Castleman's Disease of the Neck: Report of 4 Cases With Unusual Presentations

Abstract

In 1956, Castleman and colleagues first described the clinicopathologic entity of mediastinal lymphnoid hyperplasia with hyalinization of follicles and interfollicular vascular formation.1 Subsequent reports described additional sites of disease and different names were used for this entity, including Castleman’s disease (CD), angiofollicular or giant lymph node hyperplasia.2 CD represents a morphologically distinct form of lymph node hyperplasia rather than a neoplasm or hamartoma. The etiology and pathogenesis of CD are not completely understood. Microscopically, two major categories have been described.3 The first, designated as hyaline vascular type shows large follicles scattered in a mass of lymphoid tissue. The follicles show marked vascular proliferation and hyalinization of their abnormal germinal centers. Many of large cells with vesicular nuclei present in the hyaline center are follicular dendritic cells. There is a tight concentric layering of lymphocytes at the periphery of the follicles (corresponding to the mantle zone), resulting in an onion-skin appearance. The second major morphologic category of CD is known as the plasma cell type. It is characterized by a diffuse plasma cell proliferation in the interfollicular tissue. The hyaline vascular changes in the follicles are inconspicuous or absent. CD is clinically heterogeneous with either solitary CD (SCD) or multicentric CD (MCD). Over 90% of the cases are of the hyaline vascular type, and the remainder are of the plasma cell type.3 The former usually presents with asymptomatic solitary mass and can mostly be treated effectively with surgery,4–6 whereas the plasma cell type often has a more aggressive course and tends to be MCD with systemic sympotoms (including fever, night sweats, weight loss, and recurring infections) and multiple peripheral lymphadenopathies.7, 8 CD with severe systemic manifestations and poor prognosis is frequently associated with POEMS syndrome (Polyneuropathy, Organomegally, Endocrinopathy, M protein, and Skin changes), renal or pulmonary complications and malignancies, such as Kaposi’s sarcoma, non-Hodgkin’s lymphoma, Hodgkin’s lymphoma, and follicular dendritic cell sarcoma.8–13 CD may occur anywhere along the lymphatic chain, but the mediastinum is the most common location. Neck involvement with CD is relatively uncommon. To our knowledge, most descriptions of CD in the neck were isolated cases or small series and most of the previously reported cases of CD of the neck were of the hyaline vascular type. 4–6 In this article, we describe four cases of CD in the neck with unusual presentations, different histopathologic subtypes and discuss the problems arising in their clinical findings.