Scholarlink

<p>Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histiocytic disorder that typically presents with massive lymphadenopathy, while isolated extranodal involvement is uncommon. Sinonasal involvement is particularly rare and may mimic inflammatory or neoplastic conditions. We report a case of a 52-year-old male presenting with a nasal mass extending into all paranasal sinuses and the orbit, without associated lymphadenopathy. Radiological findings suggested a paranasal sinus mucocele with underlying fibrous dysplasia, posing a diagnostic challenge. The patient had previously been misdiagnosed as rhinoscleroma and had undergone two surgeries. Histopathological examination confirmed Rosai–Dorfman disease, demonstrating emperipolesis with S100 and CD68 positivity. Increased IgG4-positive plasma cells were noted (IgG4/IgG ratio ~ 30–35%) without evidence of storiform fibrosis or obliterative phlebitis, helping to exclude IgG4-related disease.This case highlights the diagnostic complexity of sinonasal Rosai–Dorfman disease due to its atypical presentation, radiological mimicry, prior misdiagnosis, and histological overlap.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Unveiling Rosai-Dorfman Disease: A Rare Case of Sinonasal Involvement and Diagnostic Challenges

  • 期刊论文

  • 发表日期：

    2026年6月22日

查看全文
  • K. V. Apoorva,
  • Rayappa Chinnusamy,
  • Archana Lakshmanan,
  • Shruti Venkitachalam,
  • Priyadharshini Mahendra Varman

摘要

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histiocytic disorder that typically presents with massive lymphadenopathy, while isolated extranodal involvement is uncommon. Sinonasal involvement is particularly rare and may mimic inflammatory or neoplastic conditions. We report a case of a 52-year-old male presenting with a nasal mass extending into all paranasal sinuses and the orbit, without associated lymphadenopathy. Radiological findings suggested a paranasal sinus mucocele with underlying fibrous dysplasia, posing a diagnostic challenge. The patient had previously been misdiagnosed as rhinoscleroma and had undergone two surgeries. Histopathological examination confirmed Rosai–Dorfman disease, demonstrating emperipolesis with S100 and CD68 positivity. Increased IgG4-positive plasma cells were noted (IgG4/IgG ratio ~ 30–35%) without evidence of storiform fibrosis or obliterative phlebitis, helping to exclude IgG4-related disease.This case highlights the diagnostic complexity of sinonasal Rosai–Dorfman disease due to its atypical presentation, radiological mimicry, prior misdiagnosis, and histological overlap.