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Patients with acromegaly usually present with characteristic clinical features or comorbidities associated with excess insulinlike growth factor 1 (IGF-1)/growth hormone (GH) or may come to medical attention secondary to mass effects causing visual field distortions. Herein, we report a case of spontaneous cerebrospinal fluid (CSF) rhinorrhea as the presenting symptom of acromegaly. A 68-year-old man presented to an outside facility with a 2-day history of headache associated with nausea, vomiting, dizziness, and clear nasal discharge and underwent 2 attempted repairs of a sphenoid sinus CSF leak. Examination on admission to our hospital was significant for fluctuating level of consciousness. Subsequently, subtle coarse facial features were appreciated. Pituitary function testing showed thyrotropin and gonadotropin deficiencies along with an elevated age- and sex-matched IGF-1 of 285 (normal level, 59-225 ng/mL). Nadir GH during oral glucose tolerance test was 5.5 ng/mL and confirmed the diagnosis of acromegaly. Magnetic resonance imaging showed pneumocephalus, an enlarged sella with an elongated pituitary stalk, and partial erosion of the anterior wall of the sphenoid sinus. A distinct adenoma could not be identified. An endoscopic, transnasal, transsphenoidal exploration and biopsy with multilayered skull base reconstruction were performed. Histologic examination of the biopsy contents was consistent with a GH-producing adenoma. Postoperatively, the patient's fluctuating level of consciousness improved and returned to baseline after his successful skull base repair. During the follow-up period, he had an IGF-1 of 713 ng/mL and started treatment with a somatostatin analogue. To our knowledge, this is the first reported case of a GH-producing pituitary adenoma presenting with spontaneous CSF rhinorrhea. Pituitary adenomas should be considered in the differential diagnosis of patients presenting with spontaneous CSF rhinorrhea with abnormal sellar image, and these patients should undergo a thorough hormonal evaluation. (C) 2011 Published by Elsevier Inc.

Previously, we reported surgical results of the simplified retrosigmoid approach for vestibular nerve sectioning in the cases with intractable vertigo. Here, we introduce in details surgical procedures of the simplified retrosigmoid (also called "subasterional" here for simplification) approach developed by one of the authors (J.H.L.). This approach is to operate cerebello-pontine (C-P) angle lesions through a small upper lateral window below the transverse-sigmoid junction. The retroauricular 4-5 cm skin incision, triangular craniectomy 2 cm in width, dural opening and closure, special considerations, and reconstruction of the skull defect were outlined. The bony landmarks for triangle craniectomy below asterion were described in detail. The C-P angle was appropriately approached for microvascular decompression, removal of C-P angle tumors, and vestibular nerve resection. This subasterional approach was characterized by short operation time of 1-3 h and less postoperative discomforts compared to the conventional retrosigmoid approach.

The loss of neurofibromatosis type 2 (NF2) tumor suppressor gene function and enhanced proliferative signal transduction through the Ras pathway are the hallmark of meningioma tumorigenesis. Although the exact mechanisms by which NF2 and Ras proteins contribute to meningioma tumorigenesis remain to be elucidated, the present knowledge is sufficient to start exploring exciting therapies that are directed at these molecular targets. Gene therapy to restore the functions of inactivated NF2 tumor suppressor protein and the use of several promising agents that target signal transduction molecules of the Ras pathway are reviewed. An increased understanding of the biologic functions of the new therapeutic targets discussed in this article will facilitate the development of meningioma treatments that are minimally toxic, yet highly effective.

Results: From the time of primary surgery, overall median survival was 3.4 years, 5 year survival was 47.2%, and median time to recurrence was 9.6 months. Patients who received adjuvant radiotherapy after their first surgery had median survival of 5.4 years (n = 3) whereas those not receiving radiotherapy survived to a median of 2.5 years (n = 10); this difference was not statistically significant. No other variable affected survival or time to recurrence including age, KPS at diagnosis, prior diagnosis of lower grade meningioma, degree of resection, or mode of salvage therapy. Notably, a single individual whose tumor has not recurred for 105 months had gross total resection with adjuvant radiotherapy to 5900 cGy. Conclusions: Our results are consistent with published data for malignant meningiomas that report 5 year survival from 32 to 64%. We found that half of 18 meningiomas diagnosed as malignant prior to 2000 failed to satisfy the new criteria for grade III tumors. This finding suggests that the new definition is substantially different than the prior one. Patient outcome remains unsatisfactory for malignant meningiomas. More studies are necessary to develop more effective interventions.