Diagnostic, Surgical, and Postoperative Challenges of Neuroendocrine Tumors of the Neck: Clinical Experience and Literature Review

Abstract

Background: Neuroendocrine tumors of the neck are rare swellings, which constitute about 0.03% of all tumors. Most are sporadically arising paragangliomas. Over 50% arise from the carotid bodies and pose difficulties in diagnosis and surgical management, which may result in disabling complications.

Objective: To determine the diagnostic, surgical, and postoperative challenges, and how to overcome them, in the management of neuroendocrine tumors of the neck.

Study design: Descriptive case series.

Methods: A retrospective analysis of 19 patients who presented to our surgery clinics between 2010 and 2015 with neck swelling and suspicion of neurogenic tumors were included in the study. All patients received preoperative evaluation with Doppler studies and neck computed tomography scan, with the finding of suspicion of neurogenic tumor. Preoperative tissue diagnosis was not attempted as this usually required excisional biopsy. All tumors were surgically excised. Data regarding mean tumor size, neurovascular invasion, postoperative complications, and histopathological findings were recorded and statistically analyzed.

Results: Nineteen patients (7 male) with median age of 45 years were included. Mean size of the tumor was 7.5 cm (range: 4.0-11.8 cm). Six of the tumors were right sided and 13 left sided. Clinical findings often were nonspecific and mimicked other conditions. Operative challenges were encountered in 11 (57.9%) patients, who had tumors of more than 5 cm. Vascular invasion was found in 2 (10.5%) patients, but bleeding requiring preoperative transfusion was not required in any patient. Neurological complications occurred in 11 (57.8%) patients; 3 had transit hypoglossal nerve palsy, with full recovery; 2 had complete nerve palsy, with reasonable rehabilitation without further surgical intervention; and 5 developed neurological deficits (hoarseness, dysphagia, or Horner's syndrome) but recovered and compensated well without further operation. Two patients who had vascular invasion required vascular reconstruction. Histopathology in 14 (73.7%) cases was carotid body tumor and 5 were Schwannomas (1 vagal and 4 sympathetic chain). All 4 patients with Schwannomas of cervical sympathetic chain developed Horner's syndrome; 2 of them have compensated well with reasonable recovery; 2 have had tarsorrhaphy for cosmetic reasons. The patient with vagal Schwannoma developed hoarseness and swallowing difficulties, which resolved without intervention. No malignancy was encountered in this series, and there was no mortality.

Conclusions: Neuroendocrine tumors of the neck present diagnostic challenges because of nonspecific clinical findings mimicking other conditions. Preoperative tissue diagnosis is mostly unsuccessful, and exact diagnosis requires excisional biopsy. Surgical excision, which is the main treatment, often has associated technical difficulties and unavoidable complications requiring further intervention.