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.