Kimura Disease of Head and Neck

Abstract

Background: Kimura disease is a rare form of chronic inflammatory disorder with unknown etiology. It occurred at subcutaneous tissues, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and salivary gland in the East. Aim and Objectives: The purpose of this paper is to present clinical picture of our cases with literatures review. Materials and Methods: We reported and analyzed 16 cases of Kimura disease from 1996 to 2005 in our hospital, including 14 males and 2 females. Besides, a review of publications in English or Chinese (MEDLINE 1966-2006) as Kimura ('s') disease was performed and compared to our series. Results: The disease presented as either single or multiple lesions over head and neck region, and mainly involved lymph nodes, subcutaneous tissues, and major salivary glands in isolation or in combination. Most cases had peripheral blood eosinophilia but no renal involvement was found. All patients have received at least tumor excision once and oral steroid were used in 5 patients. Follow-up data showed recurrence on 5 patients. Conclusion: The clinical presentation and histopathology of our cases is in accordance with other Kimura cases reported in the literatures and the overall prognosis of Kimura disease is good except high recurrent rate. It remains a therapeutic enigma until its pathogenesis is resolved. In our opinion, complete surgical excision whenever feasible is the preferred treatment despite a not low recurrence rate, especially when the lesions are disfiguring. In the case of head and neck diffuse infiltrating or repeated recurrence, conservative surgical excision is also the first choice.