Unicentric Castleman’s disease presenting as retroperitoneum lump.

Castleman disease is a rare lymphoproliferative disorder, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia. Clinically, Castleman disease is of two types: localised/unicentric type and multicentric/systemic type. Unicentric or localised Castleman disease affect a single lymph node or group of lymph nodes. The multicentric type affects two or more groups of lymph node in different part of the body. It can also affect organs containing lymphoid tissue. Histologically it is classified as hyaline vascular variant, plasma cell variant and a mixed variant. Clinical symptoms may vary from asymptomatic to symptomatic lymphadenopathy accompanied by fever, anaemia fatigue, abdominal or thoracic pain and weight loss. There is no specific test to diagnose Castleman disease. We report a case of 16 years old male who presented with a painless lump in left lumbar region without any constitutional symptoms. CECT suggested a retroperitoneal lump with calcification. Laparotomy was done and complete excision of mass done. Histopathological examination of excised tissue suggested Castleman disease of hyaline vascular variant. After six month of follow up, the patient has no complain.

Castleman disease

Castleman and Towne described a disease presenting as a mediastinal mass resembling thymoma. It is also known as "giant lymph node hyperplasia", "lymph node hamartoma", "angiofollicular mediastinal lymph node hyperplasia", and "angiomatous lymphoid hyperplasia". The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue. In addition to the mediastinal presentation, extrathoracic involvement in the neck, axilla, mesentery, pelvis, pancreas, adrenal gland, and retroperitoneum also have been described. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular variant, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell variant, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergamma-globulinemia. Castleman disease is a rare lymphoproliferative disorders. Few cases have been described world widely. In this article we reviewed the classification, pathogenesis, pathology, radiological features and up to date treatment with special emphasis on the role of viral stimulation, recent therapeutic modalities and the HIV-associated disease.