Intrathoracic glomus tumors and glomangiosarcomas: a clinicopathological and immunohistochemical study of 14 cases with emphasis on anatomic distribution

Fourteen cases of primary intrathoracic glomus tumor and glomangiosarcoma are presented. The patients were 9 men and 5 women aged 36 to 63 years (mean, 49.5 years). Clinically, 11 patients presented with nonspecific symptoms including cough, hemoptysis, chest pain, and back pain while 3 patients were asymptomatic. Anatomically, 9 tumors were in an intrapulmonary location, 3 in the mediastinum, and 2 in the trachea. Surgical resection was performed in all cases, and grossly the tumors varied in size from 1.5 to 4.5 cm in greatest diameter. Histologically, 12 cases corresponded to glomus tumor characterized by a homogenous proliferation of medium-sized cells with round nuclei and inconspicuous nucleoli and no evidence of mitotic activity or necrosis. In 2 cases, the presence of marked cellular pleomorphism, nuclear atypia, and mitotic activity was more in keeping with a diagnosis of glomangiosarcoma. The 2 glomangiosarcomas were located in the lung, one of which was associated with lymph node metastasis. Immunohistochemical stains for smooth muscle actin were positive in all cases while negative for epithelial, vascular, neuroendocrine, and neural markers. Clinical follow-up showed that all patients with a glomus tumor are alive and well after a period of 12 to 36 months after surgical resection. In contrast, the 2 patients with glomangiosarcoma died of tumor 24 and 36 months, respectively, after initial diagnosis. The current cases highlight the ubiquitous distribution of these tumors within the thoracic cavity and stress the importance of including these tumors in the differential diagnosis of thoracic neoplasms.