Clinical Observation of Wilms' Tumor

台大醬院小兒科於1978至1989年12年當中經歷42例威耳姆士氏瘤(Wilm' tumor)。包括19名男童及23名女童。診斷時年齢從7天至10歲，而以6歲以內占大多數。診斷時臨床徵候包括腹部腫瘤(89.2%)，高血壓(57.9%)，血尿(28.2%)，腸胃道症狀(26.3%)，發燒(24.3%)以及體重減輕(21.6%)；有28例源於右側，14例源於左側。各有1例於同側及對側腎臓發生再發（或轉移）；另有1例診斷時以左側腹股溝淋巴結腫大表現。所有病例均經組織學之檢查，包括典型威耳姆士氏瘤（35例），三種變異型－未分化型(anaplastic type)3例，桿球型(rhabdoid type)3例以及亮細胞型(clear cell type)l例。其好發轉移位置以肺、肝及骨骼較多見。治療當中主要的合併症有嚴重的泛血球減少症(pancytopenia)，術後黏連性或麻痺性腸阻塞(adhesive or paralytic ileus)，敗血症或肺炎，傷口癒合不良；罕見放射性肝炎及結腸炎各有1例；另有1例在治療時發生腫瘤破裂合併腹腔內出血。共有20例死亡，其中19例(95 %)發生在診斷後兩年內；死亡之主因是呼吸衰竭（因肺轉移合併肋膜積水，肺炎等），肝機能不全（因肝轉移）, 敗血症及腹腔內出血。本報告之兩年不再發存活率及兩年存活率分別爲51.2%及53.7%。

關鍵字

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並列摘要

Forty-two cases with Wilms' tumor encountered in the National Taiwan University Hospital from 1978 through 1989 were retrospectively reviewed. Included were 19 boys and 23 girls, with an age range at diagnosis from 7 days to 10 years; a majority were in the first 6 years of life. The presenting symptoms and signs included: abdominal mass (89.2%), hypertension (57.9%), hematuria (28.2%), gastrointestinal symptoms (26.3%), fever (24.3%), and body weight loss (21.6%). The initial laterality of tumor was 28 right and 14 left, with one contralateral and one ipsilateral relapse. One extrarenal Wilms' tumor (right inguinal lymph nodes) was encountered. Every case was confirmed by pathology. Histologic findings included typical Wilms' tumor (35/42), rhabdoid (3/42), anaplastic (3/42), and clear cell (1/42) types. The common sites of metastasis were lung, liver and bone. Major complications during or following therapy were severe pancytopenia, ileus, sepsis or pneumonia, delayed wound healing and tumor rupture with hemorrhage. Rare complications included irradiation hepatitis (venoocclusive disease) and colitis. There were 20 deaths. The causes of death were respiratory or hepatic failure due to tumor metastasis, sepsis and internal hemorrhage. Mortality (19/20) usually occurred within two years after diagnosis and therapy. The two-year's relapse-free survival and two-year’s survival rates were 51.2% and 53.7%, respectively.

並列關鍵字

Wilms' tumor ； clinical manifestations ； chemotherapy

國際替代計量

Clinical Observation of Wilms' Tumor

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