Malignant glomus tumors of the head and neck in children and adults: Evaluation and management.

OBJECTIVES/HYPOTHESIS: To describe our current multidisciplinary approach to pediatric malignant glomus tumors of the head and neck and review the current literature.

STUDY DESIGN: Retrospective chart review at a tertiary referral children's hospital and a comprehensive literature review.

METHODS: A comprehensive literature search of PubMed, Embase, Web of Science, Google Scholar, and EBSCO with respect to malignant glomus tumors of the head and neck was conducted. We obtained expert input from other pertinent specialties, including oncology, pathology, and radiology. To highlight the difficulty of evaluation and management of these patients, we also present a pediatric patient with a left neck malignant glomus tumor and lung metastases.

RESULTS: Only two cases of pediatric malignant glomus tumor (including our own) have been reported in the English literature. Overall, 14 malignant glomus tumors have been reported in the head and neck (11 primary and three metastatic). Surgical resection is the mainstay of treatment, but local recurrence is common (five of 11, 45%).

CONCLUSIONS: Malignant glomus tumor of the head and neck is an extremely rare tumor in children. Evaluation consists of imaging, and tissue biopsy is necessary for definitive diagnosis. Management options include surgical resection with or without an adjuvant chemotherapy protocol similar to those designed for sarcoma. Additional reports are necessary so that we may determine the utility, if any, of radiotherapy in the management of this tumor. Laryngoscope, 127:2873-2882, 2017.