Purpose or Case Report: Kimura’s disease is rare chronic inflammatory disorder of unknown etiology. The cause and pathogenesis is unclear. Our study is to describe the clinical and radiologic features of Kimura’s disease (KD) in children and young adults, which often presents as a diagnostic challenge.
Methods & Materials: We retrospectively reviewed patients under 20 years old who were pathologically confirmed as KD and have available imaging studies from January 2000 to March 2022. Clinical features including initial symptoms and signs, the presence of eosinophilia and Ig E level, treatment, follow up including recurrence, and biopsy result were reviewed. Radiologic features were assessed in terms of location, laterality, multifocality, margin, MR signal intensity, degree of enhancement, and presence of regional lymphadenopathy in the available imaging studies including CT, MR and US.
Results: There were 17 patients. 16 were males and there was only one female. Mean age was 13.5yo with range of 9-20yo. All patients presented with palpable, non-tender mass or swelling. All patients presented varying degree of eosinophilia and elevated Ig E. Bilaterality and multifocality ware common features. Up to 5 lesions were found in 1 patient. 88% patients had head and neck lesions. In 24% of patients, lesions were found not only in the head and neck, but also in other areas and 2 had lesions only in epitrochlear, and inguinal region. Radiologic features are ill-defined with adjacent fat infiltration in 21 and relatively well-defined in 12. Regional lymphadenopathy was noted in 27. 25 lesions showed mild enhancement on CT. All lesions on MR showed T1 iso-and T2 hyper signal intensity compared with adjacent muscles. Enhancement was more prominent on MR than CT. Of 17 patients/33 lesions, biopsy was done for 22 lesions and all confirmed as KD. 8 lesions were confirmed after excisional biopsy. All patients underwent surgical resection and 13 had additional steroid and some patients received chemotherapy or radiation. For median 92.7 months of follow up 12 patients experienced recurrence in the same area and 2 patients had recurrence at different site and only 3 patient had no recurrence.
Conclusions: Kimura's disease was typically presented as painless palpable mass in parotid and buccal area predominantly affecting male with associated eosinophilia and elevated Ig E level, occasionally involving forearm and inguinal region. The lesions tend to be multiple and bilateral, ill-defined masses with associated regional lymphadenopathy.