Total Pancreatectomy with Splenectomy for Multifocal Intraductal Tubulopapillary Neoplasm (ITPN) of the Pancreas Associated with Invasive Component: Report of a Rare Case

Patient: Male, 82-year-old Final Diagnosis: Pancreatic intraductal tubulopappilary neoplasm (ITPN) Symptoms: Abdominal pain • anorexia • weakness • weight loss Medication: Amlodipine 5 mg/valsartan 160 mg • nebivolol 5 mg • metformin 850 mg • pancreatin 25 000 IU Clinical Procedure: Total pancreatectomy with splenectomy Specialty: Oncology • Pathology • Radiology • Surgery OBJECTIVE: Rare disease BACKGROUND: Pancreatic intraductal tubulopapillary neoplasm (ITPN) was first described by Yamaguchi in 2009 and was recognized by World Health Organization as a distinct entity in 2010. Since then few case reports and case series have been published. Little is known about its clinicopathologic features and treatment outcomes. We present the seventh case of total pancreatectomy for ITPN reported in the English literature. CASE REPORT: Our patient was an 82-year-old male with a previous history of acute evolving-to-chronic pancreatitis. After 2 years of medical consultation, an abdominal magnetic resonance imaging was suspicious for multifocal pancreatic neoplasia. A computed tomography-guided biopsy of the lesion was performed which indicated pancreatic intraductal neoplasia with intermediate dysplasia. After oncology consultation, the patient underwent pylorus-preserving total pancreatectomy with splenectomy. The pathology report showed pancreatic ITPN with intermediate to severe dysplasia and associated invasive carcinoma. All 21 resected lymph nodes were nonmetastatic (pT3N0). The postoperative course of the patient was uncomplicated. He received adjuvant gemcitabine (single agent) for 6 months. At 18 months after surgery he was diagnosed with hepatic metastases; he was still alive at the time of this reporting. CONCLUSIONS: ITPN has been associated with previous history of acute pancreatitis in some patients. Early diagnosis, radical surgical resection, and adjuvant chemotherapy may lead to long-term survival rates even in cases with associated invasive component. Total pancreatectomy may be a preferable procedure for ITPN in selected patients.