Introduction: Secondary hypertension due to elevated catecholamine secretion is typically seen in patients with pheochromocytomas and rarely, in those with paragangliomas. We present an unusual case of a young patient with episodic hypertension and elevated metanephrines possibly due to ectopic adrenal tissue. Case: A 31-year-old female with new-onset hypertension and uterine fibroids presented with left upper quadrant abdominal pain and high blood pressure. She had a partial hysterectomy 6 months ago and since then, had persistent abdominal and back pain. Over the past year, she also had worsening anxiety attacks with sudden sensation of flushing and lightheadedness. The episodes lasted for a few minutes several times a week, and were unresponsive to sedatives. On presentation, significant findings included: elevated blood pressure (178/114 mmHg), mild hypercalcemia, and an inappropriately elevated iPTH consistent with primary hyperparathyroidism. Plasma metanephrines were elevated; 24hr 5-HIAA was normal. CT of the abdomen showed a solid, lobulated retroperitoneal mass of 2.1cm with 170 Hounsfield units. Octreotide scan did not show abnormal areas of uptake. The mass was presumed to be a paraganglioma and she underwent surgical resection. The mass seen on imaging turned out to be a hemangioma however an additional mass removed from the area of the aortic bifurcation was found to be ectopic adrenal tissue. Post-op labs showed decreased metanephrines (from 101 to 23pg/mL) and normetanephrines (from 234 to 116pg/mL). Repeat CT scan 4 months later showed no new masses. Post-op, the patient’s symptoms improved but not completely resolved. Discussion : Ectopic adrenal tissue is an exceedingly rare finding in adults. It is more common in the pediatric population and is typically identified incidentally during pelvic surgery where groin exploration is performed. Sullivan et al. studied the prevalence of ectopic adrenal tissue and noted it was discovered in <3% of ~900 children who underwent groin exploration surgery, none of which were female 1 . With aging, ectopic adrenal tissue typically atrophies due to functioning adrenal glands making it an even less common entity in adults. Our case is even more unusual in that the ectopic tissue appears to be functioning by secreting catecholamines. Furthermore, given the primary hyperparathyroidism in the setting of a potential catecholamine-producing tumor, we pose whether this warrants genetic testing for multiple endocrine neoplasia (MEN), specifically MEN 2A. Reference : 1-J.G. Sullivan, M. Gohel, R.B. Kinder. Ectopic adrenocortical tissue found at groin exploration in children: incidence in relation to diagnosis, age and sex. BJU Int 2005; 95:407-410

Introduction : Pheochromocytomas are a rare neoplasm of chromaffin cells of the adrenal medulla with an annual incidence in the United States between 500 to 1600 cases, of which about 10% are malignant. Due to this rarity, adrenal masses, especially in the context of other concurrent primary tumors, can be easily confounded with metastasis. Case Report : A 56-year-old male with diabetes mellitus and hypertension presents with left flank pain associated with hematochezia, constipation, and an 8.2kg weight loss over 2.5 months. Physical examination was unremarkable. Abdominal CT scan showed a 4x5cm left adrenal gland mass with infiltration of peri-adrenal tissues and hemorrhage. Dexamethasone suppression test was normal. Plasma metanephrines and aldosterone were not elevated. Repeat CT a month later showed marked increase of the mass to 6.2x5.8cm and an ascending colon mass. Colonoscopy with biopsy revealed a moderately differentiated adenocarcinoma with immunohistochemistry (IHC) staining negative for MSH2 and MSH6 mismatch repair proteins, typical of Lynch syndrome. The patient had a strong family history of malignancy, positive for endometrial cancer (mother) and colon cancer (mother/brothers). A right segmental hemicolectomy and left adrenalectomy-nephrectomy was performed with a presumption that the adrenal mass was a metastatic site. On pathology, the colon specimen confirmed a 5x4x2cm adenocarcinoma. Surprisingly, the adrenal specimen revealed a 7x6x6cm malignant pheochromocytoma with a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) of 18 with IHC also negative for MSH2 and MSH6. Repeat CT 4 months post-resection showed new lymphadenopathy followed by PET/CT revealing a 2.7x3.4cm FDG avid left para-aortic lymph node consistent with metastasis and a 2.5cm non-FDG avid cystic mass in the pancreatic tail. Discussion : There is a high prevalence of germline mutations associated with pheochromocytomas, commonly including succinate dehydrogenase (SDH) subunit genes, SDHAF2, TMEM127, MAX-gene, and three main associated syndromes including von Hippel Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. Lynch Syndrome is diagnosed by a mutation in at least one of these mismatch repair genes: MLH1, MSH2, MSH6, and PMS2. It is often associated with extra-colonic cancers including the endometrium, gastric, biliary, urinary tract, pancreas, small intestine, brain, and skin. There is a growing number of cases reporting neuroendocrine tumors in the setting of Lynch Syndrome. To date, there are only two cases reporting an association between pheochromocytoma and Lynch Syndrome, neither of which were described as malignant pheochromocytomas. We present a case of MSH2 and MSH6 mismatch repair protein deficiency in both colon and adrenal masses, suggesting that Lynch Syndrome may be an additional rare risk factor for malignant pheochromocytoma.

No abstract

No abstract

No abstract