25 April 2025
: Case report 
Agenesis of the Dorsal Pancreas and Its Possible Link to Pancreatic Neuroendocrine Tumors: Case Insights
Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)
Alessia Fassari
1AEF*, Edoardo Rosso 2ABE, Jyoti Lakshmi Anafack1DE, Vito De Blasi 1ADEFG
DOI: 10.12659/AJCR.947512
Am J Case Rep 2025; 26:e947512
Table 1. Studies reporting the association between agenesis of dorsal pancreas and neoplastic lesions of the pancreas or distal structures. NA, not available.
| Study | Tumor localization | Tumor histology | Treatment |
|---|---|---|---|
| Ulusan et al 2006 []6 | Pancreatic head + liver metastases | Adenocarcinoma | Laparotomic cholecystectomy and hepaticojejunostomy |
| Kapoor et al 2011 []7 | Periampullary | Adenocarcinoma | Pylorus preserving pancreaticoduodenectomy |
| Dumitraşcu et al 2012 []9 | Pancreatic head | Well-differentiated adenocarcinoma | Pancreatoduodenectomy |
| Mistry et al 2014 []10 | Ampulla | Adenocarcinoma | Resection and reconstruction by gastrojejunostomy and hepaticojejunostomy, without pancreatoenteric anastomosis |
| Sannappa et al 2014 []11 | Periampullary | Invasive poorly differentiated adenocarcinoma | Total pancreatectomy, duodenectomy, Roux-en-Y gastrojejunostomy, hepaticojejunostomy, and cholecystectomy |
| Deignan et al 1996 []12 | Rectum | Adenocarcinoma | NA |
| Rittenhouse et al 2011 []30 | Pancreatic head | Moderately differentiated ductal adenocarcinoma | Total pancreatectomy |
| Rittenhouse et al 2011 []30 | Pancreatic head | Moderately differentiated ductal adenocarcinoma | Total pancreatectomy |
| Rittenhouse et al 2011 []30 | Pancreatic head | Moderately differentiated ductal adenocarcinoma | Total pancreatectomy |
| Ulusan et al 2005 []31 | Pancreatic head | Solid, cystic and pseudopapillary tumor | Whipple’s procedure |
| Rodrigues et al 2017 []32 | Pancreatic head | Neuroendocrine low-grade G1 (well-differentiated) tumor | Cephalic duodenopancreatectomy |
| Saikaly et al 2017 []33 | Pancreatic head | Mucinous adenocarcinoma and cystic teratoma | Laparotomic Whipple procedure with total pancreatectomy |
| Erotokritou et al 2018 []34 | Between pancreatic head and neck | Neuroendocrine low-grade G1 (well-differentiated) tumor | pancreatic resection |
| Nakamura et al 2001[]35 | Pancreatic head | Solid papillary | Laparotomic partial resection of the pancreas including the tumor |
| Nonent et al 2003 []36 | Diffuse peritoneal carcinosis predominating on the transverse mesocolon | Metastatic papillary adenocarcinoma of ovarian or peritoneal origin | Debulking |
| Otani et al 2004 []37 | Bile duct | Schwannoma, Antoni type A | Surgical resection of the bile duct schwannoma in patient already treated by end-to-side choledochojejunostomy with Roux-en-Y technique and cholecystectomy |
| Sakpal et al 2009 []38 | l of the pancreas prolapsed through the ampulla of Vater into the duodenum | IPMN | Total pancreatectomy, duodenectomy, Roux-en-Y gastrojejunostomy, hepaticojejunostomy, and cholecystectomy |
| Namikawa et al 2023 []39 | Stomach | Poorly differentiated adenocarcinoma invading the serosa, with 5 lymph node metastases | Laparoscopic total gastrectomy with lymph node dissection |
| Shyr et al 2022 []40 | Pancreatic head | Pseudopapillary solid tumor | Pancreatic head sparing enucleation |
| Shyr et al 2022 []40 | Pancreatic head | Pseudopapillary solid tumor | Pancreatic head sparing enucleation |
| Shyr et al 2022 []40 | Pancreatic head | Pseudopapillary solid tumor | Pancreatic head sparing enucleation |
| Pasaoglu et al 2008 []41 | Stomach | Lymphoma | NA |
| Jha et al 2023 []42 | Breast + liver metastases | NA | NA |
| Julianov et al 2020 []43 | Pancreatic head | Pancreatic ductal adenocarcinoma | Laparotomic total pancreatectomy |
| Kabnurkar et al 2017 []44 | Tongue | NA | Chemo/radiotherapy |
| Zhou et al 2014 []45 | Lung | Poorly differentiated pulmonary adenocarcinoma | NA |
| Montero-Macìas et al 2023 []46 | Endometrium + peritoneal carcinosis | Serous adenocarcinoma of the endometrium | Neoadjuvant chemotherapy – debulking – complete cytoreduction – chemotherapy |
| Nassif et al 2016 []47 | Neck of the pancreas | Neuroendocrine low-grade G1 (well-differentiated) tumor | Resection of pancreatic tumor via spleen preserving laparoscopic approach and with uncinate process and pancreatic head preservation |
| Elhattabi et al 2020 []48 | Stomach | Invasive adenocarcinoma | Total gastrectomy with Roux -en-Y-esophagojejunostomy reconstruction |
| Present case | Pancreatic head + liver metastases | Neuroendocrine high-grade G3 (poorly differentiated) carcinoma | Percutaneous transhepatic biliary drainage |