Pheochromocytoma in children and adolescents: a clinical spectrum.
OBJECTIVE
The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children.


METHODS
Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed. Patients' age, sex, presentation, associated conditions, diagnostic and imaging modalities used, preoperative preparation, operative details, outcome, and follow-up were recorded.


RESULTS
The study included 21 children. Patients were diagnosed clinically and confirmed by biochemical tests. Tumors were localized by imaging studies and all were confirmed pathologically. Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder. Seventeen had sporadic and 4 had familial pheochromocytoma. Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma. The patient with bladder pheochromocytoma presented with gross hematuria. Hypertension and visual disturbances were prominent findings in the other patients with sporadic form. Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma. One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites. All patients were treated surgically. Seventeen patients were treated preoperatively with alpha-adrenergic blockade. Two patients continued to have significant visual disturbances. One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences.


CONCLUSIONS
Pheochromocytoma in children has unique characteristics. To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children. It also reflects the spectrum of pheochromocytoma in this age group.
Bissada
Nabil K
NK
Department of Urology, University of Arkansas for Medical Sciences, Little Rock, AR 72205-7199, USA. bissadanabilk@uams.edu
Safwat
Ahmed S
AS
Seyam
Raouf M
RM
Al Sobhi
Saif
S
Hanash
Kamal A
KA
Jackson
Richard J
RJ
Sakati
Nadia
N
Bissada
Mary A
MA
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods; D000367:Age Factors; D001707:Biopsy, Needle; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009367:Neoplasm Staging; D009493:Neurosurgery; D010643:Phenoxybenzamine / Q000627:therapeutic use; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D018434:Sickness Impact Profile; D015996:Survival Rate; D016896:Treatment Outcome
D010643:Phenoxybenzamine