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REPORT OF EXPERIENCE WITH KIMURA'S DISEASE (기무라씨 질환, 5 예 보고)

  • Seel David J.;Park Yoon-Kyu;Lee Kwang-Min
    • Korean Journal of Head & Neck Oncology
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    • v.5 no.1
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    • pp.39-46
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    • 1989
  • 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 vase 비 ar 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 inguinal swelling of 7 years' duration. The submental mass measured 5x5cm. and the inguinal mass was 8x4cm. in size. Peripheral eosinophilia varying from 14% to 40% was found. On August 20, 1982, the submental mass was removed and a superficial groin dissection was done. In May 1983 an intraoral lesion of the palate was removed. The patient is free of disease. CASE II. A 23-year-old unemployed man visited this hospital for the first time in July, 1984, with swelling of the right cheek present for 6 years. The mass was soft and ill-defined but measured 10x20cm. and extended from the submandibular upper neck to the zygomatic arch, and from the mastoid to the cheek, over the parotid gland. Eosinophilia varying from 27% to 29% was noted in the peripheral blood. On March 21, 1986, the lesion was resected. The procedure comprised an extended superficial parotidectomy from the temporalis fascia to the upper neck. Post-operatively radiotherapy 3000 rad tissue dose was administered using the 6 MeV linear accelerator. The patient remains free of disease. CASE III. A 19-year-old student came to the clinic with masses over both mastoid areas, present 3 years. On the right there were two adjacent lesions, one over the mastoid, the other in the upper jugular level of the neck. On the left it was a single mass over the mastoid. Eosinophilia varied from 13 to 32% in the peripheral blood, and 11.6% in the bone marrow. Incisional biopsy revealed 'eosinophilic granuloma' and a trial of predisolone was employed. The mass increased in size so a small dose of radiation (600 rads) was used, with substantial regression,. The lesion on the left was excised and follwed by 1000 rads radiotherapy. Finally recurrent tumor on the right side was removed on November 5, 1985. The patient remains free of disease. CASE N. A 29-year-old local merchant had had swelling of both upper necks since childhood. At the time of his first visit on March 17, 1986, the right submandibular mass measured 5x3.5cm. and the ,right upper neck and parotid tail mass measured 2.5cm. On the left there were masses in the upper neck, the largest of which measured 2.5cm, and of the parotid tail, 2.0cm. in size.(See Fig. 1) Peripheral eosinophilia of 39% was recorded. Left side partial parotidectomy and resection of the upper neck and subdigstric mases was done on May 2, 1986. The mass involving the right parotid tail and upper neck nodes was removed on Angust 7,1986. Postoperatively the patient was placed on prednisolone 30 mg. per day. No definite masses are palpable. CASE V. A 66-year-old housewife informed us, at the time of her first visit in May, 1986, that she had had multiple neck masses since 10 years ago. On the right side there was a 2.5cm. subcutaneous mass of the upper neck, over the upper jugular chain. On the left there was a 9x4.5cm. mass involving the entire parotid, the post-auricular area and the upper neck. A third mass presented in the submental area and measured 3.5cm. (See Fig. 2) Eosinophilia of 51% was noted in the peripheral blood. partial excision of the left upper neck lesion and complete excision of the submental mass were performed on june 6, 1986. post-operatively she was placed on 20 mg. of prednisolone daily, but when the mass re-grew after two months she was referred to Radiation Therapy for a 2500 rad course of treatment. A barely palpable thickening remains.

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Radiation Therapy for Carcinoma of the Oropharynx (구인두암의 방사선치료)

  • Park, In-Kyu;Kim, Jae-Choel
    • Radiation Oncology Journal
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    • v.14 no.2
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    • pp.95-103
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    • 1996
  • Purpose : A retrospective analysis for patients with oropharyngeal carcinoma who were treated with radiation was performed to assess the results of treatment and patterns of failure, and to identify the factors that might influence survival. materials and methods : From March 1985 through June 1993, 53 patients with oropharyngeal carcinoma were treated with either radiation therapy alone or combination of neoadjuvant chemotherapy and radiation therapy at the Department of Radiation Oncology, Kyungpook National University Hospital. Patients' ages ranged from 31 to 73 years with a median age of 54 years. There were 47 men and 6 women, Forty-two Patients ($79.2\%$) had squamous cell carcinoma, 10 patients ($18.9\%$) had undifferentiated carcinoma and 1 patient ($19\%$) had adenoid cystic carcinoma. There were 2 patients with stage I, 12 patients with stage II, 12 Patients with stage III and 27 patients with stage IV. According to the TNM classification, patients were distributed as follows: T1 7, T2 28, T3 10, T4 7, TX 1, and N0 17, Nl 13, N2 21, N3 2. The primary tumor sites were tonsillar region in 36 patients ($67.9\%$), base of the tongue in 12 patients ($22.6\%$), and soft palate in 5 patients ($9.4\%$). Twenty-five patients were treated with radiation therapy alone and twenty-eight Patients were treated with one to three courses of chemotherapy followed by radiation therapy. Chemotherapeutic regimens used were either CF (cisplatin and 5-fluorouracil) or CVB (cisplatin, vincristine and bleomycin). Radiation therapy was delivered 180-200 cGy daily, five times a week using 6 MV X-ray with or without 8-10 MeV electron beams A tumor dose ranged from 4500 cGy to 7740 cGy with a median dose of 7100 cGy. The follow-up time ranged from 4 months to 99 months with a median of 21 months. Results : Thirty-seven patients ($69.8\%$) achieved a CR (complete response) and PR (partial response) in 16 patients ($30.2\%$) after radiation therapy. The overall survival rates were $47\%$ at 2 years and $42\%$ at 3 years, respectively. The median survival time was 23 months. Overall stage (p=0.02) and response to radiation therapy (p=0.004) were significant prognostic factors for overall survival. The 2-year disease-free survival rate was $45.5\%$. T-stage (p=0.03), N-stage (p=0.04) and overall stage (P=0.04) were significant prognostic factors for disease-free survival. Age, sex, histology, primary site of the tumor, radiation dose, combination of chemotherapy were not significantly associated with disease-free survival. Among evaluable 32 Patients with CR to radiation therapy, 12 patients were considered to have failed Among these, 8 patients failed locoregionally and 4 Patients failed distantly. Conclusion : T-stage, N-stage and overall stage were significant prognostic factors for disease-free survival in the treatment of oropharyngeal cancer Since locoregional failure was the predominant pattern of relapse, potential methods to improve locoregional control with radiation therapy should be attempted. More controlled clinical, trials should be completed before acceptance of chemotherapy as a part of treatment of oropharyngeal carcinoma.

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