The patient received chemotherapy using epirubicin, oxaliplatin, and fluorouracil for 6 months, but developed a new hepatic lesion at the end of chemotherapy ( Figure 1F). Laparoscopic biopsy of the hepatic lesion revealed active metastatic cancer and residual cancer cells at the antrum. Palliative therapy was subsequently initiated using crizotinib, a dual ALK/MET inhibitor. Three months later, patient achieved PET-negative remission ( Figure 1G) lasting for 8 months before presenting with recurrent headaches and vision loss. Imaging studies revealed significant disease progression with mediastinal and retroperitoneal hypermetabolic lymphadenopathy, multiple extensive osseous metastases including the left pelvic area, a high degree of PET avidity in the right humerus area, left lower pulmonary nodules, a right common iliac lymph node, and a small hypermetabolic infiltrate in the right middle lobe of the brain ( Figure 1H). Despite the initiation of the third-line chemotherapy with paclitaxel and ramucirumab, patient developed multisystem organ failure as a result of widely metastatic adenocarcinoma and succumbed to his disease, 18 months after initial diagnosis.
DISCUSSIONGastrointestinal malignancies are a rare occurrence in the pediatric population and show a poorer overall survival when compared to the adult population. 1-3 A study conducted in 2015 by Poles et al. demonstrated that as compared to adults, pediatric patients are 8-17 times more likely to have higher proportions of signet ring cell histology. This aggressive form of tumor biology is a significant predicator of mortality and is increasing in prevalence, as reported by Henson et al. 4,5 Our literature review of pediatric SRCA cases in the GI tract revealed 30 cases between 1985 and 2016, many of which underwent aggressive treatment regimens (Supplementary Table S1). The prognosis for pediatric SRCA remains poor with a median survival time of 9 months (ranges from 19 days to 18 months, 20 available outcomes), compared to a median survival time of 9-11 months in the adult population for all esophagogastric cancers. 6 The outcome of SRCA was recently reported by Pernot et al. to be controversial. 7 Our patient achieved 8 months of extended quality of life after crizotinib was selected for palliative treatment, with an overall survival of 18 months.
CONCLUSIONSOur report represents a novel case of utilizing genetic testing in a pediatric patient with SRCA to influence treatment and improve outcome for a patient with a poor prognosis.