Head and neck paragangliomas: A two‐decade institutional experience and algorithm for management

Abstract

ObjectivesParagangliomas of the head and neck and cranial base are typically benign, slow‐growing tumors arising within the jugular foramen, middle ear, carotid bifurcation, or vagus nerve proper. The objective of this study was to provide a comprehensive characterization of our institutional experience with clinical management of these tumors and posit an algorithm for diagnostic evaluation and treatment.MethodsThis was a retrospective cohort study of patients undergoing treatment for paragangliomas of the head and neck and cranial base at our institution from 2000–2017. Data on tumor location, catecholamine levels, and specific imaging modalities employed in diagnostic work‐up, pre‐treatment cranial nerve palsy, treatment modality, utilization of preoperative angiographic embolization, complications of treatment, tumor control and recurrence, and hereditary status (ie, succinate dehydrogenase mutations) were collected and summarized.ResultsThe mean (SD) age of our cohort was 51.8 (±16.1) years with 123 (63.4%) female patients and 71 (36.6%) male patients. Catecholamine‐secreting lesions were found in nine (4.6%) patients. Fifty‐one patients underwent genetic testing, with mutations identified in 43 (20 SDHD, 13 SDHB, 7 SDHD, 1 SDHA, SDHAF2, and NF1). Observation with serial imaging, surgical extirpation, radiation, and stereotactic radiosurgery were variably employed as treatment approaches across anatomic subsites.ConclusionAn algorithmic approach to clinical management of these tumors, derived from our longitudinal institutional experience and current empiric evidence, may assist otolaryngologists, radiation oncologists, and geneticists in the care of these complex neoplasms.Level of Evidence4

Second primary tumors in patients with a head and neck paraganglioma

“…Based on our reported incidence rate (2.59 second primary paragangliomas of the chest, abdomen or pelvis per 1000 person‐years), 38 patients would have to get a decade of annual MRIs of the chest, abdomen, and pelvis to identify a single second paraganglioma. Given the growing demand for medical financial stewardship, the cost of such screening appears to exceed the potential benefit, especially as many paragangliomas are ultimately observed without intervention . Alternatively, the use of assays of plasma or urinary catecholamines may be of greater value .…”

“…Given the growing demand for medical financial stewardship, the cost of such screening appears to exceed the potential benefit, especially as many paragangliomas are ultimately observed without intervention. 20 Alternatively, the use of assays of plasma or urinary catecholamines may be of greater value. 19,21 The utility of biochemical screening was not examined in this study but would benefit from further research.…”

Association of common genetic variants in theCPSF7andSDHAF2genes with Canine Idiopathic Pulmonary Fibrosis in the West Highland White Terrier

“…SDHAF2 encodes a mitochondrial protein involved in the flavination of a succinate dehydrogenase complex subunit and it has largely been associated with paragangliomas in previous literature [28,29].…”

Challenges in managing a vagal schwannomas: Lesson learnt

“…They further mentioned that the most common mode of treatment is surgical excision with preoperative embolization. The resultant complications include complete unilateral vagal nerve paralysis together with additional glossopharyngeal, hypoglossal nerve, and cervical sympathetic chain disturbances [ 5 ]. Heyes et al suggested that surgical excision is the standard treatment of choice for most vagal schwannomas but they stressed that nowadays, the contemporary management evolves toward a conservative modality due to high morbidity of the surgery.…”