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Clinical Problem Solving: Pathology
September 2001

Diagnosis Pathology Quiz Case

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2001;127(9):1134-1135. doi:

Castleman's disease, which is an uncommon condition of unknown etiology characterized by massive growth of lymphoid tissue, was first described as a pathologic entity by Benjamin Castleman in1954 and later defined in 1956.1 This entity has since been referred to as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, angiomatous lymphoid hamartoma, follicular lymphoreticuloma, and benign giant lymphoma. Flendrig2 first distinguished 2 histological types, which Keller et al3 later designated the hyaline vascular type, representing 90% of cases, and the less common plasma cell type. Although CD usually presents as an indolent disease confined to a single lymph node group (unicentric CD), a more generalized lymphadenopathy, accompanied by systemic symptoms, laboratory abnormalities, hepatosplenomegaly, and a progressive clinical course with the potential for malignancy, has been elucidated (multicentric CD). The condition predominantly affects young adults aged 15 to 30 years, with an equal sex prevalence, although the range of age is broad. In fact, CD has been reported in a neonate.4 It most commonly presents as an incidental solitary mediastinal mass on a radiograph, but it can occur in many extrathoracic sites, including the neck, abdomen, retroperitoneum, pelvis, and axilla.3,5 In the head and neck, lesions are often closely associated with the sternocleidomastoid muscle, although involvement of the larynx, submandibular region, parapharyngeal region, and retropharynx has been reported.6,7

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