Endocrine Abstracts

Introduction

Solid pseudopapillary tumors (SPT) of the pancreas area rare exocrine pancreatic tumor behaving in a low-grade fashion, with limited local invasion risk and a rare metastatic evolution. They mainly affect young women. Their etiopathogenesis remains uncertain. We report the case of a 15-year-old boy, having congenital anomalies, who presented a solid and pseudopapillarytumour of the pancreas.

Case report

A fifteen-year old boy with a familial history of diabetes, infertility andepilepsia was known to have a polymalformative syndrome made up of a single left kidney and bone malformations (dorsal scoliosis and a butterfly vertebral defect at the L4 lumbar spinal level). He was admitted in October 2019 on the general surgery department of the UHC Habib BourguibaSfax for acute abdominal pain and an epigastric mass. Biological assessment was correct, whereas MRI showed a 10 cm cystic tumor lesion of the pancreas with a thick wall significantly enhanced especially on late sequences without obvious invasion of neighboring structures suggesting SPT of the pancreas. A caudal spleno-pancreatectomy with a high segmental resection removing the tumor as a single piecewas then done. The anatomopathological examination confirmed the diagnosis of SPT without lymph node metastases or local invasion. A prediabetes was revealed after surgery with a fasting plasma glucose (FPG) level of 5.8 mmol/l and a glycated hemoglobin(A1C) level of 6.17%. One year later, the patient was admitted in the endocrinology department of the UHC HediChakerSfax for an inaugural ketosis-prone diabetes. He reported an important weight loss in the last two months and a flu a week before his admission. The rest of the somatic exam was normal as well as the biological assessment except an A1clevel at 16.9%.An abdominal CT scan revealed a remaining pancreas (cephalic) of homogeneous density and enhancement. The pancreatic and thyroid antibodies were negative.A detailed physical examination, ophthalmologic examination and cardiac ultrasound did not find other associated malformations. Although a diabetes secondary to pancreatectomy seemed the most probable, we completed with a DNA sample in search of monogenic diabetes.

Discussion-Conclusion

TPS is extremely rare in men. Also, the association of TPS with a single congenital kidney or bone malformations had not been reported in the literature to our knowledge.