DIAGNOSIS AND TREATMENT OF PHEOCHROMOCYTO MA IN CHILDHOOD - A RETROSPECTIVE STUDY CONDUCTED IN FRANCE

In children, pheochromocytoma and paraganglioma are exceedingly rare a nd may exhibit a number of specific features including familial aggreg ation, a multifocal distribution, and a malignant behavior. A retrospe ctive study of 24 cases of pheochromocytoma and paraganglioma managed in French Society for Pediatric Oncology centers was conducted with sp ecial attention to long-term outcomes. Median age at diagnosis was 12. 5 years, and the most common presenting symptom was arterial hypertens ion. Catecholamine assays and modern imaging methods (MIBG scintigraph y, ultrasonography, computed tomography, and magnetic resonance imagin g) established the diagnosis and determined the location of the tumor, obviating the need for more invasive investigations. As many as six o f the 24 patients had synchronous tumors in both adrenal glands, and e ight had a familial form. There were no deaths during surgery or the i mmediate postsurgical period. Median follow-up was 5.2 years. One pati ent died, from progression of the tumor. Fourteen patients were still in complete remission at last follow-up, whereas ten had had a recurre nce and six of these ten had evidence of malignant behavior (local inv asion or metastatic spread). In half the patients, repeated or extensi ve surgery was responsible for sequelae, of which the most common were endocrine deficiencies. Factors associated with a higher risk of recu rrence or malignancy included location outside the adrenal gland, subs tantial locoregional spread at the time of the first surgical procedur e, and large tumor size. Early diagnosis (especially in patients with a family history or a predisposing disease) and accurate assessment of tumor spread before and after surgery add greatly to the likelihood o f complete surgical excision, which is the only curative therapy, incl uding during recurrences. Patients in remission should receive long-te rm follow-up (catecholamines, MIBG scintigraphy) on a yearly basis.