Lymphoproliferative disorder characterized by large,
benign, hyperplastic lymph nodes (localized form, pauci-symptomatic, and
Castleman Tumor; Angiofollicular Lymph Node Hyperplasia;
Angiomatous Lymphoid Hamartoma; Lymphatic Hamartoma; Giant Lymph Node
Hyperplasia; Giant Benign Lymphoma.
Named after B. Castleman, an American pathologist, who
first described this condition in 1956.
Rare. Localized form is more frequent than the
Three histologic variants (plasma cell,
hyaline-vascular and mixed) and two clinical variants (localized and multicentric)
exist. The hyaline-vascular variant accounts for approximately 90% of the
cases. The localized form is more common than the multicentric form.
The etiology of this disease has not been fully
determined. Current knowledge suggests the multicentric plasma cell variant
is highly associated with an infection by human herpes virus 8 (HHV-8). In
addition, these patients also seem to have an increased risk of developing
other HHV-8-associated malignancies, such as (extranodal) B-cell non-Hodgkin
lymphomas, multiple myeloma, and Kaposi sarcoma. The localized plasma cell
and the mixed variant of Castleman disease are frequently found in patients
who have a history of lymphoma. Interleukin-6 overproduction, either
endogenously or virally encoded, has been made responsible for uncontrolled
plasma cell proliferation in these two variants.
Based on the characteristic lymph node
histology and exclusion of other disorders resulting in lymph node swelling.
The localized form is often asymptomatic or becomes symptomatic once the diameter of the
lymph node is significant enough to cause local compression of important
structures, such as airway, vessels, or nerves (resulting in pain). The
multicentric form is always symptomatic because of high production of interleukin-6.
The localized variant of Castleman disease presents as a mass
lesion. The mediastinum, abdomen, and neck are the sites most commonly
affected. Lesions up to 16 cm in diameter have been reported. Constitutional
symptoms are most commonly seen in the plasma cell and multicentric forms.
Symptoms include asthenia, weight loss, fever, skin rash, hemolytic anemia,
and/or hypergammaglobulinemia. Hepatomegaly and/or splenomegaly are present
in approximately 75% of cases. In approximately 25% of
patients, the disease may also present with features of PEP (plasma cell
dyscrasia, endocrinopathy, polyneuropathy) syndrome. Amyloidosis has been
described in a number of cases. Patients with multicentric disease are prone to infections and
malignancies. Treatment is surgical excision where possible (localized
variant). Surgery, steroids, and chemotherapy are mainly used for the
multicentric variant, which has a poor prognosis. Paraneoplastic pemphigus vulgaris has been described in some patients suffering from Castleman disease. The disease in the
pediatric population appears to have a more favorable course than in adults.
Preoperative assessment should
attempt to determine the direct and systemic effects of the lesion(s).
Assess mediastinal or thoracic masses (chest radiographs, computed
tomography scanning, diagnostic bronchoscopy), and ask about positional
stridor and dyspnea to determine the degree of tracheobronchial compression
and cardiovascular involvement. Pulmonary function tests can be useful for
adults, but are less ...