Search

A 52-year-old African woman was found to have a pelvic mass at the age of 41. Following resection of small bowel tumors at the time, she was diagnosed with GIST and peritoneal metastases were confirmed. Despite treatment with imatinib, her disease progressed and was started on regorafenib. Due to lack of consistent insurance coverage, she took this medication intermittently for several years, and her tumor progressed to develop intra-abdominal peritoneal and pelvic metastases. She underwent debulking surgery, and her disease subsequently remained stable with regorafenib. Unfortunately, she developed hemoptysis which led to discontinuation of regorafenib, and while off treatment, the patient developed altered mentation, extreme lethargy, and poor PO intake. She was found to have a serum calcium of 16 mg/dL and was admitted for treatment of severe hypercalcemia. With intravenous (IV) fluids, calcitonin and zoledronic acid, her calcium improved to 11-12 mg/dL; however, despite additional therapies, including denosumab and repeat zoledronic acid, her calcium did not normalize. Further evaluation revealed: 25-hydroxy-vitamin D3 level 22.2 ng/mL (normal), 1,25-dihydroxy-vitamin D3 107 mcg/mL (elevated), PTH 6. 0 pg/ml (low), and PTHrP 3.3pmol/L (low). She was given a diagnosis of calcitriol-mediated hypercalcemia and started on IV hydrocortisone 100 mg every 12 hours. Her serum calcium started to improve within 48 hours and normalized after eight days to 9.8mg/dL. Her clinical condition improved, and she was discharged with prednisone 20 mg daily. At the time of her post-hospital follow up several days later, her serum calcium remained normal. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

INTRO ACTH-secreting pheochromocytoma is rare, posing challenges in diagnosis and management. Here we report two cases with different presentations and pre-operative approaches. CASES Patient 1 A 59 yo female who presented for basal cell carcinoma resection experienced hypertensive crisis intra-op and NSTEMI due to stress cardiomyopathy. The patient reported spells of palpitations, diaphoresis, tremors and pallor since 2015. She had elevated plasma metanephrine (M) 19.4 (0-0.49nmol/L), normetanephrine (NM) 24.7 (0-0.89nmol/L), 24-hour urine M 18711 (30-180mcg) and NM 11897 (128-484mcg). Imaging demonstrated a 5.6 x 5.5 x 5.8cm left adrenal mass. After pre-operative alpha and beta blockade, patient underwent left adrenalectomy. Pre-op 24-hour urine cortisol was checked as part of secondary HTN evaluation and found to be elevated at 219 (3.5-45mcg). She did not have any features of Cushing’s and this was initially felt to be an appropriate stress response. Due to persistent post-op hypotension and a low 10PM random cortisol level of 3.4mcg/dL, which was inappropriate given patient’s clinical status, hydrocortisone was started with subsequent hemodynamic improvement. Our suspicion was that she had an ACTH-secreting pheochromocytoma, which would have resulted in hypercortisolism pre-op, and secondary AI post-op. Pathology report later confirmed ectopic ACTH production with 25% cells positive with immunostaining. Patient 2 A 48 yo female was admitted for hypertensive urgency with symptoms of palpitations, facial and abdominal swelling, easy bruising and proximal muscle weakness. Her labs showed elevated 24-hour urine epinephrine 115 (0-20mcg), norepinephrine 366 (0-135mcg), plasma M 288 (0-62pg/mL) and NM 475 (0-145pg/mL), and an AM cortisol was 47.9mcg/dL. Aldosterone and renin levels were DISCUSSION Within the past 6 months, we have seen 2 cases of ACTH-secreting pheochromocytoma with different clinical symptoms and pre-op courses. Ectopic ACTH secretion may not cause classic Cushingoid features as in patient 1. Failure to recognize ectopic ACTH pre-op can lead to post-op complications such as AI. Patient 2 demonstrates that treatment of the hypercortisolemia may be necessary in order to adequately control BP and glucose levels prior to surgery.

Background: The incidence of bilateral macronodular adrenal hyperplasia (BMAH) is unknown, but growing in an era of increased utilization of imaging. Coexistence of BMAH and pheochromocytoma has not previously been reported. Clinical Case: A 63-year man presented with enlarging left chest-wall mass found to have a poorly differentiated sarcoma. Staging PET/CT identified a hypermetabolic 2.0 cm left adrenal nodule with SVU of 16.6 concerning for metastasis. Hormonal evaluation included dexamethasone suppression test with 7AM serum cortisol of 24.7 ug/dL (1cm with bilateral gland enlargement consistent with BMAH. Resection of his left adrenal gland was not pursued due to cormorbidities as well as biochemically-silent nature of his pheochromocytoma. To manage his hypercortisolism, he was recently initiated on osilodrostat after completing radiation therapy for his chest-wall sarcoma. Conclusion: This is the first case demonstrating clinical, biochemical, and imaging results consistent with bilateral macronodular adrenal hyperplasia and hypercortisolism also found to have a clinically and biochemically silent, biopsy proven pheochromocytoma. This is also a unique use of osilodrostat to manage BMAH.