title: Multifocal intraductal tubulopapillary neoplasm of the pancreas with total pancreatectomy: report of a case and review of literature.
abstract: Intraductal neoplasms of the pancreas are classified as intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasm (IPMNs) in the current WHO classification. ITPN is a rare tumor and there are only a few cases of ITPN reported in the literature. We present the case of an otherwise healthy 42-year-old male, who presented with upper abdominal pain. He was subsequently diagnosed with multifocal ITPN and underwent total pancreatectomy. The pathological report showed invasive growth. The postoperative course was uneventful and the patient received 6 months of adjuvant chemotherapy with gemcitabine-capecitabine. The patient is still alive 19 months after the procedure with no signs of recurrence. Literature review revealed only 30 individual cases of ITPN in the pancreas including our reported case. Mean age was 61 years (16 males/14 females; ratio 1.14:1). Mean tumor size was 3 cm. Immunohistochemical staining was positive for CK-7 in 100% of the patients, CK-19 in 95% and for MUC-1 in 88%. Trypsin was negative in all cases. β-catenin was negative in 94% and MUC-2 was negative in 96% of the cases. BRAF, KRAS, TP53 and PIK3CA mutations were infrequently seen. Invasive growth was present in 54% of the cases. Tumor size and Ki-67 index showed a statistically significant association with invasive growth. Survival rate could not be determined, due to short follow-up, and further research is needed to establish prognostic factors for disease recurrence and survival.
authors.affiliation: Department of Surgery, Clinical Sciences Lund, Lund University, Skåne University Hospital Lund, Sweden.
authors.forename: David
authors.initials: D
authors.lastname: Kölby
authors.affiliation: Department of Surgery, Clinical Sciences Lund, Lund University, Skåne University Hospital Lund, Sweden.
authors.forename: Johan
authors.initials: J
authors.lastname: Thilén
authors.affiliation: Department of Surgery, Clinical Sciences Lund, Lund University, Skåne University Hospital Lund, Sweden.
authors.forename: Roland
authors.initials: R
authors.lastname: Andersson
authors.affiliation: Department of Pathology, Clinical Sciences Lund, Lund University, Skåne University Hospital Lund, Sweden.
authors.forename: Agata
authors.initials: A
authors.lastname: Sasor
authors.affiliation: Department of Surgery, Clinical Sciences Lund, Lund University, Skåne University Hospital Lund, Sweden.
authors.forename: Daniel
authors.initials: D
authors.lastname: Ansari
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000069287:Capecitabine / Q000008:administration & dosage; D002291:Carcinoma, Papillary / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D006801:Humans; D008297:Male; D010180:Pancreatectomy; D010183:Pancreatic Ducts / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D000093542:Gemcitabine
chemical_list: D003841:Deoxycytidine; D000069287:Capecitabine; D000093542:Gemcitabine
keywords: Intraductal tubulopapillary neoplasm; invasive growth; pancreas