Abstract
Purpose
To analyze treatment results in the multidisciplinary management of patients with multiple head and neck paragangliomas (HNPs).
Methods
Retrospective analysis including all patients with multiple HNPs (VP, vagal paraganglioma; JTP, jugulotympanic paraganglioma; CBT, carotid body tumor) treated between 2000 and 2013 at a tertiary referral center.
Results
Ten patients (three men, seven women) had 25 HNPs (two VPs, eight JTPs, and 15 CBTs). The age range at diagnosis was 31–71 years (mean 40.9 years, median 37 years). Nine tumors (four CBTs, three JTPs, two VPs) were treated only with stereotactic radiotherapy (SRT; 50.4–56 Gy, mean 55.3 Gy) or in one case intensity-modulated radiotherapy (60 Gy). Nine tumors were treated with surgery alone (eight CBTs, one JTP) and three JTPs with subtotal surgery combined with adjuvant SRT. A “wait and scan” strategy was used in three cases (two CBTs, one JTP). The mean follow-up period was 4.3 years (range 0.1–13 years, median 4 years). The rate of tumor control with surgery and/or SRT was 100% (21/21). One patient with a wait-and-scan strategy for CBT had slow asymptomatic progression during a 13-year follow-up.
Conclusions
The treatment results in this series of patients with multiple HNPs show that a very high rate of long-term tumor control with low morbidity can be achieved using tailored and individualized approaches. All of the different treatment strategies available should be discussed with the patient. In particular, the treatment should involve a multidisciplinary team of experts in the fields of nuclear medicine, genetics, pathology, radiology, radio-oncology, and surgery.
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Introduction
Head and neck paragangliomas (HNPs) are rare tumors, representing less than 0.5% of all head and neck tumors. Approximately 3% of all paragangliomas occur in the head and neck area . Paragangliomas in the head and neck region are highly vascularized tumors, which in the majority of cases are benign. The incidence is two to five times higher in women. The age at manifestation is between 40 and 60 . Paragangliomas only show histopathological signs of malignancy or metastases to nonendocrine tissue in approximately 3% of cases. The mean tumor doubling rate is 4.2 years , and the mean growth rate is only approximately 0.2 cm per year . They originate in paraganglionic tissue in the area of the carotid bifurcation (carotid body tumors, CBTs), the jugular foramen and tympanic plexus (jugulotympanic paragangliomas, JTPs), the vagal nerve (vagal paragangliomas, VPs), and the facial nerve . HNPs may occur either sporadically or in the context of a hereditary familial tumor syndrome. Multilocular presentations of glomus tumors are observed in 10–20% of sporadic cases and up to 80% of hereditary cases. Hereditary HNPs are mostly caused by mutations in the succinate dehydrogenase complex ( SDHx ) genes, in particular SDHD .
Currently, there is no standard therapeutic protocol in patients with multiple paragangliomas and some patients thus end up with overtreatment, while others are undertreated. The aim of the present study was to analyze and provide treatment results in the multidisciplinary management of patients with multiple HNPs.
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