Abstract

Introduction. Angiofollicular lymph node hyperplasia (Castleman's disease) is a nonmalignant lymphoproliferative disorder that generally involves the lymph nodes of young adults, most commonly in the mediastinum. Rarely, Castleman's disease may present in the parotid gland. The disease can be further classified into unicentric or multicentric forms, with considerable differences in presentation, treatment, and prognosis. Case(s). We present cases of two pediatric patients, aged 7 and 11, who both presented with a slow-growing, painless parotid mass. In each case, the mass was excised via a superficial parotidectomy and the diagnosis made postoperatively upon further pathologic examination. At 6 months of follow-up, both had fully intact facial nerve function and no evidence of recurrence. Discussion. Castleman's disease presents a diagnostic challenge in the head and neck region, as radiographic characteristics and fine needle aspiration results are often inconclusive. Definitive diagnosis requires surgical excision for pathologic examination. The unicentric form generally presents as a painless mass and can be successfully treated with complete excision. The multicentric form is associated with constitutional symptoms and its treatment remains controversial. Conclusion. Although rare, clinicians should be aware of both forms of Castleman's disease when creating a differential diagnosis for parotid masses.

Highlights

  • Angiofollicular lymph node hyperplasia (Castleman’s disease) is a nonmalignant lymphoproliferative disorder that generally involves the lymph nodes of young adults, most commonly in the mediastinum

  • Castleman’s disease (CD) generally occurs in young adults [1] and has no gender predilection [3]

  • We present 2 cases of UCD that were treated over a 15-year span at Children’s Hospital Los Angeles

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Summary

Introduction

Dr Benjamin Castleman first described the nonmalignant lymphoproliferative disorder of angiofollicular lymph node hyperplasia in a group of patients with thymoma-like masses of the anterior mediastinum in 1956 [1]. CD generally occurs in young adults [1] and has no gender predilection [3]. This disease most commonly involves mediastinal lymph nodes (60–86%) [1, 4], it has the potential to involve any lymph node in the body. CD may present in either unicentric (UCD) or multicentric (MCD) form. The MCD, or systemic form, has been linked to HHV8 infection and presents with generalized lymphadenopathy, abnormal laboratory values, malaise, night sweats, hepatosplenomegaly, weight loss, and an aggressive clinical course [3]. Presenting as a parotid mass, 24 cases of UCD and 5 cases of MCD. We present 2 cases of UCD that were treated over a 15-year span at Children’s Hospital Los Angeles

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