Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center.
OBJECTIVE
The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children.


METHODS
A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma.


RESULTS
There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of > 6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 +/- 32 months.


CONCLUSIONS
The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of > 6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
Pham
Tuan H
TH
Department of General and Pediatric Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Moir
Christopher
C
Thompson
Geoffrey B
GB
Zarroug
Abdalla E
AE
Hamner
Chad E
CE
Farley
David
D
van Heerden
Jon
J
Lteif
Aida N
AN
Young
William F
WF
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000601:surgery*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D018365:Neoplasm, Residual; D010235:Paraganglioma / Q000453:epidemiology / Q000235:genetics / Q000601:surgery*; D010673:Pheochromocytoma / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis