Perspectives Commentary on: Nonglomic Tumors of the Jugular Foramen: Differential Diagnosis and Prognostic Implications by Guinto et al. World Neurosurg 82:1283-1290, 2014

Judicious Management of Jugular Foramen Tumors Francesco Tomasello and Alfredo Conti

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ne of the challenges of contemporary neurosurgery is to continue dealing with extremely complex situations with increasing ease, making difficult choices comprehensible to patients, avoiding all functional, cosmetic, and psychological complications. To meet this challenge is very complicated. It means, on many occasions, to restrain our own vanity, combining a very difficult neurosurgical operation with alternative solutions, including endovascular treatments and radiotherapy, always aiming at a multidisciplinary approach. The ability to choose such a “judicious” combination of procedures is particularly important in specific neurosurgical pathologies, including lesions of the jugular foramen.

Tumors of the jugular foramen are among the most challenging neurosurgical lesions because of the complex neurovascular anatomy of this region, including the jugular bulb, the 10th and 11th cranial nerves in the posterolateral pars venosa and the inferior petrosal sinus, and the 9th cranial nerve in the anteromedial pars nervosa (2). Also, tumors may frequently encase the internal carotid artery. Inadvertent injuries to neurovascular structures of this region can result in fatal consequences. With rare exceptions, lesions in this region are benign, slow-growing tumors. Paragangliomas, chemodectomas, and glomus jugulare tumors are the most common jugular foramen tumors. Schwannomas of lower cranial nerves, meningiomas, chondrosarcomas, and high-grade malignant tumors including chordomas and metastases are other lesions that may involve the jugular foramen. Radical resection of a benign jugular foramen tumor is the treatment of choice (1, 3, 5, 8, 9). Nevertheless, it should be borne in mind that they represent a formidable surgical challenge.

Key words Approach - Infratemporal fossa - Jugular foramen - Nonglomic tumors - Skull base surgery -

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Tumors in this region are generally highly vascularized and, even if histologically benign, may invade bone, blood vessels, dura mater, cranial nerves, and the brainstem. In particular, a group of “complex glomus jugulare tumors” was identified by Al-Mefty and Teixeira (1). These tumors are giant or multiple (bilateral or ipsilateral), malignant, catecholamine secreting, or associated with other lesions such as dural arteriovenous malformations or an adrenal tumor. Other extremely complex tumors are tumors in which previous treatment resulted in the sacrifice of the carotid artery, radiation therapy, postoperative deficit, or adverse effect from embolization (1). A multidisciplinary approach is always required to achieve optimal results. The preoperative evaluation is of paramount importance; the nature and extension of the tumors must be interpreted correctly because different tumors pose different surgical problems and require different management strategies. Complete otologic and neurologic examinations are necessary to evaluate speech and swallowing function preoperatively and postoperatively. Preoperative embolization is necessary in cases of highly vascularized tumors to reduce bleeding during surgery. Balloon test occlusion is required if the internal carotid artery is involved or encased. A high-flow shunt (saphenous vein or radial artery graft) may be required in some instances. Intraoperative electrophysiologic monitoring of lower cranial nerves and facial and vestibulocochlear complex and frameless stereotaxy are indispensable adjuncts to surgery. Improved microsurgical techniques have led to a significant reduction of complication rates with reported stroke rate of 0%e 3.5%, cranial nerve injury rate of 5%e39%, and overall mortality

Department of Neurosurgery, University of Messina, Messina, Italy To whom correspondence should be addressed: Francesco Tomasello, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2015) 83, 5:756-757. http://dx.doi.org/10.1016/j.wneu.2014.09.013

