Endocrine Abstracts

Pheochromocytoma (PCC) is a rare neuroendocrine tumor, mainly sporadic, many cases are discovered incidentally by computed tomography or magnetic resonance imaging of the abdomen. Malignancy in pheochromocytoma is difficult to diagnose microscopically. Therefore, only the presence of distant metastases, derived from pleomorphic chromaffin cells, is widely accepted as a criterion of malignancy (1)

Case presentation: In March 2016, a 43 year old women presented with an episodic hypertension. Abdomen CT scan showed a mass in adrenal gland measuring 4.2×3.8 cm in the right. Preoperatively blood test confirmed PCC and a right adrenalectomy was performed. After histological evaluation pheochromocytoma was also certified. Patient had remission of symptoms for almost half a year until hypertension episodes appeared again. In September 2017, abdomen CT scan showed a contrast accumulation in the right adrenal place Hormonal test showed a significant increase in chromogranin A level (2594.2 ng/ml), immeasurable plasma normetanephrine level, normal metanephrin (0.2 nmol/l, n. 0–0.456). Other tests (morning cortisol, aldosterone, renin, DHEA-S, 17OHP, PTH levels) were also normal. For further evaluation an I-123 MIBG scan was performed and it showed intensive activity in right adrenal gland projection, moderate uptake of I-123 MIBG in sternum, ileum and low uptake in lowest right costal bone. There were two suspicious activity nodules in the right liver lobe margin, which were confirmed as metastasis during computed tomography imaging of the abdomen and pelvis. During chest CT scan, multiple metastasis in both lungs with pathological mediastinal, parathracheal and axillary lymph nodes were diagnosed. Laparoscopy with adrenal mass and partial liver resection was done. In January, 2018, patient was operated again. Unfortunately, during laparotomy multiple metastases in peritoneum cavity, liver surface, liver gate, ligaments of both small and large intestines were found. Samples from masses in liver gates were taken and malignant PCC was confirmed. At this moment, treatment by chemotherapy is being planned.

Comments: After PCC diagnosis and surgical extirpation, proper, intensive follow-up is very important in case to exlude malignant PCC.

Reference

[1] Ajallé R., Plouin P.F., Pacak K., Lehnert H. Treatment of malignant pheochromocytoma. J Hormone and Metabolic Research 2009;41(9): 687–696.