Abstract
Background: Glomus tumors are uncommon vascular tumors of the hand. Malignant glomus tumors, known as glomangiosarcomas, are very rare and offer management challenges. Methods: A review of the diagnosis and treatment of malignant glomus tumors is presented in along with a report of the largest malignant glomus tumor of the hand to date. Results: The review illustrates the management of glomangiosarcomas. Only 6 previous such cases have been reported. Conclusion: The rare nature of malignant glomus tumors mandates case presentation and longitudinal analysis to help guide surgical management an optimize outcomes.