Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients.
Sporadic pheochromocytoma is a rare tumor of childhood and accounts for less than 1% of cases of hypertension. We describe the presentation and outcome of 19 adolescents with sporadic pheochromocytoma seen over past 10 years at a tertiary care center in north India. The mean age (+/- SD) at presentation was 15.1 +/- 2.4 years with range from 9-18 years. The male to female ratio was 12:7. The lag time between onset of symptoms to diagnosis ranged from 1 month to 5 years with mean (+/- SD) of 1.09 +/- 1.02 years. The majority of children presented with hypertension and paroxysms. Paroxysms, characterized by the triad of headache, palpitations and sweating, was present in 13 (68%) of these patients. Twelve (63%) patients had postural fall in blood pressure, ten (53%) had abdominal pain, four (21%) had visual blurring, and three (16%) each had palpable abdominal mass and significant weight loss at presentation. Nausea and vomiting are common symptoms in children with pheochromocytoma and were present in six (32%) and three (16%) patients, respectively. Café-au-lait macule was present in only two (11%) patients. Urinary vanilyl mandelic acid (VMA) was found to be significantly high in ten (53%) patients, and urinary epinephrine and norepinephrine in eight (42%). Six (32%) patients had both VMA and urinary epinephrine and norepinephrine within normal limits and five (26%) had significant elevation of both. The tumor was localized by ultrasonography in 17 (89%) patients and by computed tomography in 18 (95%), and in one patient it was localized by 131I-MIBG scan. Sixteen (84%) patients had adrenal pheochromocytoma (including four with bilateral masses), while the remaining three (16%) had abdominal extra-adrenal pheochromocytoma originating from sympathetic ganglions. The mean (+/- SD) diameter of the tumor was 4.4 +/- 1.7 cm, ranging from 2.2-7.5 cm. Pre-operatively, hypertension was managed by phenoxybenzamine in six (32%), sustained release prazosin in 12 (63%), beta-blockers in 14 (74%), calcium channel blockers in 12 (63%), and angiotensin converting enzyme inhibitors and diuretics in only two (11%) patients. Eighteen (85%) patients underwent exploratory laparotomy for removal of the tumor. On follow-up, 13 (72%) patients became normotensive, while six (32%) patients continued to have hypertension. In conclusion, childhood pheochromocytoma is characterized by atypical symptomatology; ultrasonography is a useful modality in localizing the lesions in the majority of patients; surgery is rewarding in most patients.
Bhansali
A
A
Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. anilbhansali_endocrine@rediffmail.com
Rajput
R
R
Behra
A
A
Rao
K L N
KL
Khandelwal
N
N
Radotra
B D
BD
D019797:3-Iodobenzylguanidine; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*; D000317:Adrenergic alpha-Antagonists / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010673:Pheochromocytoma / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014642:Vanilmandelic Acid / Q000652:urine
D000317:Adrenergic alpha-Antagonists; D002121:Calcium Channel Blockers; D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine; D014642:Vanilmandelic Acid