Endocrine Abstracts

Introduction: Extra-adrenal paragangliomas are neoplasms arising from cells of neural crest origin anywhere along the distribution of the sympathoadrenal neuroendocrine system. Nearly 85% are intra-abdominal, 12% are intrathoracic, and 3% are cervical. 1 Some of the unusual sites for paragangliomas include the kidney, urethra, urinary bladder, prostate.

Case report: A 27-year old man presented with sever hypertensive crisis. He had a medical history of high blood pressure since the age of 15 years old. The patient had recurrent crises of three classic symptoms of pheochromocytoma: headache, swatting and heart palpitation in association with markedly elevated blood pressure (220/100 mmHg). These crises are consistent and sever after ejaculation, associated with tremors weakness and abdominal pain. On exam the patient was 175 cm tall and weighed 94 kg. He had no signs of neurofibromatose type1, MEN type 2 or Von Hippel-Lindau syndrome. Laboratory data showed elevated metanephrines in 24-h urine collection for three consecutive days (20.94 μmol/l >4 times the upper limit of normal). NSE level was high 54.1 ng/ml (12.5–25). MRI of the body showed normal size prostate with a nodule on the right side measuring 9 mm, T₂ hyperintense with microcalcification. Adrenal glands were normal and no other lesions were seen on the rest of the body. MIBG sintigraphy showed no uptake (physiologic uptake in the urinary tract). Prostatectomy was indicated after medical therapy preparation. Unfortunately surgery was refused by the patient.

Conclusion: Prostatic paragangliomas are very rare tumors, with no more than ten cases reported in the literature. Because of the nonspecific clinical symptoms, it is difficult to give a definite diagnosis preoperatively. Biochemical studies can confirm the diagnosis of pheochromocytoma. However topographic diagnosis remains a challenge in ectopic pheochromocytomas.