Case Of Hepatic Castleman'S Disease Presenting As A Malignancy: Challenging Diagnosis And Literature Review

Castleman's disease (CD) is a very rare lymphoproliferative disorder, first reported as a form of localized mediastinal lymphadenopathy. The pathogenesis of CD has not been fully elucidated, however, a defect in immune regulation has been suggested to cause excessive proliferation of lymphoplasma cells. To the best of our knowledge, 14 cases of hepatic CDs have been reported. The current study presents a rare case of localized hepatic CD with a review of the related literature. A 60-year-old asymptomatic woman underwent partial hepatectomy due to a hepatic mass incidentally found and presumed to be malignant. Grossly, the liver showed a well-defined pinkish yellow mass of 3.8 x 3.5 cm in size in segment 6/7. Microscopically, it was composed of follicular hyperplasia with extensive inter-follicular hyalinized vessels and numerous plasma cells, suggesting CD of the mixed hyaline vascular and plasma cell type. Based on this case and a review of the literature, preoperative diagnosis of CD at unusual sites is challenging and sometimes inaccurate. After undergoing surgical excision only, the patient was followed up over a nine-year period with no recurrence or progression to the more aggressive form.