Acinar cell carcinoma in childhood: A case report of a very rare tumor

Introduction Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment. Patient and Methods An 8-year old girl presented with jaundice. Workup revealed a tumor originating from the head of the pancreas with multiple metastatic lesions in her liver. Evaluation of tumor markers revealed elevated levels of AFP. Pathology report was indicative of acinar cell carcinoma of the pancreas. Results After consulting the EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors), chemotherapy was initiated. Partial response was observed after the first 4 courses with decrease of AFP levels. While planning her surgery, AFP elevated and a second-line course of chemotherapy was administered. Our patient underwent Whipple’s Duodenopancreatectomy with partial metastasectomy. Although the postoperative period was uneventful, AFP continued to rise even after postoperative chemotherapy was administered. There were signs of metastatic disease progression. Our patient received a third-line regimen with no improvement. She received local radiotherapy and a next-line chemotherapy course. Local relapse and metastatic disease progression placed our patient in palliative care. She passed away nine months after the initial diagnosis. Conclusions Acinar cell carcinoma of the pancreas is a rare type of pediatric cancer with very challenging diagnosis and treatment. Cooperation at the European level and multicenter management of those rare cases is vital for the optimum outcome.