A 21-year-old woman was referred to our hospital to treat bilateral pheochromocytomas (PCCs) after a diagnosis of multiple endocrine neoplasia type 2A (MEN2A). We performed bilateral laparoscopic adrenalectomy. One year after the operation, urinary fractionated metanephrines in 24-h urine increased. MRI showed a 30 mm tumor on the interaortocaval region and 123I-MIBG concentrated in this area. We excised the tumor and performed para-aortic lymphadenectomy. Histopathologic examination confirmed a PCC arising from ectopic adrenal tissue. Urinary fractionated metanephrines in 24-h urine declined to basal levels immediately after the operation. We detected no recurrence of paraganglioma or PCC for 5 years after the treatment.
- Most ectopic adrenal tissue is associated with no symptoms and contains only the adrenal cortex.
- Adrenocortical tumors sometimes arise from ectopic adrenal tissues similarly to in the normal adrenal gland.
- PCC arising from ectopic adrenal tissue occurs infrequently.
- MEN2-related PCC is accompanied by adrenal medullary hyperplasia, which might be part of tumorigenesis.