小腸平滑肌肉瘤相當罕見。回顧過去十四年來,三軍總醫院共有27例小腸平滑肌肉瘤經病理組織切片證實。患者發病年齡為54歲(自20至71歲)。男女發病比率為1.8:1。除兩例於接受其他手術時意外發現外,大部份的平滑肌肉瘤皆因引起臨床症狀,進而手術獲致診斷。出血或貧血是最常見的症狀共計19例,某次是腹痛14例,腫塊11例,穿孔6例,體重減輕5例及腸阻塞4例。大致上,腫瘤呈均勻地散布在小腸各部份。腹部電腦斷層,血管攝影及小腸顯影劑照相三者的診斷敏感度最好。分別有24例及3例的病患接受根除性或姑息性手術治療。手術死亡率為7%。小腸平滑肌肉瘤預後欠佳,這可由其一年,三年及五年的存活率分別為84%,57%及38%可見一斑。其中,患者的存活率有意義的受腫瘤發現時的大小,Dukes的分期及可施行的手術方式所影響。熟捻這類腫瘤的症狀,同時保持高度的警覺心仍為獲致早期診斷及良好預後的不二法門。
Leiomyosarcomas of the small intestine are relatively uncommon. Between 1983 and 1996, 27 pathology proven cases of leiomyosarcoma at the TSGH were reviewed. Patient age ranged from 20 to 71 years (mean, 54 years) with a 1.3:1 male-female ratio. Most caused symptoms leading to surgical diagnosis; two were detected serendipitously during operations for hepatoma and perforated duodenal ulcer, respectively. The most common presentation was bleeding/anemia (70%) followed by pain (52%), palpable mass (41%), perforation (22%), weight loss (19%) and obstruction (15%). Tumors occurred evenly throughout the small intestinal tract. Abdominal CT scan, angiography, and small intestinal contrast studies had the most useful sensitivity rates. Tumors were operated by curative attempt in 89% and palliatively in 11% of patients. The operation-related mortality rate was 7%. Dismal survival rate was demonstrated with 1-year, 3-year, and 5-year survival rate of 84%, 57%, and 38%, respectively. The survival rate was significantly influenced by tumor size (P<0.05), stage of Dukes' classification (P<0.05) and operation performed (P<0.02). Awareness of the constellation of symptoms & a high clinical suspicion remain the cornerstone of early diagnosis and better outcome.