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ObjectiveTo describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS).MethodsWe report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature.ResultsA 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum.ConclusionThis report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations.IndexingAcromegaly, ESS, pituitary adenoma, sphenoid sinus septum.

ObjectiveTo describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS).MethodsWe report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature.ResultsA 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum.ConclusionThis report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations.IndexingAcromegaly, ESS, pituitary adenoma, sphenoid sinus septum.

It is described a case of combined empty sella syndrome and adrenal tumor with pseudo precocious puberty in 4 years old boy. Before the operative treatment of adrenal tumor pseudo precocious puberty was determined, then after surgery hypogonadotropic hypogonadism and short stature on the background of empty sella syndrome were diagnozed. The treatment with gene engineering growth hormone (the patient had open physis) and course of chorionic gonadotropin are required., Описан случай сочетания синдрома пустого турецкого седла и опухоли надпочечника с ложным преждевременным половым развитием у четырехлетнего мальчика. При этом у пациента до удаления опухоли надпочечника отмечалось ложное преждевременное половое созревание, а после удаления опухоли диагностирован гипогонадотропный гипогонадизм и низкорослость на фоне синдрома пустого турецкого седла. Необходимо назначение терапии генно-инженерным гормоном роста (у пациента зоны роста открыты), а также курсы терапии хорионическим гонадотропином., Описаний випадок поєднання синдрому пустого турецького сідла і пухлини надниркової залози з несправжнім передчасним статевим розвитком у чотирирічного хлопчика. При цьому у пацієнта до видалення пухлини надниркової залози відзначалося несправжнє передчасне статеве дозрівання, а після видалення пухлини діагностовано гіпогонадотропний гіпогонадизм і низькорослість на тлі синдрому пустого турецького сідла. Необхідне призначення терапії генно-інженерним гормоном росту (у пацієнта зони росту відкриті), а також курси терапії хоріонічним гонадотропіном.

SummarySpontaneous lateral sphenoid cephaloceles arise from bony defects in the lateral sphenoid, in the absence of predisposing factors such as trauma, surgery, mass, or congenital skull base malformation. We reviewed CT and MR imaging findings and clinical data of 26 patients with spontaneous lateral sphenoid cephaloceles to better understand anatomic contributions to pathogenesis, varying clinical and imaging manifestations, and descriptive terminology. Two types of spontaneous lateral sphenoid cephaloceles were identified. In 15 of 26 patients, a type 1 spontaneous lateral sphenoid cephalocele was noted, herniating into a pneumatized lateral recess of the sphenoid sinus, and typically presenting with CSF leak and/or headache. In 11 of 26 patients, a type 2 spontaneous lateral sphenoid cephalocele was noted, isolated to the greater sphenoid wing without extension into the sphenoid sinus, presenting with seizures, headaches, meningitis, cranial neuropathy, or detected incidentally. All patients had sphenoid arachnoid pits, and 61% of patients had an empty or partially empty sella, suggesting that altered CSF dynamics may play a role in their genesis.

We present the case of a woman with 'empty sella syndrome' who experienced generalized urticaria after the administration of sodium succinate hydrocortisone in two episodes.

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Prolactin dynamic was investigated in 43 premenopausal patients with primary empty sella (PES) diagnosed by pneumoencephalography and CT scan. Only normoprolactinemic patients were included in this study. Basal PRL levels ranged from 4 to 25 ng/ml. PRL responses to TRH (200 micrograms i.v.) and metoclopramide (MCP, 10 mg p.o.) were not significantly different from those in normal subjects, although a trend toward higher responses was present in PES patients. The administration of nomifensine (NOM, 200 mg p.o.) induced a PRL decrease, which was not significantly different from that in normal subjects. However, a sequential stimulation with TRH plus MCP (1 h after TRH administration) induced an exaggerated PRL increase which was significantly different from that in normal subjects. The peak PRL responses after stimulation were not significantly correlated with estradiol levels or FSH/LH ratios in our patients. The influence of body weight was also excluded on the basis of the responses observed in 8 obese control subjects that were significantly lower than in PES patients. Moreover, in 19 patients we studied the intracranial pressure (ICP) through an indwelling catheter inserted into the lumbar subarachnoid space. ICP was normal in 5 patients and elevated in 14 patients. When we compared PRL dynamics in patients with normal or elevated ICP, a significant difference was noted between the percentage of PRL decrease after NOM, that was lower and delayed in patients with increased ICP, suggesting an influence of ICP on neuronal dopamine reuptake. In conclusion, an augmented PRL reserve is present in premenopausal patients with PES. A correlation can be found between ICP and the function of dopaminergic neurons controlling lactotroph cells.

A young man is reported with an autoimmune polyglandular syndrome (APS) characterized by Addison's disease, primary hypothyroidism, primary hypogonadism, vitiligo, associated with primary empty sella and partial impairment of pituitary hormone secretion. Two years later the patient showed a null cell type acute lymphocytic leukaemia, immediately after surgery for an inguinal hernia. Pathogenetic mechanisms are postulated on the basis of HLA studies and lymphocytic typing.