Castleman tumor, also known as Castleman disease or angiofollicular lymph node hyperplasia, is a rare noncancerous or neoplastic disorder that involves the abnormal growth and expansion of lymphoid tissue. It was first described by pathologist Dr. Benjamin Castleman in 1954. This condition affects the lymph nodes and can manifest as a localized or multicentric disorder.
Castleman tumor is characterized by the proliferation of lymphocytes (white blood cells) within the lymph nodes, leading to the formation of numerous abnormal blood vessels. It is further classified into two major subtypes: unicentric (localized) Castleman disease and multicentric Castleman disease.
Unicentric Castleman disease is more common and usually affects a single lymph node or a group of adjacent lymph nodes. It typically presents as a localized mass that can be easily removed through surgical excision. On the other hand, multicentric Castleman disease affects multiple lymph nodes throughout the body, including those in the chest, abdomen, and neck. This form is associated with systemic symptoms, such as fever, weight loss, fatigue, night sweats, enlarged liver and spleen, and abnormalities in blood cell counts.
The exact cause of Castleman tumor remains unknown. However, it is believed to involve an overactive immune response or an abnormal reaction to certain infections, including human herpesvirus-8 (HHV-8) in the case of multicentric Castleman disease.
Treatment for Castleman tumor depends on the subtype and presentation. Surgical excision is the primary approach for unicentric cases, while systemic therapy with medications, such as corticosteroids, chemotherapy, immunotherapy, or targeted therapies, is typically employed for multicentric Castleman disease. Regular follow-up and lifelong monitoring are often necessary due to
