Introduction: Functioning gonadotroph adenomas, also known as follicle-stimulating hormone (FSH)-secreting pituitary adenomas (FSH-omas), are rare pituitary tumors characterized by the secretion of biologically active FSH. Unlike the more common clinically nonfunctioning gonadotroph adenomas, these tumors produce endocrine manifestations due to excessive FSH secretion. Clinical presentation may result from local mass effects such as headache, visual disturbances due to optic chiasmal compression, or from hormonal activity affecting reproductive function. The endocrine manifestations vary between males and females and can lead to diagnostic challenges. Cases: We report two patients with functioning gonadotroph adenomas presenting with distinct clinical manifestations. The first case involved a 23-year-old female who presented with secondary amenorrhea for 4 years, accompanied by personality changes. Abdominal ultrasound demonstrated bilaterally enlarged multicystic ovaries suggestive of ovarian hyperstimulation, which subsequently regressed postoperatively. Biochemical evaluation revealed inappropriately elevated FSH levels (3.5-fold increase), with suppressed luteinizing hormone (LH) and markedly elevated estradiol levels (11-fold increase). Pituitary magnetic resonance imaging (MRI) identified a large sellar–suprasellar mass with mass effect, consistent with a pituitary macroadenoma. The second case involved a 68-year-old male who presented with progressively worsening vision over 2 years. Hormonal evaluation demonstrated elevated FSH levels (4.5-fold increase), with relatively normal LH and low testosterone levels. Pituitary MRI revealed a sellar mass consistent with a pituitary adenoma. Both patients subsequently underwent pterional craniotomy with tumor debulking. Histopathological examination confirmed gonadotroph adenomas, with positive immunohistochemical staining for FSH. Conclusion Functioning gonadotroph adenomas are rare and may present with diverse clinical features related to gonadal hyperstimulation or mass effects. Early recognition of the characteristic hormonal profile and radiological findings is crucial for diagnosis and appropriate management. These cases highlight the importance of considering functioning gonadotroph adenoma in patients presenting with unexplained gonadal hyperstimulation or atypical reproductive hormonal profiles.
Gonadotrophs ; Adenoma

Introduction: Normotensive pheochromocytomas in Von Hippel-Lindau (VHL) syndrome present unique perioperative challenges. Standard alpha-blockade may induce intolerable orthostatic hypotension, making calcium channel blockers (CCB) a practical alternative. Furthermore, the primary intraoperative danger in these specific phenotypes may not be a hypertensive crisis, but profound vasoplegia. We report a VHL patient undergoing synchronous robotic surgery exhibiting this paradoxical hemodynamic response. Case: A 37-year-old female with VHL syndrome presented with an incidental 3.5-cm left adrenal mass and bilateral renal masses. Biochemistry confirmed a normotensive, noradrenergic pheochromocytoma (24-hour urine normetanephrines 4.2x upper limit of normal). Renal biopsy revealed a clear cell papillary renal cell tumor. Due to prior severe intolerance to Prazosin (hypotension/dizziness with low-dose Prazosin 0.5 mg ON), we utilized amlodipine for preoperative optimization. She was only able to tolerate low-dose 2.5 mg OD alongside oral sodium chloride and ample oral fluid loading. She underwent a synchronous robotic-assisted left adrenalectomy and left midpole renal tumor excision. Strikingly, tumor manipulation did not precipitate a hypertensive crisis. Instead, she developed hypotension requiring an intravenous noradrenaline infusion prior to adrenal vein ligation and tumor removal. Vasopressor support was successfully weaned 12 hours postoperatively, and she was discharged well. Conclusion Normotensive, noradrenergic pheochromocytomas in VHL are hemodynamically fragile. Chronic catecholamine excess induces homologous desensitization and downregulation of alpha-1 adrenergic receptors. This physiological adaptation explains the normotensive presentation and highlights the intraoperative vasoplegia experienced once sympathetic tone is altered by anesthesia. While CCB monotherapy with volume expansion safely facilitates prolonged, synchronous robotic surgeries, clinicians must anticipate and combat refractory hypotension rather than classical hypertensive spikes.