Controversy regarding their management still exists.
Surgery plays a crucial role.
Still, it remains challenging, particularly in cases of tumor growth inside the bony canal of the jugular foramen (JF).
The variety of surgical approaches makes selection of an approach difficult.
There are lateral, posterior, and anterior routes that access various parts of the jugular foramen. Removal of the jugular process of the occipital bone provides access to the posterior aspect of the foramen, the infralabyrinthine mastoidectomy provides access to the lateral edge and dome of the jugular bulb, and the preauricular approaches provide access to the anterior margin of the bulb and foramen. Additions to these approaches may include cervical and vertebral artery exposure, facial nerve transposition, foramen magnum exposure, and external canal and condylar resection 1).
The surgical approach selection to JFSs should be tailored individually to their extension pattern. The judicious application of endoscope-assisted retrosigmoid infralabyrinthine and transcervical techniques allow for safe and more radical removal of JFSs with a major intraosseous part 2).
A multiinstitutional retrospective analysis of 117 patients with JFSs who were treated with Gamma Knife surgery (GKS) at 18 medical centers of the Japan Leksell Gamma Knife Society. The median age of the patients was 53 years.
Fifty-six patients underwent GKS as their initial treatment, while 61 patients had previously undergone resection. At the time of GKS, 46 patients (39%) had hoarseness, 45 (38%) had hearing disturbances, and 43 (36%) had swallowing disturbances. Eighty-five tumors (73%) were solid, and 32 (27%) had cystic components. The median tumor volume was 4.9 cm3, and the median prescription dose administered to the tumor margin was 12 Gy. Five patients were treated with fractionated GKS and maximum and marginal doses of 42 and 21 Gy, respectively, using a 3-fraction schedule.
The median follow-up period was 52 months. The last follow-up images showed partial remission in 62 patients (53%), stable tumors in 42 patients (36%), and tumor progression in 13 patients (11%). The actuarial 3- and 5-year progression-free survival (PFS) rates were 91% and 89%, respectively. The multivariate analysis showed that pre-GKS brainstem edema and dumbbell-shaped tumors significantly affected PFS. During the follow-up period, 20 patients (17%) developed some degree of symptomatic deterioration. This condition was transient in 12 (10%) of these patients and persistent in 8 patients (7%). The cause of the persistent deterioration was tumor progression in 4 patients (3%) and adverse radiation effects in 4 patients (3%), including 2 patients with hearing deterioration, 1 patient with swallowing disturbance, and 1 patient with hearing deterioration and hypoglossal nerve palsy. However, the preexisting hoarseness and swallowing disturbances improved in 66% and 63% of the patients, respectively.
GKS resulted in good tumor control in patients with either primary or residual JFSs. Although some patients experienced some degree of symptomatic deterioration after treatment, persistent adverse radiation effects were seen in only 3% of the entire series at the last follow-up. Lower cranial nerve deficits were extremely rare adverse radiation effects, and preexisting hoarseness and swallowing disturbances improved in two-thirds of patients. These results indicated that GKS was a safe and reasonable alternative to surgical resection in selected patients with JFSs 3).
Sixteen consecutive patients with JFSs were retrospectively evaluated. Tumor extension was classified using a modification of a previous classification of Samii, and surgical approach was selected accordingly. The extent of tumor resection, cranial nerve outcome, approach-related morbidities, and recurrence of the tumors were documented.
Four cases of JFSs were subtotally resected elsewhere and 12 were primary. The most common type was B2 (6 cases). The average tumor size was 38.5 mm. Gross total resection was achieved in all patients, and none recurred during follow-up. There was no operative mortality or new permanent neurological deficits. A postoperative cerebrospinal fluid leak developed in 2 patients, 1 managed with lumbar drain and the other with wound revision. The preoperative swallowing difficulty temporarily deteriorated in 3 patients, but recovered well within the first 2 postoperative weeks in all 3. Improvement of the preoperative lower cranial nerve dysfunction was observed in 5 patients 4).
Ten patients with jugular foramen schwannomas treated by the senior author between January 2007 and December 2012. Three patients had undergone partial tumour resection elsewhere. The initial symptom for which they sought medical help was hearing loss, dysphagia, hoarseness, and shoulder weakness. Preoperative glossopharyngeal and vagal nerve deficits were the most common signs. In the series, tumour extension was classified according to Kaye-Pellet grading system. In two cases the tumours were classified into type A and 8 patients presented with type D tumours. A retromastoid suboccipital craniotomy was performed for type A tumours and modifications of cranio-cervical approach were suitable for type D.
No death occurred in this series. Four patients deteriorated after surgery: in two patients preoperative cranial nerve deficits deteriorated after surgery while new cranial nerve palsy occurred in 2 other patients. In four patients, the cranial nerve dysfunction had improved at the last follow-up examination. In all other patients, the cranial nerve dysfunction remained the same. One patient experienced tumour recurrence over a follow-up period of 40 months. This patient underwent a successful second surgery without further evidence of tumour growth 5).
23 patients. One patient (a 73-year-old woman) with normal lower cranial nerves function was managed with watchful expectancy and regular clinical and radiologic follow ups. The infratemporal fossa approach-type A (IFTA-A) was performed in 3 cases. One patient underwent a transcochlear-transjugular approach. Of the 22 patients surgically treated, 12 patients were operated on by the petrooccipital transsigmoid approach (POTS). In one patient with a preoperative dead ear, a combined POTS-translabyrinthine approach was adopted. Two patients were operated on through the POTS approach combined with the transotic approach. In another case (a 67-year-old woman), a subtotal tumor removal through a transcervical approach was planned to resect a 10-cm mass in the neck. One patient underwent a first-stage combined transcervical-subtotal petrosectomy approach to remove a huge tumor in the neck; the second-stage intradural removal of the tumor was accomplished through a translabyrinthine-transsigmoid-transjugular approach. The last patient underwent a first-stage combined transcervical-subtotal petrosectomy approach to remove the neck tumor component; this patient is now waiting for the second-stage intradural removal of the tumor. Complete tumor removal was accomplished in 21 cases and in one case, a residual schwannoma was left in place in the area of the jugular foramen. The 3 patients who were operated on by IFTA-A underwent permanent anterior transposition of the facial nerve. At 1-year follow up, 2 of these patients had House-Brackmann grade I and 1 reached grade IV. The patient who underwent a transcochlear-transjugular approach had a permanent posterior transposition of the facial nerve. At 1-year follow up, he had grade III facial nerve function. Postoperative facial nerve function was normal (House-Brackmann grade I) in all patients operated on by the POTS approach. Twelve patients had hearing-preserving surgery using the POTS approach. Good hearing was preserved in 10 cases (83.3%), the majority of whom (58.3%) maintained their preoperative hearing level. There was no perioperative mortality. One patient (4.5%) experienced a postoperative cerebrospinal fluid leak. After surgery, all patients did not recover the function of the preoperatively paralyzed lower cranial nerves. A new deficit of one or more of the lower cranial nerves was recorded in 50% of cases. So far, no patient has experienced recurrence during the follow-up period as ascertained by computed tomography or magnetic resonance imaging.
Surgical resection is the treatment of choice for jugular foramen schwannomas. The POTS approach allowed single-stage, total tumor removal with preservation of the facial nerve and of the middle and inner ear functions in the majority of cases. Despite the advances in skull base surgery, new postoperative lower cranial nerve deficits still represent a challenge 6).