Two cases of agenesis of the dorsal pancreas and a review of the literature

Abstract

Background Agenesis of the dorsal pancreas (ADP) is a very rare disease with no specific symptoms, and the pathogenesis is not clear. Some patients will be accompanied by other diseases, such as pancreatic tumor or pancreatitis. But most cases are very atypical and difficult to distinguish. Some syndromes of pancreatic exocrine insufficiency are common in patients with ADP. Here, we report two cases of ADP and summarize the clinical features, diagnosis, and treatment of ADP. Case presentation Case A is a 65-year-old Chinese woman who presented with abdominal pain accompanied by nausea, bloating and acid reflux. The enhanced abdominal CT scan found nothing meaningful except the absence of the body and tail of the pancreas. The diagnosis was considered as gastrointestinal dysfunction cause by exocrine pancreatic insufficiency and recovered after symptomatic treatment. Case B is a 61-year-old Chinese woman who presented with abdominal pain accompanied by fever, vomiting and bloating. The abdominal CT showed multiple stones in the gallbladder, and the body and tail of the patient’s pancreas were absent. She was diagnosed with cholelithiasis and recovered after laparoscopic cholecystectomy. Conclusion Agenesis of the dorsal pancreas (ADP) is a rare congenital disease with an unclear pathogenesis that presents multiple symptoms. It should be considered when the patients have non-specific, persistent and unexplained symptoms such as bloating or uncontrolled blood sugar. Imaging examination is helpful for diagnosis. And it does not require surgical intervention unless it accompanies other diseases, EPI need to be considered when the non-specific gastrointestinal symptoms appear.

Genetically engineered pigs manifesting pancreatic agenesis with severe diabetes

“…At present, the pathogenesis of this disease is not fully understood. 39 In our Tg model with genetically engineered pigs, the symptoms emerged drastically, but the dorsal lobe was present and the exocrine pancreas was normal. Thus, the pattern for emergence of pancreatic agenesis was different from humans.…”

Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

“…Herein, we describe another three cases, making a total of ve reported thus far. More than ten ADP cases have been associated with pancreatic neoplasia, although only about 100 occurrences of ADP are recorded in the literature [1][2][3][4][5][6]. The mechanism of tumorigenesis in ADP patients is unclear, but chronic pancreatitis caused by the ADP itself could be one of the risk factors for neoplasia [2].…”

“…This condition is characterized by the partial or total loss of the body and tail of the pancreas. Most patients with this anomaly are asymptomatic but may develop diabetes mellitus, pancreatitis, and other pancreatic diseases such as pancreatic tumors [1][2][3][4][5][6]. ADP is compatible with life and requires no special treatment; however, it is sometimes necessary to treat any accompanying diseases [2].…”

“…Most patients with this anomaly are asymptomatic but may develop diabetes mellitus, pancreatitis, and other pancreatic diseases such as pancreatic tumors [1][2][3][4][5][6]. ADP is compatible with life and requires no special treatment; however, it is sometimes necessary to treat any accompanying diseases [2]. Such a disease includes the solid pseudopapillary tumor (SPT) of the pancreas, which is a rare low-grade malignant neoplasm.…”

Partial agenesis of dorsal pancreas. Report of two cases

“…A very limited number of pancreatic tumors are reported in association with agenesis of the dorsal pancreas, including a malignant IPMN [ 22 ] and cystic lesions [ 23 ]. The association is unclear, but chronic pancreatitis is considered one of the risk factors for cancer.…”