Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report

Introduction Hemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy. Case presentation An 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma (World Health Organization grade I). Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence. Conclusions A review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy

Pituitary surgery for small prolactinomas as an alternative to treatment with dopamine agonists

Despite the fact that consensus guidelines recommend long-term dopamine agonist (DA) therapy as a first-line approach to the treatment of small prolactinoma, some patients continue to prefer a primary surgical approach. Concerns over potential adverse effects of long-term medical therapy and/or the desire to become pregnant and avoid long-term medication are often mentioned as reasons to pursue surgical removal. In this retrospective study, 34 consecutive patients (30 female, 4 male) preferably underwent primary pituitary surgery without prior DA treatment for small prolactinomas (microprolactinoma 1-10mm, macroprolactinoma 11-20mm) at the Department of Neurosurgery, University of Bern, Switzerland. At the time of diagnosis, 31 of 34 patients (91%) presented with symptoms. Patients with microprolactinomas had significantly lower preoperative prolactin (PRL) levels compared to patients with macroprolactinomas (median 143μg/l vs. 340μg/l). Ninety percent of symptomatic patients experienced significant improvement of their signs and symptoms upon surgery. The postoperative PRL levels (median 3.45μg/l) returned to normal in 94% of patients with small prolactinomas. There was no mortality and no major morbidities. One patient suffered from hypogonadotropic hypogonadism after surgery despite postoperative normal PRL levels. Long-term remission was achieved in 22 of 24 patients (91%) with microprolactinomas, and in 8 of 10 patients (80%) with macroprolactinomas after a median follow-up period of 33.5months. Patients with small prolactinomas can safely consider pituitary surgery in a specialized centre with good chance of long-term remission as an alternative to long-term DA therap

Leiomyomatoid angiomatous neuroendocrine tumor (LANT) of the pituitary: a distinctive biphasic neoplasm with primitive secretory phenotype and smooth muscle-rich stroma

We describe a hitherto undocumented variant of dimorphic pituitary neoplasm composed of an admixture of neurosecretory cells and profuse leiomyomatous stroma around intratumoral vessels. Radiologically perceived as a macroadenoma of 3.8cm in diameter, this pituitary mass developed in an otherwise healthy 43-year-old female. At the term of a yearlong history of amenorrhea and progressive bitemporal visual loss, subtotal resection was performed via transsphenoidal microsurgery. Discounting mild hyperprolactinemia, there was no evidence of excess hormone production. Histologically, solid sheets, nests and cords of epithelial-looking, yet cytokeratin-negative cells were seen growing in a richly vascularized stroma of spindle cells. While strong immunoreactivity for NCAM, Synaptophysin and Chromogranin-A was detected in the former, the latter showed both morphological and immunophenotypic hallmarks of smooth muscle, being positive for vimentin, muscle actin and smooth muscle actin. Architectural patterns varied from monomorphous stroma-dominant zones through biphasic neuroendocrine-leiomyomatous areas, to pseudopapillary fronds along vascular cores. Only endothelia were labeled with CD34. Staining for S100 protein and GFAP, characteristics of sustentacular cells, as well as bcl-2 and c-kit was absent. Except for α-subunit, anterior pituitary hormones tested negative in tumor cells, as did a panel of peripheral endocrine markers, including serotonin, somatostatin, calcitonin, parathormone and vasoactive intestinal polypeptide. Mitotic activity was absent and the MIB-1 labeling index low (1-2%). While assignment of this lesion to any established neoplastic entity is not forthcoming, we propose it is being considered as a low-grade neuroendocrine tumor possibly related to null cell adenom

Endovascular Treatment of Anterior Circulation Cerebral Aneurysms by Using Guglielmi Detachable Coils: A 10-Year Single-Center Experience with Special Emphasis on the Use of Three-Dimensional GDC

