Localized pancreatic Castleman disease presenting with extrahepatic dilatation of bile ducts: A case report and review of published cases

Abstract Introduction: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unknown etiology, which usually develops in the mediastinum. It can also occur in the cervical, retroperitoneal and axillary regions. Localized pancreatic CD is quite rare [ 1 ]. Presentation of case: The authors herein present a case of a 34 years old female that was diagnosed during a symptomatic cholelithiasis evaluation. During the evaluation, an abdominal ultrasonography revealed a tumor at the head of the pancreas, which went on to generate a dilatation of the extrahepatic bile ducts. This finding was confirmed by abdominal magnetic resonance imaging (MRI). Subsequently, the patient underwent a laparotomy, where a capsulated tumor was found at the head of the pancreas with well-defined margins. The decision was made for tumor excision. The histopathology and immunohistochemistry established CD, hyaline vascular variation. Discussion: The authors of the present paper also performed a literature review concerning Pancreatic CD, where there were found only 33 cases until the time of the writing of this paper, and we have subsequently carried out a retrospective analysis of all cases. In a patient with atypical images, there might be a benefit from a preoperative diagnosis of CD, by using immunohistochemistry analysis in an image guided biopsy. Thus, avoiding unnecessary procedures and surgeries. Conclusion: Localized pancreatic CD is a very rare condition with good prognosis, but it can mimic many common diseases, such as gastrointestinal stromal tumor (GIST), pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.