Castleman’s disease is a rare disorder that involves an overgrowth of cells in your body’s lymph nodes (small oval-shaped organs that contain immune cells to attack foreign cells like viruses). The most common form of this disorder affects a single lymph node (unicentric Castleman’s disease), usually in the chest or abdomen. But the topic of our discussion is of a more rare form of this already recherché disorder which occurs in the head of the pancreas.
General information about the disease
The cause of Castleman’s disease is not clear but researchers suspect that Multicentric Castleman’s disease (affects multiple lymph nodes) can be caused by an infection from a virus called human herpesvirus 8 (HHV-8). This virus has also been linked to HIV/AIDS. (a sexually transmitted disease). Studies report that HHV-8 is present in nearly all HIV-Positive people with Castleman’s disease and in about half of HIV-Negative people with Castleman’s disease. Castleman’s disease affects more men than women and affects people in their 50s or 60s more. Common symptoms include fever, fatigue, night sweats, nausea, unintended weight loss and enlarged spleen, liver and lymph nodes.
Case study - A rare specimen
This case is of a 58-year old African American male with an incidentally discovered lesion in the head of the pancreas. His studies exhibited an encapsulated mass with features similar to localised/unicentric Castleman’s disease. To date, only eight cases of Castleman’s disease related to the pancreas have been reported in the literature.
The patient had been diagnosed with hepatitis C due to experiencing mild intermittent upper abdominal pain consistent with a history of gastroesophageal reflux disease. After further tests and studies like a PCR analysis and CT scan, a lesion within the head of his pancreas was found and was first considered to be pancreatic carcinoma (cancer that forms in the cells of pancreas). Further morphologic and immunohistochemical findings proved to be the most consistent with a diagnosis of Castleman’s disease.
Castleman’s disease infiltrates normal pancreatic tissue. (black arrow)
Conclusion
Castleman’s disease is a poorly misunderstood disease that provides a source of dilemma for surgeons and doctors. The best chance of a cure is the surgical resection of the unicentric disease. Radiation therapy has also been used for treatment for unresectable lesions. Options other than surgery include systemic therapy in the form of steroids or multiple drug chemotherapies. A better understanding of the pathology of this disease can pave the path for new treatments and an improvement on the current modalities available for treatment.
Written by: Svasti Tewari
Edited by: Sakshi Deshpande
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