Introduction

Pancreatoblastoma (PB) is a rare form of pancreatic tumor, accounting for < 1 % with an annual incidence rate of approximately 0.004 per 100,000 individuals.[1] It is often misdiagnosed as a neuroblastoma.[2] In the year 2014, the Papanicolaou Society of Cytopathology (PSC) officially recognised the name "Pancreatoblastoma" for this particular type of tumor. PB is found to occur more frequently among Asian individuals compared to Caucasian people.[3] It is more aggressive in adults compared to pediatric cases and bears a poor prognosis.[4] Because of the lack of specific symptoms, it often leads to a delay in diagnosis.[5] PB is inconsistently documented in the medical literature, with case reports being the most common and a comprehensive systematic analysis is required.[6] In this case report, we described the clinical characteristics, pathologic features and treatment of pancreatoblastoma in a 55-year-old female patient.

Case Presentation

A 55-year-old female presented with complaints of vomiting and abdominal pain radiating posteriorly. She had experienced a similar episode 4 months ago, which was relieved with medication. The patient was admitted to our department for further management. There was no family history of comorbidities or any malignancies.

On physical examination, the baseline vitals were stable. Baseline investigations were done; all were within normal limits, but her serum amylase (383 U/L) and lipase levels (1634 U/L) were found elevated.

Contrast-enhanced computed tomography (CECT) abdomen revealed a large exophytic well well-marginated mass lesion arising from the inferior aspect of the head of the pancreas in the periampullary region and pancreaticoduodenal groove. The lesion measures 6.5 x 9.3 x 8.5 cm (Figure 1). Mild post-contrast enhancement, which is slightly hypoenhancing to the pancreas, is seen with multiple eccentric non-enhancement areas. The fat plane with the corresponding, surrounding structure was well maintained. The distal common bile duct and the main pancreatic duct are severely compressed by the mass lesion, with upstream dilation of the main pancreatic duct.

Discussion

Adult pancreatoblastoma (PB) is a rare malignant tumor of the pancreas, difficult to diagnose because of its resemblance to other solid cellular neoplasms of the pancreas, like solid pseudopapillary neoplasms, neuroendocrine neoplasms and pancreatic acinar cell carcinomas.[7] PB is characterised by a slow growth rate, soft and well-circumscribed nature, often reaching significant sizes upon diagnosis. Approximately half of these tumours are located within the pancreatic head region.[8]

In PB, abdominal pain, weight loss and abdominal mass are the main symptoms in adult patients, whereas anorexia, bowel habit changes and jaundice are also some common symptoms.[9] Our patient had abdominal pain and vomiting, which aligns with the findings of previous research. PB predominantly exhibits a preference for the head of the pancreas in approximately 50% of cases. These tumours range in size from 1.5 to 20 cm, demonstrating significant variability. A conclusive diagnosis of PB should invariably be established following a comprehensive histological examination.[10] The imaging characteristics of PB are characterised by their non-specific nature, with the majority of tumours presenting as lobulated masses which are typically well-circumscribed or partially circumscribed and accompanied by necrosis and enhancing septations on CECT.[11] Our case was greatly aided by CECT, which identified the precise lesion location.

In our case, amylase and lipase levels were elevated. Comparable tissue destruction in pancreatic cancer could lead to variations in peripheral amylase and lipase concentrations, which could be explained by tumour infiltration of pancreatic tissue.[12] The definitive treatment approach for pancreatic cancer involves complete surgical resection (RO). Our patient underwent a Whipple procedure, during which the entire affected area was completely resected. The degree of surgical resection required to ensure comprehensive debulking is assessed based on the position of the tumour within the body.[13] According to Schmidt et al., a considerable number of consecutive Whipple procedures have been performed without any fatalities in the last decade.[14]