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[Isolated Rosai-Dorfman disease].
A M Gassel1, D M Lamskemper, J Sold-Darseff
1Institut für Pathologie, Universität Würzburg. a.gassel@mail.uni-wuerzburg.de
Der Pathologe
|May 6, 2004
Summary
This case report details extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) presenting unusually in the orbit and sinuses. Early diagnosis is crucial for managing this rare systemic condition.
Area of Science:
- Oncology
- Pathology
- Immunohistochemistry
Background:
- Extranodal sinus histiocytosis with massive lymphadenopathy (ENSHML), also known as Rosai-Dorfman disease, is a rare benign proliferative disorder.
- While typically affecting lymph nodes, extranodal presentations can occur, posing diagnostic challenges.
Observation:
- A patient presented with an intracranial tumor, exophthalmos, and a large orbital and paranasal sinus mass.
- Initial biopsy revealed fibrous tissue with macrophages; subsequent evaluation identified a retroperitoneal mass causing ureteral stenosis.
- Histological and immunohistochemical analysis of the orbital mass confirmed S-100-positive histiocytes and emperipolesis, indicative of ENSHML.
Findings:
- The diagnosis of extranodal sinus histiocytosis was delayed due to its rare, isolated extranodal location.
- The case highlights the importance of considering systemic disease in unusual presentations.
Implications:
- This report underscores the need for comprehensive evaluation in suspected rare histiocytic disorders.
- Reviewing literature on extranodal ENSHML manifestations is essential for accurate diagnosis and management.
- Referral to specialized centers is recommended for complex and rare cases.