Castleman’s disease (CD) is a rare, benign lymphoproliferative disorder. The association of human herpes virus 8 (HHV8) and human immunodeficiency virus infections with CD is well established however the role of Epstein-Barr Virus in CD is less well understood. We present a unique case of Castleman’s disease in a patient with concomitant EBV infection, which mimicked the clinical presentation of Nasopharyngeal Carcinoma (NPC) versus lymphoma. After a delayed diagnosis, the patient underwent a left superficial parotidectomy and neck dissection and has had no recurrence of disease.
Castleman’s disease (CD) is a rare, benign lymphoproliferative disorder affecting the head and neck region second only to the mediastinum . The presentation is divided clinically into unicentric or multicentic disease and pathologically may manifest as hyaline-vascular or plasma cell subtypes. The association of human herpes virus 8 (HHV8) and human immunodeficiency virus (HIV) infections with CD are well established. The role of Epstein-Barr virus (EBV) in CD is less well understood. We present a unique case of CD in a patient with concomitant EBV infection, which mimicked the clinical presentation of nasopharyngeal carcinoma (NPC) versus lymphoma.
A 68-year-old male from Southern China presented for evaluation of a painless left level IIa neck mass that was present for 1 year. Biopsy demonstrated reactive changes with follicular hyperplasia. Two years later, the patient returned with multiple enlarged cervical nodes in levels IIa, IIb, and V. Endoscopy and repeat lymph node biopsy again revealed only reactive changes and follicular hyperplasia without evidence of malignancy or lymphoma.
Three years later, the patient presented with enlarging diffuse left neck and parotid lymphadenopathy ( Fig. 1 A and B ) with multiple inconclusive repeat FNAs. Hematology-oncology and infectious disease consultation were unrevealing and considered more aggressive surgical management necessary to assist in diagnosis. Radiographically, CT scan showed interval increase in the size and number of cervical adenopathy suggestive of metastatic disease ( Fig. 1 A and B). The patient consequently underwent a left superficial parotidectomy and neck dissection.
Surgical histopathology displayed reactive lymphoid tissue with a morphologic pattern consistent with hyaline-vascular type of Castleman’s disease with extensive fibrosis ( Fig. 2 A ). Immunohistochemistry staining was positive for EBV (EBER, Fig. 2 B). The patient had negative serology for HIV and the specimen was negative for HHV8. Postoperatively, the patient did well and has required no further operative intervention. This case report was in accordance with the Tufts Medical Center Institutional Case Reports Policy.