Introduction: Steroid cell tumors are rare sex cord-stromal tumors, accounting for <0.1% of ovarian neoplasms. Extraovarian steroid cell tumors are exceptionally rare, often androgensecreting, and pose significant diagnostic challenges. Early recognition is essential to prevent prolonged morbidity from hyperandrogenism. Case: A 60-year-old female, 15 years after total hysterectomy and bilateral salpingo-oophorectomy for a large ovarian mass with massive ascites, presented with deranged liver function tests on routine follow-up. Ultrasonography and computed tomography imaging revealed multiple hypervascular lesions in the liver, retroperitoneum, and peritoneum, suggestive of metastatic disease, with normal-appearing adrenal glands. Biopsy of a liver lesion demonstrated a metastatic neoplasm with morphology and immunoprofile favoring a steroid cell tumor. However, metastasis from the adrenal cortex or an ovarian primary could not be excluded. Given the prior bilateral oophorectomy, metastatic adrenocortical carcinoma was initially suspected, prompting endocrine evaluation. Further history revealed a 1-year history of progressive virilization, including increased facial hair and frontal balding. Hormonal studies demonstrated elevated testosterone (12.2 mmol/L and reference range 0.1–1.42) and dehydroepiandrosterone sulfate (15.9 µmol/L and reference range 0.510–5.560) and the adrenocorticotropic hormone level of 11.7 pmol/L (reference range 1.60–13.9) with suppressed gonadotrophins. Additional workup for catecholamine, cortisol, and aldosterone excess was unremarkable. The discordance between androgen excess and normal adrenal imaging, despite absent ovarian tissue, suggested an extraadrenal androgen-secreting steroid cell tumor. A second histopathology review and multidisciplinary discussion with radiology, gynecologic oncology, and pathology teams were undertaken. As the disease was deemed inoperable, repeat retroperitoneal lesion biopsy confirmed metastatic steroid cell tumor and guided palliative chemotherapy. She was subsequently referred to gynecologic oncology for systemic chemotherapy. Conclusion Extra-adrenal steroid cell tumors, though rare, should be considered in patients with hyperandrogenism long after bilateral oophorectomy, especially when adrenal imaging is normal. Multidisciplinary evaluation and repeat biopsy are often crucial for establishing the diagnosis and guiding treatment.
Hyperandrogenism ; Ovariectomy ; Steroids ; Neoplasms

Introduction: Atypical parathyroid adenoma (APA) constitutes approximately 0.5–4.0% of all cases of primary hyperparathyroidism (pHPT). Here, we report a case of APA presenting with severe hypercalcemia, complicated with renal impairment, bilateral medullary nephrocalcinosis, and multiple fragility fractures. Case: A 45-year-old male initially presented with a 6-month history of constipation, polyuria, lethargy, bone pain, and difficulty in initiating micturition. Laboratory investigations revealed impaired renal function with an estimated glomerular filtration rate of 41.4 mL/min, severe hypercalcemia (4.07 mmol/L), and an elevated intact parathyroid hormone (iPTH) level of 104.0 pmol/L (reference range: 1.58–6.03 pmol/L), confirming the diagnosis of pHPT. He was also found to have vitamin D deficiency, with a serum total 25-hydroxyvitamin D level of 46 nmol/L. Ultrasound of the abdomen demonstrated bilateral medullary nephrocalcinosis, while neck ultrasound and Tc-99 m sestamibi parathyroid scintigraphy revealed a concordant lesion in the posterior aspect of the left thyroid lobe, suggestive of a parathyroid adenoma. He returned 3 months later with closed fractures of the right subtrochanteric femur and the right humerus following a fall from standing height. In view of persistent hypercalcemia despite hyperhydration and treatment with zoledronic acid, subcutaneous denosumab (60 mg) was administered, resulting in an improvement in serum calcium levels. A left inferior parathyroidectomy was then performed concurrently with internal fixation of the right femur. The surgery was uneventful. Histopathological examination confirmed an atypical parathyroid adenoma. Postoperatively, the serum calcium and iPTH levels normalized, and the patient remained asymptomatic and normocalcemic during regular follow-up. Conclusion APA remains a diagnostic and therapeutic challenge due to its clinical, biochemical, and histopathological features of equivocal malignancy. Surgical resection remains the mainstay of management of APA, and long-term surveillance is essential in view of its uncertain malignant potential and risk of recurrence.
Hypercalcemia ; Parathyroid Neoplasms

