Neurofibromatosis type 1 (NF1) is an autosomal dominant disease caused by mutations in the NF1 gene located on chromosome 17q11.2. Approximately 50% of patients with NF1 present with de novo…
The average size of a symptomatic catecholamine-secreting tumor is 4.5 cm. Some of the very large pheochromocytomas or paragangliomas (PPGLs) (e.g., >10 cm in diameter) are biochemically nonfunctioning. However, there…
All pheochromocytomas are biochemically undetectable when small—they require a sizable “factory” to become biochemically detectable. Thus publications that report that a laboratory test is 98% or 100% sensitive in detecting…
The prognosis of adrenocortical carcinoma (ACC) depends on several factors, including the European Network for the Study of Adrenal Tumours (ENSAT) stage, R0 (complete) surgical resection by an expert surgeon,…
The clinical presentation of pheochromocytoma has changed dramatically since Dr. Charlie Mayo successfully resected the first pheochromocytoma in North America in 1926. In the early days nearly all patients presented…
The prognosis of patients with adrenocortical carcinoma (ACC) depends on several factors, including the European Network for the Study of Adrenal Tumours (ENSAT) stage, R0 (complete) surgical resection by an…
Although adrenocortical nodularity is fairly common in patients with multiple endocrine neoplasia type 1 (MEN-1), adrenocortical carcinoma (ACC) is rare. The case presented herein is a cautionary notice for clinicians…
Most cases of adrenocortical carcinoma (ACC) are sporadic; however, some occur as a part of a hereditary syndrome, such as Li-Fraumeni syndrome, multiple endocrine neoplasia type 1, familial adenomatous polyposis,…
Most cases of primary aldosteronism (PA) are the results of either an aldosterone-producing adenoma or bilateral hyperplasia. Adrenocortical carcinoma (ACC) is a very rare cause of PA, accounting for <1%…
Approximately 50% of adrenocortical carcinomas (ACCs) hypersecrete adrenal steroids. When secretory, ACCs are typically plurihormonal—most commonly glucocorticoids and adrenal androgens. Rarely ACCs secrete a single steroid in excess. The case…
