Background Pheochromocytoma (PC) is a rare neuroendocrine tumor with an annual incidence of 2 to 8 cases per million that can secrete excess catecholamines precipitating hyper-adrenergic symptoms. In rarer instances, PCs can also secrete substances other than catecholamines. Case A 51-year-old female underwent MRI lumbar spine that demonstrated an incidental 13 cm right adrenal mass. History was significant for newly diagnosed hypertension and a five-year history of episodic headaches, diaphoresis, palpitations and anxiety. A previous cardiac evaluation including Holter monitor, echocardiogram, and stress test was unrevealing. Physical exam was significant only for chronic back pain and BMI 20.4. Labs revealed elevated serum catecholamines with metanephrines and normetanephrines >40x and >65x the upper limit of normal respectively, elevated urine catecholamines and elevated chromogranin A suggestive of PC. Labs also demonstrated mild hypercalcemia >10.3 mg/dL (8.6-10.4 mg/dL) with elevated PTH 78 pg/mL (14-64 pg/mL) and low vitamin D 25-OH 25 ng/mL (30-100 ng/mL). Calcitonin, albumin and phosphorus were normal. She was treated with non-competitive alpha and beta-blockade and metyrosine before undergoing successful right adrenalectomy. Post-operative labs demonstrated normalization of catecholamines, PTH and calcium. Surgical pathology confirmed the diagnosis of PC and demonstrated tumor cells which stained for PTH confirming ectopic PTH secretion, a rare phenomenon. The PC was very large with an elevated PASS (Pheochromocytoma of the Adrenal Gland Scaled Score) indicating increased risk for malignancy/recurrence; however, there was low Ki-67 activity and no capsular involvement, which suggested benign behavior. Furthermore, an MIBG scan was negative for metastatic disease, and an extensive genetic workup for familial PC/PGL (including MEN, RET, SDHx) was negative. Conclusion PC is a rare neuroendocrine tumor that can secrete excess catecholamines precipitating hyper-adrenergic symptoms and potentially fatal complications including hypertensive crises, cardiovascular disease, ischemia and multi-organ failure if untreated. Consequently, early recognition of PCs is imperative. The patient in this case had a five-year history of symptoms seen with PC and newly diagnosed hypertension despite normal BMI and negative cardiac workup. However, she had no evaluation for secondary causes of hypertension. Despite the large tumor size and significantly elevated catecholamines, she functioned without significant morbidity potentially due to adrenergic receptor desensitization. This case also highlights the rare phenomenon of ectopic secretion in PCs, specifically ectopic PTH secretion. A 2019 systemic review identified only 150 cases of pheochromocytomas/paragangliomas with ectopic hormone secretion – of those, only 17 secreted PTH or PTH-related peptide [1]. It is important to consider ectopic secretion in patients with PCs as they may have additional clinical manifestations associated with the hormone or substance secreted. Fortunately, this patient had no overt manifestations of hyperparathyroidism or hypercalcemia. References [1] Angelousi A, et al. Malignant pheochromocytomas/paragangliomas and ectopic hormonal secretion: case series and review of literature. Cancers. 05/2019;11(724). Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
A 56 year old male with history significant for myasthenia gravis, non-functional pituitary macroadenoma and age related macular degeneration (ARMD), who presented to the hospital with 3 days of diplopia, nausea, vomiting, generalized weakness, and bitemporal headaches. Two months prior to presentation, he was diagnosed with an idiopathic disseminated rash for which he was treated with high dose steroids. Steroids were tapered rapidly and stopped abruptly 4 days ago. Initial presentation was significant for hypotension with BP of 95/64, hyponatremia [sodium 127(136 - 145 mmol/L)], chloride was 90 (98 - 107 mmol/L), potassium 5.9 (3.4 - 5.3 mmol/L) consistent with acute adrenal crisis. He was immediately initiated on high dose Solu-Cortef and intravenous fluids. Of historical significance was new visual field deficit which had occurred in the background of his existing ARMD. This raised the suspicion for pituitary apoplexy. MRI pituitary showed enlarging pituitary adenoma (2.1×2. 0×2.6 cm from 1.6×1.6×2.3 cm 5 months prior) with necrotic changes and internal signal complexity. Ophthalmology was consulted stat and on confirmation of new right optic neuropathy, urgent surgical decompression was performed via transnasal endoscopic resection of pituitary tumor by neurosurgical and ENT team. Further lab testing revealed secondary adrenal insufficiency and pan hypopituitarism. Patient's vision recovered remarkably well post operatively and he was initiated on thyroid hormone supplementation in addition to continuing steroids. He was discharged with recommendations to follow up with endocrinology outpatient for further management. Conclusion Pituitary apoplexy although well known can easily be missed clinically without a high degree of suspicion. While our patient presented with acute adrenal crisis leading to some of the presenting complaints, his new visual field deficits were unaccounted for. This case highlights the importance of a through history and examination to delineate between acute and chronic visual field symptoms especially in the background of pre-existing retinal diseases. Secondly, although the patient's adrenal insufficiency could be due to apoplexy, we hypothesize that it is more likely that it could have resulted due to abrupt discontinuation of high dose steroids leading to adrenal crisis. The fluctuation in BP in turn could have triggered an apoplectic event in an otherwise stable macroadenoma leading to two independent but interrelated endocrine emergencies. Presentation: No date and time listed
Background Secondary causes for low bone density may be present in 30% of women and up to 50% of men with low bone density and osteoporosis. Idiopathic hypercalciuria is associated with lower bone density and higher bone turnover, and an increased risk of fractures. Thiazide diuretics have been used often for treatment for idiopathic hypercalciuriaCase Series: We present a case series of 9 patients who presented for low or declining bone density. They underwent workup for secondary causes of osteoporosis and were noted to have idiopathic hypercalciuria with high bone turnover on initial evaluation. They were started on thiazide diuretics or amiloride and the dose titrated up daily based on their blood pressure tolerance. They were monitored for hypokalemia and hyponatremia. Several of these patients exhibited a > 30% decrease in bone resorption, as measured by urine N-telopeptide on a second fasting specimen. Follow-up bone density at 1 year and 3-year after starting diuretic therapy showed stability or significant increase in bone density. Some of these patients were able to maintain bone density in the osteopenic range on follow up for > 6 years. Conclusion Some patients with idiopathic hypercalciuria may have a more robust response to thiazide diuretics compared to others even in the absence of anti-resorptive therapy. Significant reduction of > 30% in bone resorption markers may identify patients who are more likely to have a robust response in the bone density after starting thiazide medications. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
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