[Malignant pheochromocytoma. A case report in a 10-year-old boy with vertebral metastasis]. / Phéochromocytome et problèmes de malignité. A propos d'un cas de phéochromocytome malin avec invasion vertébrale.

ABSTRACT

This is a case report of a malignant pheochromocytoma in a 10 years old boy with attacks of abdominal pains, diarrhea, visual difficulties and hypertension. No familial history was noted. At that time, a large functionally active and extraadrenal tumor was surgically removed just above the diaphragm. 3 years later, a retroperitoneal active recurrence required a 2nd operation. Then, a vertebral invading obliged to resect almost the whole T 12 and to stabilise the rachis with a double osteosynthesis, by a posterior and anterior approach. Although this may represent compression and invasion of the bone rather than a true metastasis, the authors think that the clinical course demonstrates malignancy. They emphasize the fact that the acceptance of strict criteria should help to overcome much of the confusion regarding the diagnosis of a malignant pheochromocytoma. It is generally accepted that such a diagnosis cannot be made in the absence of metastases, irrespective of the histologic picture. The technical problems of peri-neural surgery are also discussed.