Management of Cushing's disease: a single-center experience.

The purpose of this study was to review therapeutic outcomes and comorbidities of patients with Cushing's disease (CD) in a single center. We conducted a retrospective study of 33 patients with CD undergoing transsphenoidal surgery from January 2007 to February 2014 (27 females and 6 males, median age 38 years, range 18-71 years). The diagnosis of Cushing's syndrome was established on the basis of the patient's history, characteristic clinical features, and laboratory data including an elevated 24-h urinary free cortisol level, lack of serum cortisol suppression after dexamethasone suppression tests and an elevated midnight cortisol level. In 28/33 patients, the tumor was visualized on MR of the sellar region, while in 5 it was diagnosed using an inferior petrosal sinus sampling. Out of the 33 patients, 10 had macroadenoma and the remaining 23 had microadenoma. Twenty-one patients (63.6%) had hypertension, 17 (51.5%) dyslipidemia, and 7 (21.2%) had type 2 diabetes or impaired glucose tolerance. The median follow-up period was 28 months. Remission after transsphenoidal surgery was achieved in 78.8% of patients, while 7 patients failed to achieve disease remission. Those patients were treated with second-line treatment modalities (second operation, radiotherapy, bilateral adrenalectomy, and/or ketoconazole). One patient rejected all the treatment modalities after surgery. Cumulative remission after all the treatment modalities was achieved in 87.9% patients. Patients with Cushing's disease should be managed in centers with much experience due to high patient load. In our Center, the remission of the disease has been achieved in 78.8% of the patients following transsphenoidal surgery. Multimodal treatment which included radiotherapy and medical treatment led to biochemical remission of the disease in 87.9% of patients.

Predicting the "usefulness" of 5-ALA-derived tumor fluorescence for fluorescence-guided resections in pediatric brain tumors: a European survey.

BACKGROUND: Five-aminolevulinic acid (Gliolan, medac, Wedel, Germany, 5-ALA) is approved for fluorescence-guided resections of adult malignant gliomas. Case reports indicate that 5-ALA can be used for children, yet no prospective study has been conducted as of yet. As a basis for a study, we conducted a survey among certified European Gliolan users to collect data on their experiences with children. METHODS: Information on patient characteristics, MRI characteristics of tumors, histology, fluorescence qualities, and outcomes were requested. Surgeons were further asked to indicate whether fluorescence was "useful", i.e., leading to changes in surgical strategy or identification of residual tumor. Recursive partitioning analysis (RPA) was used for defining cohorts with high or low likelihoods for useful fluorescence. RESULTS: Data on 78 patients <18 years of age were submitted by 20 centers. Fluorescence was found useful in 12 of 14 glioblastomas (85 %), four of five anaplastic astrocytomas (60 %), and eight of ten ependymomas grades II and III (80 %). Fluorescence was found inconsistently useful in PNETs (three of seven; 43 %), gangliogliomas (two of five; 40 %), medulloblastomas (two of eight, 25 %) and pilocytic astrocytomas (two of 13; 15 %). RPA of pre-operative factors showed tumors with supratentorial location, strong contrast enhancement and first operation to have a likelihood of useful fluorescence of 64.3 %, as opposed to infratentorial tumors with first surgery (23.1 %). CONCLUSIONS: Our survey demonstrates 5-ALA as being used in pediatric brain tumors. 5-ALA may be especially useful for contrast-enhancing supratentorial tumors. These data indicate controlled studies to be necessary and also provide a basis for planning such a study.

[Endoscopic transsphenoidal pituitary surgery]. / Endoskopska transsfenoidalna hipofizna kirurgija.

Endoscopic surgery has been performed in our Department since 1996 as assistance in micro-neurosurgical procedures. In the same way the endoscope was used in transsphenoidal pituitary surgery, but from the beginning of 2004 we started with "pure" endoscopic surgery. We present our experience with the first ninety patients to demonstrate our way of operating. Endoscopic pituitary surgery is performed through a natural nasal air pathway without any incisions. A 4-mm endoscope is placed in front of the tumor in the sphenoidal sinus and the tumor is removed with specially designed surgical tools. Postoperative nasal packing is not necessary and postoperative discomfort is minimal so the hospital stay lasts 3 days. The first control was after one month. There were eighteen recurrences of tumors.

Large intracranial intradural mucocele as a complication of frontal sinus osteoma.

Paranasal sinus osteomas are mostly asymptomatic; however, secondary mucocele can develop if they impede the natural sinus drainage. Such a mucocele can destroy the bone and extend into the adjacent structures. We report on an unusual case of frontal sinus osteoma in a 27-year-old patient, complicated by large secondary mucocele that eroded the bone and extended into the frontal lobe of the brain. Unexpectedly, the patient did not report any visual or other symptoms attributable to central nervous system deficit. The mucocele was completely resected through bifrontobasal osteoplastic craniotomy, whereas osteoma was evacuated in its entirety by both drilling and mobilizing. Open surgical approach remains the main treatment for complicated paranasal sinus osteoma, and radical removal of intracranial mucocele is mandatory to prevent the development of life-threatening infections. Although intradural extension of a secondary mucocele is extremely unusual, head and neck surgery specialists should take this severe complication in consideration.