Malignant Familial Glomus Jugulare Tumor: A Case Report

Abstract

Paragangliomas originate from neural crest-derived tissue. In the head and neck, paragangliomas occur most frequently in the carotid body and in the jugulotympanic region. At this last site they are classified by their origin into glomus jugulare and glomus tympanicum tumors. Malignant paragangliomas are distinctly uncommon. Histological criteria do not predict the clinical behavior of paragangliomas, and their malignant potential is notoriously difficult to predict. The diagnosis of malignancy is made clinically by the presence of metastatic spread and by a rapid, invasive growth. Familial glomus tumors constitute approximately 20% of cases, and in children of men who had a glomus tumor, these lesions developed at a rate consistent with autosomal-dominant inheritance (50%). Al-Mefty and Teixeira in 2002 classified as “complex glomus jugulare tumors” a subgroup of tumors that is rarely encountered and seldom reported, and that represents a challenge to treatment. To be classified as complex, one or more of the following criteria had to be met: giant size, multiple paragangliomas, malignancy, catecholamine secretion, association with other lesion, previous treatment with adverse outcome, radiation therapy, or adverse effects from embolization. In this report we describe a case of glomus jugulare tumor with cervical lymph nodes metastases in a 25-year-old woman with positive familiar history (father with concomitant glomus jugulare and contralateral carotid body tumor). The work-up and surgical treatment of this patient is discussed in detail and is used to explore controversial aspects of treatment of paragangliomas of the head and neck.