Background. Pheochromocytoma is neuroendocrine tumor classified as a rare disease (ORPHA:717). Pheochromocytomas are usually benign, however approximately 10% of them can be malignant. Currently no molecular or histopathological markers are available to detect early stages of malignancy and only radiological evidence of metastases after initial operation suggests malignant pheochromocytoma. Surgical resection of the metastatic tumors is the treatment of choice for ma lignant pheochromocytoma. Postoperative follow-up is based on annual biochemical testing of urinary or plasma metanephrines, chromogranin A as well as radiological imaging studies every 1-2 years.
Material and methods. We are presenting a clinical case of a patient who underwent surgical treatment of pheochromocytoma when she was 19 years old. The patient was at a high-risk group because of young age and based on guidelines recommendations lifelong annual follow-up should be offered for her. 6 months post surgery she developed typical symptoms again. After clinical and radiological evaluation the recurrence of malignant pheochromocytoma was diagnosed. However, due to unplanned pregnancy the patient refused surgery. Pregnancy went well and the successful cesarean section was periormed on the 39th week of pregnancy. Two surgical resections of the recurrent neoplasm were performed, followed by postoperative administration of chemotherapy. The patient is still on follow-up, her laboratory tests, radiological analyses are within normal limits without any signs of recurrent disease.
Conclusions. Continuous surveillance of high-risk patients following surgical management of pheochromocytoma is significant in prevention, early detection of tumor recurrence as well as preventing progression of metastatic process.