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PERSPECTIVES

of 0%e2.7% (4). Nevertheless, 15% of patients may be unable to return to their preoperative daily activities. Despite the use of combined approaches, treatments result in subtotal resection in a substantial number of cases (14% as reported by Al-Mefty and Teixeira (1) and 17% according to Patel et al. (6)). Radiotherapy is an effective adjuvant treatment for glomus jugulare tumors. External-beam radiotherapy provided long-term tumor control in 80%e100% of cases, with complication rates of 0%e10% (7) and 71% of patients reporting improvement of their clinical symptoms (4). Glomus jugulare tumors, schwannomas, and meningiomas of the jugular foramen can be suitable targets for radiosurgery. Pollock (7) reported data of 42 patients treated by Gamma Knife (Elekta AB, Stockholm, Sweden) radiosurgery. Tumor size decreased in 31%, was stable in 67%, and increased in 2% of patients at 44 months. Hearing decline occurred in 19% of cases, but no other complications were recorded. Lim et al. (4) reported data of 13 patients with glomus jugulare tumors treated by radiosurgery with a linear accelerator or CyberKnife (Accuray Inc., Sunnyvale, California, USA). Tumor control was achieved in all patients, and no permanent neurologic deficit was recorded during follow-up (4e139 months). In a paper recently published in WORLD NEUROSURGERY, Guinto et al. report clinical data and surgical results of a series of 30 patients harboring non-glomic tumors of the jugular foramen. The authors combined a series of different histologic tumor subtypes with different clinical behaviors and different prognosis. Malignant tumors included chordomas and metastatic carcinomas whose prognosis and clinical results were eventually dismal. There were 24 benign tumors in this series (18 schwannomas and 6 meningiomas), which is a significant number of cases compared with the other series in the literature. Subtotal resection was performed in 19 patients (72%), 6 with meningiomas and 13 with schwannomas. Patients who received adjuvant radiosurgery were progression-free at the

REFERENCES 1. Al-Mefty O, Teixeira A: Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg 97:1356-1366, 2002. 2. Inserra MM, Pfister M, Jackler RK: Anatomy involved in the jugular foramen approach for jugulotympanic paraganglioma resection. Neurosurg Focus 17:E6, 2004. 3. Kadri PA, Al-Mefty O: Surgical treatment of dumbbell-shaped jugular foramen schwannomas. Neurosurg Focus 17:E9, 2004.

last follow-up evaluation. The main morbidity in the series was facial paresis occurring in patients operated through an infratemporal approach with anterior transposition of the facial nerve. These results suggest that judicious management of these tumors can result in satisfactory patient outcome in most instances. The retrosigmoid approach can be sufficient for resection of the intracranial portion of these tumors. Optimal functional results can be achieved avoiding extensive manipulation of the lower cranial nerves, even at the cost of a subtotal resection. As stated earlier, radiosurgery was demonstrated to be safe and effective in controlling the long-term growth of small residual tumors inside the foramen jugulare. The retrosigmoid approach is insufficient when the foraminal and extraforaminal components are very extensive. As clearly stated by the authors, it is impossible to gain access to this foramen via the retrosigmoid approach by bone drilling, as it is performed in cases of vestibular schwannomas, because of the presence of the sigmoid sinus. An extended approach is necessary to manage a large intraforaminal and extraforaminal component of jugular foramen tumors. Dumbbell-shaped schwannomas can be resected through a suprajugular approach—a presigmoid infralabyrinthine route (3). Large glomus jugulare tumors require an infratemporal approach with sacrifice of the sigmoid sinus and possibly transposition of the facial nerve. In conclusion, judicious management of jugular foramen tumors may result in optimal control of tumor growth in most cases. The preoperative diagnostic work-up should be very extensive and guide the overall management strategy. Non-glomic tumors are generally more easily controlled, and most cases can be managed by a retrosigmoid approach. Given the satisfactory results obtained by radiosurgery and its low risk of neurovascular injuries, its use should be considered when a radical resection cannot be achieved with reasonable risks.

5. Mattos JP, Ramina R, Borges W, Ghizoni E, Fernandes YB, Paschoal JR, Honorato DC, Borges G: Intradural jugular foramen tumors. Arq Neuropsiquiatr 62:997-1003, 2004. 6. Patel SJ, Sekhar LN, Cass SP, Hirsch BE: Combined approaches for resection of extensive glomus jugulare tumors. A review of 12 cases. J Neurosurg 80:1026-1038, 1994. 7. Pollock BE: Stereotactic radiosurgery in patients with glomus jugulare tumors. Neurosurg Focus 17: E10, 2004.

Neurosurgery 57(1 Suppl):59-68; discussion 59-68, 2005. 9. Ramina R, Neto MC, Fernandes YB, Aguiar PH, de Meneses MS, Torres LF: Meningiomas of the jugular foramen. Neurosurg Rev 29:55-60, 2006.

Citation: World Neurosurg. (2015) 83, 5:756-757. http://dx.doi.org/10.1016/j.wneu.2014.09.013 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com

4. Lim M, Gibbs IC, Adler JR Jr, Chang SD: Efficacy and safety of stereotactic radiosurgery for glomus jugulare tumors. Neurosurg Focus 17:E11, 2004.

8. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, Pfeilsticker LN, Coelho Neto M: Tumors of the jugular foramen: diagnosis and management.

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Judicious management of jugular foramen tumors.

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