Purpose:: To analyze the immediate, long-term angiographic and clinical results of endovascular treatment of anterior circulation aneurysms with special regard to the use of three-dimensional Guglielmi detachable coils (3D-GDC). Patients and Methods:: Between 1993 and 2003, 116 patients with 116 anterior circulation aneurysms were treated. 88 patients (75.9%) underwent embolization due to high surgical risk. To analyze the use of 3D-GDC, patients treated before (group 1) and after (group 2) implementation of 3D-GDC in 1999 were compared. Mean duration of angiographic follow-up was 13.9 months. Clinical follow-up was set at hospital discharge and using a questionnaire for long-term follow-up (mean 46.8 months). Results:: Overall, at initial intervention, complete occlusion was achieved in 65 aneurysms (56.0%), neck remnant in 42 (36.2%), and incomplete occlusion in nine (7.8%). Procedure-related permanent morbidity was 4.3% and mortality 2.6%. Recanalization rate at radiologic follow-up was 16.7%. Occlusion success at initial treatment correlated with aneurysm neck size (p = 0.001). Clinical outcome at hospital discharge was dependent on Hunt & Hess grade at presentation (p = 0.01). Subgroup analysis revealed that the use of 3D-GDC produced a higher initial obliteration rate compared to standard coils, but did not reach statistical significance (p = 0.059). Neither aneurysm neck size nor aneurysm dome size nor the use of 3D-GDC significantly influenced recanalization rate. Conclusion:: GDC technology is effective and safe, particularly in case of patients with high surgical risk. Aneurysm neck size was predictive of occlusion rate and Hunt & Hess grade of clinical outcome. Introduction of 3D-GDC probably improved occlusion rate, but did not significantly influence recanalization rat

Collateral-flow measurements in humans by myocardial contrast echocardiography: validation of coronary pressure-derived collateral-flow assessment

Aims Myocardial blood flow (MBF) is the gold standard to assess myocardial blood supply and, as recently shown, can be obtained by myocardial contrast echocardiography (MCE). The aims of this human study are (i) to test whether measurements of collateral-derived MBF by MCE are feasible during elective angioplasty and (ii) to validate the concept of pressure-derived collateral-flow assessment. Methods and results Thirty patients with stable coronary artery disease underwent MCE of the collateral-receiving territory during and after angioplasty of 37 stenoses. MCE perfusion analysis was successful in 32 cases. MBF during and after angioplasty varied between 0.060-0.876 mL min−1 g−1 (0.304±0.196 mL min−1 g−1) and 0.676-1.773 mL min−1 g−1 (1.207±0.327 mL min−1 g−1), respectively. Collateral-perfusion index (CPI) is defined as the rate of MBF during and after angioplasty varied between 0.05 and 0.67 (0.26±0.15). During angioplasty, simultaneous measurements of mean aortic pressure, coronary wedge pressure, and central venous pressure determined the pressure-derived collateral-flow index (CFIp), which varied between 0.04 and 0.61 (0.23±0.14). Linear-regression analysis demonstrated an excellent agreement between CFIp and CPI (y=0.88x+0.01; r2=0.92; P<0.0001). Conclusion Collateral-derived MBF measurements by MCE during angioplasty are feasible and proved that the pressure-derived CFI exactly reflects collateral relative to normal myocardial perfusion in human

Risk of decompression illness among 230 divers in relation to the presence and size of patent foramen ovale

Background The risk of developing decompression illness (DCI) in divers with a patent foramen ovale (PFO) has not been directly determined so far; neither has it been assessed in relation to the PFO's size. Methods In 230 scuba divers (age 39±8 years), contrast trans-oesophageal echocardiography (TEE) was performed for the detection and size grading (0-3) of PFO. Prior to TEE, the study individuals answered a detailed questionnaire about their health status and about their diving habits and accidents. For inclusion into the study, ⩾200 dives and strict adherence to decompression tables were required. Results Sixty-three divers (27%) had a PFO. Overall, the absolute risk of suffering a DCI event was 2.5 per 104 dives. There were 18 divers (29%) with, and 10 divers (6%) without, PFO who had experienced ⩾1 major DCI events \batchmode \documentclass[fleqn,10pt,legalpaper]{article} \usepackage{amssymb} \usepackage{amsfonts} \usepackage{amsmath} \pagestyle{empty} \begin{document} $(P=0.016)$ \end{document}. In the group with PFO, the incidence per 104 dives of a major DCI, a DCI lasting longer than 24 h and of being treated in a decompression chamber amounted to 5.1 (median 0, interquartile range [IQR] 0-10.0), 1.9 (median 0, IQR 0-4.0) and 3.6 (median 0, IQR 0-9.8), respectively and was 4.8-12.9-fold higher than in the group without PFO \batchmode \documentclass[fleqn,10pt,legalpaper]{article} \usepackage{amssymb} \usepackage{amsfonts} \usepackage{amsmath} \pagestyle{empty} \begin{document} $(P{<}0.001)$ \end{document}. The risk of suffering a major DCI, of a DCI lasting longer than 24 h and of being treated by recompression increased with rising PFO size. Conclusion The presence of a PFO is related to a low absolute risk of suffering five major DCI events per 104 dives, the odds of which is five times as high as in divers without PFO. The risk of suffering a major DCI parallels PFO siz