Castleman disease (CD) is a rare but benign condition in which there is lymph node enlargement. It can either be unicentric (single lymph node group involved), which is the more common type or the less common multicentric type
Castleman disease (CD) is a rare but benign condition in which there is lymph node enlargement. It can either be unicentric (single lymph node group involved), which is the more common type or the less common multicentric type
We report a rare case of an incidental pancreatic lesion that proved to be Castleman disease in a peripancreatic lymph node, which mimicked a high-grade pancreatic neuroendocrine tumour (PNET) based on findings on positron emission tomography (PET). The disease was discovered as an incidental finding on CT imaging of the abdomen and was investigated and managed as PNET. Surgical resection was performed with distal pancreatectomy and splenectomy, however, histology revealed the lesion was a lymph node affected by Castleman disease. Often termed the great mimic, Castleman disease is a rare lymphoproliferative disorder that is often mistaken for other primary lesions of the organ due to its location and should be considered a differential of fluorodeoxyglucose (FDG)-avid PET lesions on imaging.
A new focus
In the EU, a disease is defined as rare if it affects no more than 1 in 2,000 people and similarly in the US it is defined as a condition that affects less than 200,000 people.
1 Despite being referred to as ‘rare’, collectively, there are 6,000 to 8,000 known rare diseases
2 and more than 300 million people around the world are living with one.
3 For these people, living with a rare disease can mean a long wait to receive an accurate diagnosis, less options in terms of care, and many unanswered questions. In order to address this current unmet need, we must bring a new focus to the challenges faced by those with rare diseases.