with a clear prognostic dichotomy. Early diagnostic recognition and complete surgical resection are decisive for favorable outcomes, whereas malignant variants remain associated with dismal prognosis, underscoring the urgent need for molecularly driven therapeutic strategies.