Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 30, 2020
Kimura Disease
- Background:
- Kimura disease is a rare entity seen primarily in Asian patients characterized by lymphfolliculoid granuloma with infiltration of the surrounding tissues by eosinophils, often with concomitant peripheral blood eosinophilia and elevated serum IgE.
- Clinical course is typically progressive but the disease is benign and may be self-limited, though it may be complicated by renal involvement, notably nephrotic syndrome.
- Clinical Presentation:
- Painless masses (most commonly in the head and neck) and local lymphadenopathy
- Key Diagnostic Features:
- Imaging triad
- Subcutaneous and deep-tissue masses in the head and neck that are isoattenuating on CT and show enhancement
- Salivary gland masses: parotid > submandibular; the masses are avidly enhancing on a background of less-enhancing salivary tissue, causing a heterogeneous appearance of the gland. On MRI the masses are typically T1 iso/hypointense and T2 hyperintense relative to the salivary tissue.
- Solid cervical lymphadenopathy with enhancement
- Clinical triad
- Painless subcutaneous head and neck masses with regional adenopathy
- Blood and tissue eosinophilia
- Markedly elevated serum IgE
- Imaging triad
- Differential Diagnoses:
- Benign reactive nodes/infectious mononucleosis/drug reactions: acute onset, no subcutaneous nodules
- Tuberculous adenitis: centrally hypoattenuating and may be confluent
- Nodal metastases: ill-defined, hypoechoic masses with abnormal vascularity and associated primary malignancy (SCCA, NPC, melanoma)
-
Lymphoma/leukemia: multiple solid, hypoechoic nodes; reticulated echo pattern; hilar > peripheral vascularity; ± posterior enhancement
-
Castleman disease: mostly mediastinal nodes showing avid enhancement + calcification in 15%
- Parasitic infections: well-defined lymph nodes with normal morphology and without accompanying subcutaneous nodules
- Treatment:
- There is no standard treatment. Options include conservative management, radiotherapy, resection, or medical management (eg, steroids, oxyphenbutazone [with only transient improvement], and cyclosporine).