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Age / Sex : 37 / M
Age/Sex: 37/M
C.C.: Palpable mass
1) What is your impression?
Courtesy: Mi Sook Sung, MD., The Catholic University of Korea, Holy Family Hospital
Diagnosis: Kimura's disease
Discussion
Findings:
Axial T1-weighted and axial, coronal T2-weighted spin-echo (SE) MR image show partially defined mass in medial subcutaneous tissue of left epitrochlear region. Mass is isointense relative to muscle on T1-weighted SE images and hyperintense relative to muscle on T2-weighted SE images. Stranding in surrounding soft tissue and serpentine signal voids suggest vascular structures. Gadolinium-enhanced coronal fat suppressed T1-weighted SE image with fat saturation shows nearly homogeneous enhancement of mass without central necrotic portion and surrounding soft-tissue edema. Note series of contiguous soft-tissue masses indicating chain of enlarged epitrochlear lymph nodes.
Differential Diagnosis:
tuberculous lymphadenopathy cat-scratch disease lymphoma metastasis soft-tissue sarcomas
Diagnosis: Kimura’s disease
Discussion:
- Kimura's disease is a rare chronic inflammatory disorder of unknown origin that occurs mainly in young Asian male patients.
- male preponderance (3:1), second and third decades of life
- most commonly presents as painless unilateral cervical adenopathy or subcutaneous masses in the head and neck region, mainly involving the major salivary glands and regional lymph nodes; axilla, popliteal region, groin, forearm.
- Consistent location of the soft-tissue mass in the epitrochlear region corresponded with involvement of the lymphatic chain adjacent to the medial neurovascular bundles.
- peripheral blood eosinophilia (10–70%), increased serum IgE (800–35,000 U/mL)
- deposits of IgE in the germinal centers, peripheral eosinophilia, elevated serum IgE level, the appearance of mast cells: immune-mediated disease and atopic in nature
- unknown cause; the histopathologic and laboratory findings suggest an inflammatory response associated with a self-limited allergic or an autoimmune reaction to a stimulus unknown
- Confusion about whether Kimura's disease & other disease entities are variants of the same disease; angiolymphoid hyperplasia with eosinophilia, epithelioid hemangioma, angiofollicular lymphoid hyperplasia, low-grade angiosarcoma, atypical or pseudopyogenic granuloma, eosinophilic granuloma of the soft tissues
References:
1. Choi JA, Lee GK, Kong KY, Hong SH, Suh JS, Ahn JM, Lee YJ, Cho KH, Park JG, Choi JY, Kang HS. Imaging findings of Kimura's disease in the soft tissue of the upper extremity. AJR Am J Roentgenol. 2005 Jan;184(1):193-9.
2. Huang GS, Lee HS, Chiu YC, Yu CC, Chen CY. Kimura's disease of the elbows. Skeletal Radiol. 2005 Sep;34(9):555-8. Epub 2005 May 14.
3. Oguz KK, Ozturk A, Cila A. Magnetic resonance imaging findings in Kimura's disease. Neuroradiology. 2004 Oct;46(10):855-8.
4. Lee YS, Ang HK, Ooi LL, Wong CY. Kimura's disease involving the median nerve: a case report. Ann Acad Med Singapore. 1995 May;24(3):462-4.
5. Kuo TT, Shih LY, Chan HL. Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol. 1988 Nov;12(11):843-54.
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