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肿瘤与遗传的关系已成为现代生物学和医学遗传学的一个重要课题。我们遇到同一家族姐弟六人中患恶性神经源性肿瘤2例现报告如下。例1,男,22岁,1年前突发右腰腹疼痛,伴恶心呕吐及大汗,曾诊断为阑尾炎而手术。术中见阑尾正常,术后一周出现血尿,伴低热盗汗,曾做B超及肾盂造影诊断为右输尿管结石,右肾积水。多次治疗无效,於1986年5月入院,入院后经B超,下腔静脉,腹腔动脉造影及CT检查为腹膜后肿瘤,剖腹探查,术中见右肾上方内侧10×6×5cm实性肿物固定,右肾受压移位,因肿瘤与大血管粘连无法切除取活检,病理报告为腹膜后恶性神经鞘瘤。於1988年5月因肿瘤广泛转移而死亡。例2,女,32岁,於4年前开始出现月经不调,面部潮红,继之右上腹发现包块逐渐增大,曾做B超诊断为肝癌,於1992年9月入院。查体:重度贫血貌,身高1.44米,体重35kg,BP26/18kPa,腹部触及巨大实性肿物 40 ×30×25cm,经B超,胃肠造影及MR-CT诊断为腹膜后肿瘤,行剖腹探查见肿瘤占据整个腹腔,上界达膈下,下界达骨盆入口,右肾受压推移至右骼窝并与肿瘤联为一体,因肿瘤巨大又行开胸切除整个肿瘤及右肾,切除肿瘤整体标本为35×28×25cm,重10kg。病
Tumor and genetic relationship has become an important topic in modern biology and medical genetics. We met the same family of six siblings suffering from malignant neurogenic tumors are reported 2 cases are as follows. Example 1, male, 22 years old, sudden right lower quadrant pain 1 year ago, with nausea and vomiting and sweating, had been diagnosed as appendicitis and surgery. Surgery see the normal appendix, one week after hematuria, fever with night sweats, have done B-and pyelography diagnosis of right ureteral calculi, right hydronephrosis. Repeated treatment was ineffective, and was admitted to hospital in May 1986. After admission, B-mode ultrasound, inferior vena cava, celiac artery angiography and CT examination were performed on retroperitoneal tumor and laparotomy. See the inside of the upper right kidney for 10 × 6 × 5 cm Fixed tumor, the right kidney pressure shift, due to tumor and large vessel adhesions can not be removed biopsy, the pathological report of retroperitoneal schwannoma. In May 1988 due to extensive tumor metastasis and death. Case 2, female, 32 years old, 4 years ago began to appear irregular menstruation, facial flushing, followed by the right upper quadrant found that the mass gradually increased, had done B-ultrasound diagnosis of liver cancer, admitted in September 1992. Examination: severe anemia, height 1.44 meters, weight 35kg, BP26 / 18kPa, abdomen touches a huge solid mass 40 × 30 × 25cm, by B-, gastrointestinal imaging and MR-CT diagnosis of retroperitoneal tumor, cesarean section Exploring the tumor to occupy the entire abdominal cavity, the upper limit of the diaphragm, the lower bound of the pelvis entrance, the right kidney pressure pushed to the right iliac fossa and with the tumor as a whole, because the tumor is huge and thoracotomy resection of the entire tumor and the right kidney, resection of the tumor The overall specimen is 35 × 28 × 25cm and weighs 10kg. disease