Removal of Giant Extra-Intracranial Hormone-Releasing Paraganglioma: The Nearest Postoperative Result

D.A. Gulayev, S.Ya. Chebotarev, N.V. Kolakutsky, V.E. Olyushin, A.Yu. Shcherbuk, O.Yu. Petropavlovskaya, M.Yu. Petropavlovskaya, K.Yu. Orlov, I.A. Savina, A.N. Kondratyev, L.M. Tsintsiper, S.S. Lesina

(Polenov Research Neurosurgical Institute, Saint Petersburg)

Female patient L., born in 1962. According to anamnesis data, in 1993 there appeared noise in the left ear, accompanied by gradual reduction of hearing. Then the patient began to complain of awkward movements of the left hand and pain in the left part of a head. Removal of tumor, located in the area of the left jugular glome, was performed in the Municipal Hospital N 2 on February 1, 1996. Radical operation on the left ear with an exposure and revision of the jugular vein bulb was made on June 7, 1996. In 2002 she noted, that it was impossible to close her left eye completely; besides, her face became distorted. Then there appeared swelling in the left-side parotid area. Control MRI-examination demonstrated a prolonged growth of tumor. A neurological picture was represented by a lesion of the left seventh, eighth, ninth, twelfth nerves. Local examination revealed a pathologic formation (4´4 cm) in the parotid-masticatory area. It was dense, elastic, painless, not fused with the skin and not mobile.

Endocrinologic examination was indicative of norepinephrine and somatotropin-producing tumor. The content of norepinephrine, epinephrine, dopamine and vanilylmandelic acid in daily urine (21.02.05) was as follows: 170 mg/day, 10 mg/day, 2217 mg/day (65-400) and 1.3 (1.4-6.5) respectively.

CT of the brain (18.09.03) demonstrated a mass in projection of the left jugular foramen. It spread intracranially along a posterior surface of the left pyramid into a widened internal acoustic meatus and caudally via the jugular foramen along a left-side squamous portion of the occipital bone up to the left occipital condyle. Its dimensions were 49´52´63 mm. Computed angiography showed, that a distal part of the extracranial segment of the left ICA was displaced by a mass in a forward and medial direction. The left jugular vein was not visualized. Superselective embolization of feeders (a. auricularis post., a. meningea accessoria) and a vascular network of tumor with polyvinylalcohol and histacryl was performed on February 16, 2005 (a surgeon: K.Yu. Orlov). Removal of tumor of the left jugular glome with resection of the pyramid of left temporal bone, affected dura, sigmoid and transverse sinuses was made on March 4, 2005. A defect of soft tissues of a lateral surface of the neck and skull base was eliminated with applying a thoracodorsal graft with a neurovascular pedicle (surgeons: D.A. Gulyaev, S.Ya. Chebotarev, N.V. Kolakutsky).

Histologic study (N 824-30/05) was indicative of chemodectoma.

Control endocrinologic examination was carried out on April 2, 2005. There were normal levels of catecholamines and a bit high level of hydrocortisone. Blood pressure became normal (120-140/80 mm Hg). The content of norepinephrine, epinephrine, dopamine and free hydrocortisone in daily urine (14.03.05) was as follows: 78 mg/day (15-80), 0 mg/day (0-20), 252 mg/day and 19.3 (10-15) respectively.

Control CT demonstrated a state after block-resection of the temporal bone pyramid. There were no data on further growth of tumor.

The demonstration goal was to illustrate possibility of total removal of giant hormone-producing tumors of the jugular glome and expediency of an interdisciplinary approach to their surgical treatment.