Acinar cell carcinoma in childhood: A case report of a very rare tumor
Article Category: Research Article
Published Online: Apr 22, 2021
Page range: 3 - 8
Received: Dec 12, 2019
Accepted: Apr 29, 2020
DOI: https://doi.org/10.2478/fco-2019-0017
Keywords
© 2020 Apostolos Pourtsidis,Smaragda Papachristidou, Orthodoxos Achilleos, Darius Mirza, Marina Servitzoglou, Dimitrios Doganis, Konstantina Kapetaniou, Maria Nikita, Evgenia Magkou, Nikolaos Ptochis, Anastasia Papazoglou, Antonia Moutafi, Georgios Pantalos, Aikaterini Michail, Margarita Baka, published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.
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.