The jugular foramen is deeply located at the cranial base, contains important neurovascular structures , and is in close relation to vital structures of the brain and neck. Tumors that develop in or around the jugular foramen (JF) are rare, mostly benign, deeply located, involve or embed vessels and cranial nerves, and very often highly vascularized. These characteristics pose significant difficulties in diagnosis, management, and rehabilitation. In the past, these lesions were considered inoperable and the lack of adequate diagnosis, surgical strategies, and postoperative care made prognosis of patients with benign jugular foramen tumors very poor. In spite of all diagnostic and surgical developments in the last decades, radical removal of large jugular foramen lesions with preserving the involved neurovascular structures remains a challenge. A wide variety of tumors can affect the JF, the most common are: paragangliomas, schwannomas, meningiomas, chordomas, chondrosarcomas, metastases, and primary bone tumors [1]. Bone tumors are uncommon and include a large number of neoplasias as giant cell tumors, aneurismatic bone cysts, plasmocytomas, osteoblastomas, and others. Some of these tumors like giant cell tumors and plasmocytomas of the jugular foramen may present with pulsatile tinnitus, hearing loss, and on otoscopy a hypervascular mass can be observed behind the tympanic membrane. The most frequent tumor arising in the JF region is paraganglioma . They have been called chemodectomas , nonchromaffin tumors , and glomus tumors . These tumors develop from neural crest tissue (paraganglia) and may arise sporadically. They can be familial with autosomal dominant inheritance and incomplete penetrance and as part of a genetic syndrome [2]. Familial cases of paragangliomas present higher incidence of multicentricity [3]. Paraganglia or glomus bodies are small masses developed from the neural crest. Guild [4], in 1941, was the first to describe carotid body-like structures in the temporal bone called them “glomus jugulare body .” In the jugular bulb they are found in the adventitia of the jugular bulb beneath the floor of the middle ear, in the bony walls of the tympanic canals associated with the auricular branch of the vagus nerve (Arnold nerve) or the tympanic branch of the glossopharyngeal nerve (Jacobson nerve). In the head and neck region they can be found close to the mucosal lining of the middle ear in the ciliary ganglion, the ganglion nodosum of the vagus nerve, and in the walls of large arteries [5]. Paragangliomas rarely release catecholamines [6]. Hypertension, headache, arrhythmias, nausea, and palpitations may occur when the tumor secretes catecholamine; these symptoms must be preoperatively recognized to avoid morbidity or even mortality. Perioperatively, these patients should be treated with alpha adrenergic blockade to normalize blood pressure and eliminate episodic hypertension. Alpha adrenergic antagonists like phenoxybenzamine, and more selective α1 antagonists like prazosin, are used one to two weeks prior to surgery. Beta blockade is rarely used. Beta blockade before establishment of α blockade may result in myocardial infarction, organ ischemia, and death from unopposed α agonism [6].

Paragangliomas are more frequent in the jugular foramen region (glomus jugulare), the middle ear (glomus tympanicum), and along the course of the vagus nerve (glomus vagale). Rosenwasser [7, 8], in April 1942, operated on a patient with a bleeding mass in the middle ear and the ear canal. Histologically this tumor was identical with the benign carotid body tumors. In 1945, he published the first description of a middle ear paraganglioma and associated these tumors with the glomus jugulare bodies [7]. Alford and Guilford [9] classified these lesions as glomus tympanicum and glomus jugulare tumors.

Schwannomas are the second more frequent neoplasia in the jugular foramen. They are usually benign, noninfiltrative lesions [1, 10, 11]. Primary meningiomas of the jugular foramen are rare and only small series have been reported in the literature. These tumors arise from arachnoid cells in the jugular bulb and may infiltrate surrounding bone and nerve tissues [12]. In our series of jugular foramen lesions 13 patients had meningiomas [13]. Four patients presented malignant or aggressive tumors (3 anaplastic and 1 papillary). One patient developed a radiation-induced malignant tumor after subtotal resection followed by radiotherapy for a benign meningioma in other institution. Chordomas and chondrosarcomas may also originate in the jugular foramen.

Metastatic lesions cause more commonly “classic jugular foramen syndromes ” (Vernet and Collet-Sicard syndrome ) than paragangliomas or schwannomas [14]. Metastatic adenocarcinomas, melanomas, and renal cell carcinomas may radiologically resemble glomus tumors.

Management of each patient with a jugular foramen tumor requires careful and individual consideration. These tumors, once considered to be one of the most difficult skull base tumors to remove, are now managed with reasonable morbidity and mortality rates. The goal of treatment is radical removal with preservation of cranial nerves and vessels. Surgical difficulties are related to the deep location of these lesions, hypervascularization, involvement of cranial nerves (VII, VIII, IX, X, and XI) and vessels like the internal carotid artery and the vertebral artery (VA) and its branches, and the extensive surgical defect due to bone removal from the cranial base and involved dura mater causing CSF leak. Surgical morbidity and mortality are usually associated with damage to the lower cranial nerves.