Introduction: Giant prolactinomas represent 2–3% of prolactin-secreting pituitary adenomas and show a male predominance. They present with mass effect symptoms and hypogonadism, but may be overlooked, leading to delayed diagnosis. Although dopamine agonists are first-line therapy, management remains challenging due to the large size and invasive behavior. This study aims to describe the clinical and radiological features, treatment modalities, and outcomes of three cases of giant prolactinomas. We retrospectively reviewed three men with giant prolactinomas, including their clinical, biochemical, and radiological features, along with treatment and outcomes. Cases: Three male patients aged 35–59 years with giant prolactinomas were included. Two patients presented with visual disturbances, headache, and features of hypogonadism, while one patient had acute confusion and visual loss secondary to obstructive hydrocephalus requiring ventriculoperitoneal shunt insertion. Imaging in all cases demonstrated large invasive pituitary macroadenomas with extensive local extension. Baseline serum prolactin levels were markedly elevated, ranging from 86,568 to 441,116 uIU/mL (86–324 uIU/mL). All patients had secondary hypogonadism, while secondary hypothyroidism and hypocortisolism were each identified in two patients. One patient also had poorly controlled diabetes mellitus at presentation. Dopamine agonist therapy was initiated as the primary therapy for all the patients. Two patients developed cerebrospinal fluid leak following initiation of low-dose dopamine agonist therapy (one patient received cabergoline 0.25 mg weekly, while another received 0.5 mg weekly), which resolved spontaneously with conservative management. Serum prolactin levels decreased markedly, with significant improvement in symptoms related to mass effect following treatment. Conclusion Giant prolactinomas may present with significant mass effect and multiple pituitary hormone deficiencies. Dopamine agonists remain the cornerstone of management and can result in substantial biochemical and clinical improvement even in large invasive tumors. However, rapid tumor shrinkage may lead to complications such as cerebrospinal fluid leak, highlighting the importance of close monitoring during treatment initiation.
Prolactinoma

Introduction: Arginine vasopressin (AVP) deficiency is an uncommon but important presentation of infiltrative hypothalamic– pituitary disorders. In adults, isolated AVP deficiency with pituitary stalk thickening is diagnostically challenging, especially in the absence of systemic disease. Langerhans cell histiocytosis (LCH) is a rare cause and may precede systemic involvement by several years. Case: We report a 42-year-old male who presented in 2017 with polyuria and polydipsia, with urine output of up to 10 L/ day. AVP deficiency was confirmed by the water deprivation test, and desmopressin was initiated. Initial pituitary magnetic resonance imaging (MRI) was normal, baseline anterior pituitary hormonal evaluation was unremarkable, and contrast-enhanced computed tomography (CECT) of the thorax and abdomen showed no abnormalities. Repeat pituitary MRI 1 year later demonstrated loss of the posterior pituitary bright spot with pituitary stalk thickening. In the absence of systemic involvement, a presumptive diagnosis of lymphocytic hypophysitis was made, and pituitary biopsy was deferred due to the high procedural risk. Serial pituitary MRIs over the following years showed persistent infundibular thickening and continued absence of the posterior pituitary bright spot, without interval progression or development of additional hormonal deficiencies. The patient remained clinically stable until June 2024, when new-onset left hip pain prompted MRI, revealing a heterogeneous mass involving the femoral neck and intertrochanteric region with a pathological fracture. Histopathological examination following wide resection confirmed LCH, with negative BRAF V600 mutation. Postoperative PET scan revealed a multisystem disease involving the skeleton, lymph nodes, spine, and gastrointestinal tract, with no bone marrow involvement. He subsequently completed six cycles of intravenous methotrexate and cytarabine. Conclusion Adult-onset AVP deficiency may be the earliest manifestation of occult multisystem Langerhans cell histiocytosis. This case highlights the importance of longterm follow-up and reconsideration of the initial diagnosis when new systemic features emerge.
Histiocytosis, Langerhans-Cell

Introduction: Insulinoma is a rare functioning pancreatic neuroendocrine tumor and the most common cause of endogenous hyperinsulinemic hypoglycemia. Although biochemical confirmation is usually straightforward, tumor localization may be difficult when lesions are small and not detected on conventional cross-sectional imaging. In such cases, endoscopic ultrasound (EUS) plays an important role in identifying occult lesions and facilitating definitive treatment. Case: A 52-year-old female was admitted in April 2025 with recurrent seizures secondary to hypoglycemia for 3 years, with increasing frequency over time. She fulfilled Whipple’s triad, with documented capillary glucose of 1.8 mmol/L during an episode and symptom resolution following glucose administration. A supervised prolonged fasting test confirmed endogenous hyperinsulinemic hypoglycemia, with plasma glucose 1.4 mmol/L, insulin 122 pmol/L, and C-peptide 1,010 pmol/L. Short Synacthen test demonstrated adequate adrenal reserve. Due to persistent hypoglycemia, she required high-dose diazoxide. Contrast-enhanced computed tomography abdomen did not reveal a pancreatic lesion but incidentally detected a right ovarian teratoma. She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy in June 2025, with histopathology confirming a mature cystic teratoma without malignancy. However, hypoglycemic episodes persisted. Further evaluation with EUS in July 2025 identified a highly vascular isoechoic 8 × 8 mm lesion in the pancreatic body. Fine-needle biopsy confirmed a well-differentiated neuroendocrine tumor (WHO grade 1) with Ki-67 index of 2%. She subsequently underwent EUSguided radiofrequency ablation in August 2025. Follow-up EUS in December 2025 showed post-ablation change, and her hypoglycemic episodes resolved completely, allowing diazoxide to be discontinued. Conclusion This case highlights the diagnostic challenge of occult insulinoma in the presence of negative conventional imaging. EUS was pivotal for tumor localization and tissue diagnosis, while EUS-guided radiofrequency ablation provided effective minimally invasive treatment in a carefully selected patient.
Insulinoma ; Tomography, X-Ray Computed