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Objective: Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is one of the rare causes of endogenous hyperinsulinism. Its diagnosis is challenging and may require selective intraarterial calcium stimulation and concomitant hepatic vein sampling (SACVS). Impaired counterregulatory hormones' production in response to hypoglycemia has been previously described in patients with diabetes, insulinoma and infancy hypoglycemia. We present a case of endogenous hyperinsulinism, secondary to NIPHS, with deficient cortisol response to hypoglycemia which resolved after diazoxide treatment., Design-Results: A 43-year-old woman was admitted with recurrent episodes of registered fasting and postprandial hypoglycemia. Abdominal computed tomography, magnetic resonance imaging and endoscopic ultrasonography of the pancreas failed to reveal any lesion while SACVS sampling demonstrated a 5- to 8-fold increase in insulin levels in diverse parts of the pancreas. Counterregulatory hormones' measurement revealed an attenuated cortisol response. Treatment with diazoxide resulted in disappearance of hypoglycemic episodes. Twelve months later, an insulin tolerance test was performed which revealed a normal cortisol response., Conclusions: This report describes the first, to our knowledge, reported case in the literature of NIPHS with deficient cortisol response to hypoglycemia which resolved after diazoxide treatment. It is important for clinicians to include NIPHS in the differential diagnosis of hypoglycemia and identify possible impairment of counterregulatory hormones' production.

Objective: Dopamine agonists (DA) are the treatment of choice in patients with macroprolactinomas. Brain and optic chiasm herniation are unusual complications following treatment with DA., Report: We present a case of a giant prolactinoma complicated by visual deterioration following cabergoline treatment. A 42-year-old man was admitted with seizures, right visual loss and visual defect in the upper left temporal quadrant. Magnetic resonance imaging (MRI) identified a giant adenoma, which proved to be a prolactinoma, compressing the optic chiasm and extending into the suprasellar region. Treatment with cabergoline was initiated resulting in improvement in visual fields, tumor shrinkage and prolactin level decrease. Five months later and despite tumor reduction, a deterioration of his visual fields was observed. The second MRI revealed brain and optic chiasmal herniation into the pituitary sella. Cabergoline dose was reduced and surgical resection of the adenoma along with untethering of the optic nerve was performed leading to improvement of the visual defects., Conclusions: This report describes a rare case of brain and optic chiasmal herniation attributed to DA therapy for a macroprolactinoma. It is important for clinicians to examine visual fields and promptly identify any visual deterioration in patients with macroprolactinomas receiving DA treatment.

Objective: Primary central nervous system (CNS) non-Hodgkin's lymphoma is a rarely encountered clinical entity. Here we present a case of a primary CNS diffuse large B-cell non-Hodgkin's lymphoma developed on a previously operated and irradiated pituitary macroadenoma. DESIGN-RESULTS: A 60-year-old woman presented with muscle weakness and eye lid ptosis. Thirty years ago, she was diagnosed with a non-functioning pituitary macroadenoma requiring repeated incomplete operations and conventional radiotherapy and accompanied by partial anterior pituitary deficiency. On admission, the magnetic resonance imaging (MRI) identified a pituitary sellar mass extending into the suprasellar region, compressing the optic chiasm and invading the left cavernous sinus. Following transsphenoidal surgery, the histological investigation revealed the presence of a diffuse large B-cell non-Hodgkin's lymphoma without other loci from the systemic staging. Following chemotherapy and despite a marked resolution of the neoplastic pituitary mass in the post-chemotherapy MRI scan, the patient's course was complicated with consciousness deterioration attributed to epileptic seizures and she died of a hospital acquired infection., Conclusions: Clinicians should include primary CNS lymphoma in the differential diagnosis of an isolated invasive sellar mass. The possible association of primary CNS lymphoma development with the history of operated and irradiated pituitary adenoma is herein discussed.

Thyrotoxic Periodic Paralysis (TPP) is a rare manifestation of hyperthyroidism characterized by muscle weakness and hypokalemia. Thyroid-Stimulating Hormone (TSH)-secreting pituitary adenoma is a rare cause of hyperthyroidism. Even more rare is the occurrence of TPP as the first manifestation of a TSH-secreting pituitary adenoma. We report a 31-year-old Asian male patient suffering from TPP caused by a TSH-secreting adenoma, who was evaluated for persistent episodes of muscle paralysis. Laboratory investigation revealed hypokalemia as well as elevated levels of both thyroid hormones and TSH. The Magnetic Resonance Imaging (MRI) of the pituitary gland revealed a microadenoma, thus suggesting the presence of a TSH-secreting adenoma. The patient underwent transphenoidal resection and the pathological investigation confirmed the diagnosis of TSH-secreting pituitary adenoma. After the adenomectomy and the restoration of euthyroidism, the patient did not experience any episode of hypokalemic paralysis or weakness. Despite its rarity, TSH-secreting pituitary adenoma should be included in the differential diagnosis of TPP.