기무라씨 질환, 5예 보고

• 영문명: REPORT OF EXPERIENCE WITH KIMURA'S DISEASE
• 발행기관: 대한두경부종양학회
• 저자명: 설대위(David J Seel) 박윤규(Yoon Kyu Park) 이광민(Kwang Min Lee)
• 간행물 정보: 『대한두경부종양학회지』제5권 제1호, 39~46쪽, 전체 8쪽
• 주제분류: 의약학 > 종양학
• 파일형태: PDF
• 발행일자: 1989.11.30

국문 초록

기무라씨 질환은 특히 두경부 부위에 피하 종괴를 일으키는 만성염증성, 증식성 질환이다. 저자들은 최근 치험하였던 본 질환 5 예를 한국외과 문헌에 처음으로 보고하는 바이다. 기무라씨 질환은 크게는 ALHE(Angiolymphoide Hyperplasia with Eosinophilia) 의 범주에 속한다. 본 질환의 병리학적 특징은 증식된 lymphoid follicles, eosinophilic infiltration 과 혈관의 증식성이다. 이 질환은 이하선, 악하선 및 상부 경부 부위등에 흔히 종괴를 일으키며 이들 종괴들은 피하조직 뿐만 아니라 타액선과 상부 경부 임파선에까지도 파고 든다. 저자들의 증례 중 한명에서는 서혜부에 종괴가 있었으며 새로이 증식된 혈관과 동상들 (Sinusoids) 로 인하여 혈관 분포가 매우 풍부하였다. 저자들 증례 5 명의 평균 연령은 35세이었지만 한명을 제외한 나머지 모두는 38세 이하이었다. 남녀비는 3:2 이었으며 증상의 평균 기간은 5.2 년이었다. 전례에 있어서 말초 혈액 소견상 Eosinophilia 가 있었다. 전례에서 다발성 종괴들을 보였으며 가끔은 대칭적이기도 하였다. 저자들이 시행한 치료 양상은 수술만 시행한 경우와 수술 및 스테로이드 홀몬요법 시행 경우가 각각 1례씩이었고 수술과 방사선조사 경우가 2례이었으며 나머지 1례에서는 수술, 스테로이드 홀몬요법 및 방사선조사의 복합치료를 실시하였다. 저자들은 기무라씨 질환과 ALHE 질환과의 관계를 고찰해 보았으며 기무라씨 질환의 치료 경험을 보고하는 바이다.

영문 초록

Kimura's Disease is a chronic inflammatory and proliferative condition producing subcutaneous masses especially in the head and neck area. This report of our experience with 5 patients with this disease is the first in the Korean surgical literature. Kimura's Disease is thought to be part of the larger spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). It is characterized pathologically by hyperplastic lymphoid follicles, eosinophilic infiltration, and vascular proliferation. It produces masses which are most common in the area of the parotid, submandibular gland and upper neck. These masses occupy the subcutaneous tissues but also extend into salivary tissue and into upper neck nodes. One of our patients had masses in the groin. The tumors are extremely vascular due to the presence of new proliferative vessels and sinusoids. The average age of our 5 patients was 35, but all but one case were younger than 38 years of age. The male: female ratio was 3 : 2, and the average duration of symptoms was 5,2years. All patients had peripheral blood eosinophilia. All had multiple masses, sometimes symmetrical. The management was surgery alone in one case, surgery and steroids in one case, surgery and radiotherapy in two cases, and all three modalities in one case. The relationship of this entity to ALHE and our experience in the management of this disease are presented. A clinicopathological discrepancy alerted us to the existence of Kimura's Disease. A nineteen-year old male presented with subcutaneous masses over both mastoid areas present for 3 years (Case III). When biopsy on each side was reported as 'eosinophilic granuloma' we submitted the slides to an internationally expert pathologist. Symmetrically occurring tumors in the peri-parotid subcutaneous areas did not fit any category of neoplasm or granuloma known to us. The diagnosis, made by Dr. Gist Fan at the Ochsner Clinic, was Kimura's Disease. We found two additional cases in a review of soft tissue eosinophilic granuloma previously reported at Presbyterian Medical Center, and since then have diagnosed two new cases. These five cases constitute the basis for this, the largest series to be reported in Korea. These vascular, tumor-like lesions of the skin, subcutaneous areas and subjacent structures of the head and neck have been a variety of names, such as angiolymphoid hyperplasia with eosinophilia, eosinophilic hyperplastic lymphogranuloma, angioblastic lymphoid hyperplasia with eosinophilia, histioid hemangioma, and epithelioid hemangioma. The history of this disease spectrum dates back to 1937 when Kimm and Szeto (1) reported 7 cases of 'eosinophilic hyperplastic lymphogranuloma' in the Proceedings of the Chinese Medical Journal. In 1948 Kimura and his associates(2) reported additional cases in Japan under the title 'On the unusual granulation combined with hyperplastic changes of lymphatic tissue.' From then until 1966 several hundred cases were reported in China and Japan. The first report from the West was by Wells and Whimster(3) in the British Journal of Dermatology, in 1969. These authors coined the term, angiolymphoid hyperplasia with eosinophilia (ALHE). Since that time a debate has ensued as to whether Kimura's Disease and ALHE are distinct entities, or whether Kimura's is part of the larger spectrum of ALHE, perhaps a later or advanced phase. From the clinical perspective, surgeons should be aware of the diagnosis of Kimura's Disease not only as part of the differential diagnosis of head and neck tumors but also because these lesions are indolent, and generally require conservative surgical removal as part of the management program. CASE Ⅰ. A 37-year-old female company employee presented in August 1982 with submental swelling of 12 years'duration and with in guinal swelling of 7 years'duration. The submental mass measured 5x5cm. and the inguinal mass was 8×4cm. in size. Perip-heral eosinophilia varying from 14% t

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Summary of Clinical Experience

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