2.1. Patient
The patient was a 10-month-old female. She had been treated for
gastrointestinal allergy from six months old. Watery diarrhea persisted
for 4 months, and growth impairment (body height 65 cm; -2.96 standard
deviations [SD], body weight 6.1 kg; -2.8 SD), dehydration, and
hypokalemia were recognized. A 4-cm right adrenal tumor was detected on
computed tomography, but lymphocytic and distant metastasis were not
detected (Fig. 1A, B). I123-metaiodobenzylguanidine
scintigraphy (Fig. 1C) showed no isotope accumulation in the tumor.
Laboratory data showed elevated levels of neuron-specificenolase (42.2
ng/mL), urinary vanillylmandelic acid (24.8 μg/mg Creatinine), and
urinary homovanillic acid (51.2 μg/mg Creatinine). Dopamine (6023 pg/mL)
was elevated, but adrenaline (9.0 pg/mL) and noradrenaline (82.0 pg/mL)
were within normal levels. Based on the clinical course, imaging
findings, and laboratory data, a VIPNT of adrenal was clinically
diagnosed.
Octreotide therapy was started, but the diarrhea persisted. The clinical
stage was defined as L1 according to the International Neuroblastoma
Risk Group (INRG) staging system. In addition, image-defined risk
factors (IDRF) were negative, with no tumor infiltration into the
surrounding organs or encasing the primary vessels. Tumor extirpation by
laparoscopic surgery was planned based on the staging, IDRF, and tumor
size.