Bladder Pheochromocytoma in a 10-Year-Old Girl

Abstract

Pheochromocytoma of the bladder in children is a rare entity that usually presents with the triad of sustained hypertension, hematuria and post-micturitional syncope. The youngest previously reported patient was 11 years old.' We report on a 10-year-old girl with pheochromocytoma of the bladder. Not only does this case lower the age of inclusion but the clinical presentation highlights the need for a thorough history and high index of suspicion for accurate diagnosis. Pheochromocytomas in children are more often multicentric and extra-adrenal than they are in adults. Only about 2% of pheochromocytomas in children are malignant but most of these are extra-adrenal.2 CASE REPORT A 10-year-old white girl was referred for terminal hematuria 6 weeks in duration. The patient initially denied any significant health problems, although medical history included severe headaches often associated with voiding. hevious evaluation by a neurologist included cranial magnetic resonance imaging (MRI) which was normal. The headaches resolved spontaneously and had not recurred in more than 12 months. Physical examination, including heart rate and blood pressure, was normal. Urinalysis revealed red blood cells too numerous to count. Urine culture was negative. Bladder ultrasound showed an intravesical mass (fig. 1). At the time of transurethral biopsy the patient had blood pressure swings (up to 200 mm. Hg systolic) with manipulation of this 3 X 3 cm. vascular tumor located between the ureteral orifices on the trigone. The biopsy was nondiagnostic and showed only hemorrhagic material. Because of our suspicion of a neuroendocrine tumor, fractionated urinary catecholamines and vanillymandelic acid levels (VMA) were obtained. The 24-hour urine study revealed norepinephrine 559 pg. (normal 13 to 65), epinephrine 5.3 pg. (normal 0 to lo), dopamine 1,203 pg. (normal 60 to 460) and VMA 1,767 pg. (normal 20 to 550). Since the patient remained normotensive and a firm histological diagnosis had not been established, a second transurethral biopsy was done using general endotracheal anesthesia with phentolamine successfully to treat short periods of hypertension. This deeper biopsy revealed a vascular mass of questionable histology by routine microscopic examination. Electron microscopy revealed dense membrane bound granules (90 to 400 nm.) in the cytoplasm, and abundant mitochondria and rough endoplasmic reticulum. These findings were consistent with a diagnosis of pheochromocytoma.3 Computerized tomography and MRI revealed a calcified mass without obvious multicentricity, although there was concern about possible invasion into the anterior vaginal wall (fig. 2). A chest x-ray was normal. In preparation for removal of the pheochromocytoma the patient was treated with a 10-day course of a blockade and intravenous hydration in the hospital. She had no cardiac