Disease and Treatment-Related Sequelae in Patients with Complex Jugulotympanic Paraganglioma

Ali Harati,Rolf Schultheiß,Stefan Rohde,Thomas Deitmer

doi:10.3390/jcm7030051

Abstract

Background: Jugulotympanic paraganglioma (JTP) are benign, high-vascularized lesions that frequently invade the jugular foramen, temporal bone, the upper neck, and the posterior fossa cavity, resulting in a wide variety of clinical symptoms. Methods: In this retrospective study, we assess the clinical symptoms and discuss the individual multidisciplinary treatment and outcome of 22 patients with JTP. Results: In 12 patients, a hearing deficit was the presenting symptom, whereas pulsatile tinnitus and otalgia were present in six and four patients respectively. Facial nerve involvement was seen in six patients (three HB Grade 1–2 and three HB Grade 4–6). Four patients presented with lower cranial nerve impairment. Rare symptoms were ataxia caused by brainstem compression and papilledema due to cerebral sinus obstruction. A new or worsening of the preoperative facial nerve or lower cranial nerve function occurred in two and four patients respectively. Conclusion: The treatment strategy and the surgical approach for JTP should be tailored to the tumor extension and the patient’s clinical symptoms.

Highlights

Paragangliomas (PGLs), known as chemodectomas, have their origin in paraganglia of the chemoreceptor system
Mild facial nerve involvement was seen in three patients (HB Grade 1–2) and progressive facial nerve palsy was present in three patients (HB Grade 4–6)
Four patients presented with affection of cranial nerves IX and X

Summary

Introduction

Paragangliomas (PGLs), known as chemodectomas, have their origin in paraganglia of the chemoreceptor system. Jugulotympanic paraganglioma (JTP), known as glomus jugulare tumors, arise from a small group of cells in the adventitia of the jugular bulb Despite their suspected benign origin, they represent locally aggressive, destructive neoplastic lesions, with frequent invasion of the middle ear, the temporal bone, the upper neck, and through the jugular foramen, into the posterior cranial fossa. Based on their location, size, and extent, JTP can be classified by Fisch into four categories (Table 1). Jugulotympanic paraganglioma (JTP) are benign, high-vascularized lesions that frequently invade the jugular foramen, temporal bone, the upper neck, and the posterior fossa cavity, resulting in a wide variety of clinical symptoms. Conclusion: The treatment strategy and the surgical approach for JTP should be tailored to the tumor extension and the patient’s clinical symptoms

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