Acinar cystic transformation of the pancreas: A rare case report.

Abstract

Background: Acinar cystic transformation (ACT) of the pancreas is a rare non-neoplastic transformation of the pancreas. Adult women are the majority of patients with ACT, but few cases have been reported in pediatric patients. Given that there are currently no guidelines for the treatment of ACT, current treatment is based primarily on expert opinions and clinical experiences. Here, we report the case of the youngest child with ACT to date. Additionally, a literature review on pediatric ACT cases was performed to summarize previous clinical experience and treatment methods.

Case summary: A 1-year-old Chinese girl presented with progressive abdominal distension for 6 months. A detailed consultation revealed an uneventful history. The patient showed no signs of fever or abdominal pain and had a good appetite and normal feces. A mass of about 20 cm × 10 cm × 10 cm in size was detected in the abdomen. Both abdominal ultrasound and computed tomography examination revealed a multilocular cystic mass about 21.7 cm × 16.8 cm × 8.9 cm in size. At first, due to the large size and the possible retroperitoneal origin of the cyst, a total resection of the lesion was not possible. A single-port laparoscopic lymphangioma puncture and Pingyangmycin injection were performed in March 2023. One month after surgery, the abdominal cyst rapidly enlarged to its pre-operative size. After consulting with the experts in the angiology department and interventional department, sclerotherapy combined with oral sirolimus was performed in May 2023. After confirming that the tumor was not sensitive to sclerotherapy combined with oral sirolimus, our surgical team performed tumor reduction in August 2023. This surgery confirmed that the polycystic mass originated from the head of the pancreas, and pathological and immunohistochemical findings diagnosed pancreatic ACT. The patient showed no signs of cyst lesions after 6 months of follow-up and remains in good health up to the time of this report.

Conclusion: ACT is a rare non-neoplastic transformation of the pancreas, more rarely seen in children. Manifestation and examinations show no specificity for diagnosis, and final diagnosis is mainly based on histological findings. To reach a specific diagnosis and rule out malignancy is a priority in clinical practice, and repeated biopsy or radical surgery should be considered before malignancy is ruled out. However, once a diagnosis of ACT is made, a conservative treatment with consecutive follow-up is recommended until symptoms present or obvious enlargement occurs because ACT is considered a slow-growing and benign tumor.