Pulmonary and mediastinal glomus tumors - Report of five cases including apulmonary glomangiosarcoma: A clinicopathologic study with literature review

Pulmonary and mediastinal glomus tumors are rare lesions, with four previou sly reported primary pulmonary cases and three mediastinal cases. The autho rs report one mediastinal glomus tumor, a locally infiltrative type, and fo ur pulmonary glomus tumors, including the first case of primary pulmonary g lomangiosarcoma. These tumors show a variety of clinical and pathologic dif ferences from the more common cutaneous variety, including later age at pre sentation, larger size, and more frequent atypical/malignant features. Medi astinal and pulmonary glomus tumors both have an average patient age at pre sentation of 45 years. However, compared with their pulmonary counterparts, mediastinal glomus tumors are less common, more often symptomatic, and an larger (average size, 5.4 cm). Additionally, mediastinal glomus tumors more often demonstrate malignant or atypical features, Pulmonary glomus tumors average 3.3 cm in greatest dimension, with the majority measuring less than 2.5 cm. The pulmonary glomangiosarcoma presented was large, measuring 9.5 cm, and showed increased mitotic count (9 mitoses/10 high-power fields), ne crosis, cytologic atypia, and was associated with disseminated disease. Reg ardless of clinical symptoms, histologic features, and even metastases, the vast majority of all benign and malignant glomus tumors are indolent and c ured surgically, with adjuvant therapy needed only for occasional patients with more advanced disease. The four patients with glomus tumors reported a re currently alive and free of disease as of last follow up. The patient wi th the glomangiosarcoma developed widespread metastases and died of disease 68 weeks after initial therapy.