engleski

Intraductal papillary mucinous neoplasm in a patient with Peutz-Jeghers syndrome: a clinical challenge

Intraductal papillary mucinous neoplasms (IPMN) have been recognized as precursor lesions of pancreatic cancer. Hereditary polyposis syndromes present a high-risk feature for developing pancreatic cancer. We report our first case of IPMN in a patient with Peutz-Jeghers syndrome (PJS). A 36-year-old female patient, diagnosed with Peutz-Jeghers syndrome at the age of 12 was referred to our Center in 2016 for further management of incidentally found pancreatic cystic lesion. She has a history of several endoscopic small and large bowel polypectomies and 2 small bowel resections due to volvulus and small bowel perforation. She underwent hysterectomy for cervical adenocarcinoma and cholecystectomy for gall bladder adenocarcinoma. In 2016 a surveillance magnetic resonance findings have revealed 13 mm large semisolid cystic lesion in the pancreatic head, with no contrast enhancement of solid component. Endoscopic ultrasound (EUS) with fine needle aspiration was performed, resulting in nonspecific cytologic findings. Close follow-up was continued in 6-month intervals combining EUS and magnetic resonance cholangiopancreatography. In December 2018 a contrast enhanced EUS has shown a slight increase in the cyst size (17 mm) with solid component showing enhancement after contrast administration and no dilatation of main pancreatic duct (Figure 1, 2). Citology of mural nodule was positive for IPMN with no signs of dysplasia (Figure 3). PET-CT revealed no areas of pathologic fluorodeoxyglucose (FDG) uptake. Our case raises an important question of optimal timing for surgery in high risk individuals (HRI) for developing pancreatic cancer. Current guidelines do not address the problem of specific approach to surveillance and management of IPMN in HRI. Since we are lacking evidence on the frequency and rate at which IPMNs progress to invasive cancer in HRI the decision on further treatment, including assessing the right time for surgery should be made individually and based on multidisciplinary agreement.

intraductal papillary mucinosu neoplasm, IPMN, Peutz-Jeghers syndrome

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