Castleman's disease in ear, nose, and throat practice

Castleman's disease, also called angiofollicular lymph node hyperplasia or benign giant lymphoma, is a rare lymphoproliferative disorder of unknown etiology. Three histologic subtypes are described--hyaline vascular (80-90%), plasma cell (10-20%), and mixed (rare). In the clinical setting, localized and multicentric entities are distinguished. Due to the lack of tumor-specific clinical, biochemical, and radiological features, final diagnosis of Castleman's disease depends on histopathology. Surgical tumor resection is the treatment of choice for localized disease. Prognosis is good, and adjuvant therapy is not required. Therefore, early invasive removal and histopathological differentiation from neoplasia is mandatory. In contrast, the prognosis for multicentric Castleman's disease remains poor even if multimodal treatment regimens are employed. Major clinical symptoms, histology, and therapy are summarized, and the disease characteristics are highlighted presenting the case of an 11-year-old girl. On admission, the patient complained of a painless pharyngeal tumor mass and ipsilateral lymph node swelling. Magnetic resonance imaging revealed a parapharyngeal contrast-enhancing lesion extending from the hypopharynx to the skull base without signs of infiltration and accompanied by ipsilateral lymph node hyperplasia of the neck. Laboratory test results were within normal limits. After tumor resection, histopathological examination, and clinical staging, localized Castleman's disease was diagnosed (hyaline vascular subtype).