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Joseph Junewick, MD FACR
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LCH Adenitis - Rosai Dorfman Disease

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

6 month old male with extensive adenopathy.


Case Images


Diagnosis

LCH Adenitis – Rosai Dorfman Disease

Findings

MR – Coronal post-gadolinium images show massive bilateral nuchal adenopathy, salivary gland involvement and thymic infiltration.

Discussion

Rosai-Dorfman disease is a benign, pseudolymphomatous entity with various imaging manifestations in the head and neck. Involvement of the cervical lymph nodes resulting in massive bilateral lymphadenopathy is the most characteristic finding described in the literature. Extranodal disease of the head and neck occurs in up to 43%of patients and occurs in the absence of lymphadenopathy in approximately 23% of patients. Although Rosai-Dorfman disease is most commonly diagnosed in children and adolescents presenting with cervical lymphadenopathy, all age groups may be encountered. Postulated causes include infectious causes, immunodeficiency, autoimmune disease, and a neoplastic process. most of those afflicted are younger than 20 years (80%). There is also an increased incidence among male patients and those of African and West Indian heritage. Although no specific imaging characteristics allow differentiation of lymphadenopathy in Rosai-Dorfman disease from myriad other disease processes, massive painless bilateral cervical lymph node enlargement, particularly when occurring in children and adolescents, should promote its consideration.

Reference

LaBarge DV, Salzman KL, Harnsberger R, et al. Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): Imaging Manifestations in the Head and Neck. AJR (2008); 191:W299-306



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