Pheochromocytoma in children.

BACKGROUND/
PURPOSE: Etiopathogenesis, management, and outcome of pediatric pheochromocytoma (PHEO) still is obscure because of limited number of cases. Therefore, a retrospective clinical study was performed to present an updated picture of the entire spectrum of pediatric PHEO based on the authors' 30 years' experience consisting of one of the largest noncollected series treated in a single medical center.
METHODS: Records of patients treated for PHEO in the authors' unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical and family history, clinical characteristics, diagnostic methods, treatment, pathologic findings, and outcome.
RESULTS: Sixteen children with a mean age of 10.7 +/- 2.9 years consisting of 12 boys and 4 girls were treated for PHEO. Most of the tumors were right sided (n = 6) and bilateral (n = 6). Sporadic cases of PHEO accounted for 14 patients (88%), whereas 2 children had von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia type 2b (MEN2b). Hypertension was the most common symptom followed by headache and sweating. The diagnosis of PHEO was made by laboratory and radiologic studies. Preoperative medical therapy was done in all patients. Laparotomy confirmed that 11 patients had localized, 4 patients had regional, and 1 patient had metastatic disease. The localized tumors were excised totally by bilateral (n = 4) and unilateral (n = 6) adrenalectomy. Surgical procedures performed for regional disease were total excision (n = 2), incisional biopsy (n = 1) and partial excision (n = 1). Incisional biopsy could be taken only from a patient with metastatic disease at presentation. Two patients with localized disease and 2 patients with regional disease had benign recurrences in right (n = 2) and left (n = 2) adrenal glands within 3 to 7 years after operation. Total excision of the recurrent tumors was done in all patients. Pathologic examination found apparently malignant features in 3 patients who presented with regional (n = 2) or metastatic (n = 1) disease and underwent incisional biopsy (n = 2) or partial excision (n = 1). Pathologic features suggestive of malignancy were noted in 4 patients presenting with regional (n = 2) and localized disease (n = 2). Apparently benign pathologic features were noted in the remaining 9 patients. There was not any operative mortality in our series. Adjuvant chemotherapy was commenced postoperatively in all patients with malignant and suggestive of malignant pathologic features. During the long-term follow-up for 16 years, 3 patients died (19%). One patient with VHL disease died of astrositoma 5 years after her recurrent PHEO was excised. Of the 3 patients with malignant disease, 2 patients in whom only incisional biopsies were done had distant metastases and died of disease within 2 years. Another patient with malignancy who had MEN2b was lost to follow-up.
CONCLUSIONS: Early diagnosis and total excision are the most important aspects of accurate treatment for childhood PHEO. Pre- intra- and postoperative medical management is as important as the surgical procedure. Our surgical treatment policy is mainly minimizing the risk of recurrence while preserving adequately functioning adrenal medullar tissue. Incomplete excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy. Because of the steadily increasing incidence of precancerous genetic syndromes related to adrenal glands and poor prognosis of advanced-stage PHEO, childhood cases of hypertensive disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults.