Purpose-Most of the previously reported cases of Castleman's disease (CD) in the neck were of the hyaline vascular type and the most common sign was an asymptomatic neck mass. To achieve a more general recognition of CD in the neck, we present four cases of CD in the neck with unusual presentations.Patients and Methods-We describe four cases of CD in the neck with unusual clinical manifestations, different histopathologic subtypes and discuss the problems arising in their clinical findings.Results-Two cases were plasma cell type with multiple bilateral enlarged lymph nodes and systemic manifestations. Two cases presented as a solitary mass were hyaline vascular type. One case underwent twice local recurrence and evolved from the hyaline vascular CD to follicular dendritic cell sarcoma.Conclusions-Cervical plasma cell CD may have a tendency to present as multiple masses on the both sides with systemic manifestations. It is necessary to pay more attention on the overgrowth of follicular dendritic cells in the hyaline vascular CD, which might represent a precursor of follicular dendritic cell sarcoma.
KeywordsCastleman's disease; Neck; POEMS syndrome; Follicular dendritic cell sarcoma; Clinical manifestation 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 Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. 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 sy...