先天性无肛直肠舟状窝瘘手术186例

1临床资料1988-06/2003-12我院共186例,均为女性,无肛门,其中0～6月龄21例,0.5～1岁96例,1～3岁53例,3～8岁15例,13岁1例.以大便从会阴部舟状窝瘘口处排出而就诊,患儿有不同程度的排便困难,严重时伴腹胀呕吐,多数患儿有营养不良性贫血.瘘口直径0.3～0.6 cm,直肠盲端到肛穴皮肤之间的距离1.0～2.5 cm,属于中、低位肛门直肠畸形.本组有3例合并先天性心脏病-室间隔缺损,2例合并骶前囊肿,1例合并脐疝.     

Autoimmune Esophagitis: IgG4-related Tumors of the Esophagus

We present a case of a 23-year-old gentleman who presented with dysphagia, weight loss, and recurrent esophageal strictures requiring multiple dilatations. An endoscopic ultrasound with esophagogastroduodenoscopy revealed a mass present in the distal esophagus. Fine needle aspiration suggested that the mass in the lower esophagus resembled a gastrointestinal stromal tumor. After surgical resection, final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammatory cells with an increase and predominance of IgG4-positive plasma cells. The microscopic appearance was consistent with a benign condition as a result of an IgG4-related process. He did not, however, have any other symptoms indicative of systemic autoimmune disease or connective tissue disorders. We present the pre-operative imaging, operative management, pathologic diagnosis, and literature review of this rare condition and the first known report of autoimmune esophagitis as part of the IgG4 spectrum of diseases.     

Squamous cell carcinoma of the anal margin

PURPOSE: To define the optimal treatment of patients with squamous cell carcinoma (SCCA) of the anal margin. METHODS: Nineteen patients treated with curative intent by radiotherapy (RT) alone or combined with adjuvant chemotherapy (CTX) between 1979 and June 2000 were analyzed. The pertinent literature was reviewed and discussed as it related to our experience. RESULTS: Local control after RT or RT and CTX was observed in all 19 patients (100%). One T1 patient developed inguinal lymph node metastases and subsequently died secondary to regional and distant disease. This patient did not receive elective inguinal node RT; the lymph nodes of the other 18 patients in this analysis were irradiated. Four patients died of intercurrent disease at 25, 29, 37, and 113 months after RT, respectively. The remaining 14 patients were alive and disease-free from 52 to 143 months after treatment. No patient suffered a severe complication or required a diverting colostomy or an abdominoperineal resection (APR) after treatment. Review of the literature reveals that the probability of cure is similar after RT alone or combined with CTX compared with surgery. Therefore, the choice of treatment depends on the anticipated functional result. CONCLUSIONS: Patients with SCCA of the anal margin have a high likelihood of cure with sphincter preservation after RT or RT and CTX. Patients with well to moderately differentiated T1 tumors may undergo excision if it can be accomplished without compromising the sphincter. The remainder are treated with RT. Adjuvant CTX is indicated for those with T3-T4 tumors and/or involved regional nodes.     

Aggressive fibromatosis

The purpose of this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis. Data indicate the likelihood of local recurrence after surgery is high, particularly if margins are positive. Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75% to 80%. Treatment with pharmacologic agents results in objective response rates of approximately 40% to 50%; duration of response is variable. Thus, the optimal management for aggressive fibromatosis depends on tumor location and extent. Surgery is indicated if the lesion appears to be completely resectable with acceptable function and cosmesis. Radiotherapy is indicated for patients with unresectable tumors; those with positive margins after resection should be considered for adjuvant radiotherapy depending on the location and extent of the tumor. Pharmacologic treatment should be considered for patients with progressive disease after unsuccessful local-regional therapy.     

Inducible nuclear factor-kappaB activation contributes to chemotherapy resistance in gastric cancer

BACKGROUND: Activation of nuclear factor-kappaB (NF-kappaB) inhibits chemotherapy-induced apoptosis in some cancer cell lines. Inhibition of NF-kappaB by adenoviral delivery of an IkappaBalpha superrepressor (Ad.IkappaBalpha-SR) should potentiate 5-fluorouracil (5-FU) and irinotecan chemotherapy in gastric cancer cells. STUDY DESIGN: NCI-N87 and AGS human gastric cancer cells were studied. Chemotherapy-induced NF-kappaB activation was assessed using a luciferase reporter assay. Inhibition of NF-kappaB was assessed by luciferase reporter assay and by electrophoretic mobility shift assay. Cells were pretreated for 1 hour with Ad.IkappaBalpha (25 MOI) and incubated with 5-FU or the active metabolite of irinotecan (SN-38). Cell growth was assessed by cell proliferation assay and induction of apoptosis was determined by flow cytometry and caspase 3/7 assay. RESULTS: 5-FU and SN-38 significantly induced NF-kappaB activation as measured by luciferase reporter assay (p < 0.001). Ad.IkappaBalpha-SR treatment inhibited NF-kappaB binding as demonstrated by electrophoretic mobility shift assay and by luciferase reporter assay. In AGS cells, pretreatment with Ad.IkappaBalpha-SR followed by 5-FU (0.005 mmol/L) or SN-38 (10 ng/mL) led to increased growth inhibition of 13% and 59%, respectively (p < 0.001). Similarly, growth inhibition in NCI cells was significantly increased by pretreatment with Ad.IkappaBalpha followed by 5-FU (0.001 mmol/L) or SN-38 (0.5 ng/mL) (p < 0.001). In both cell lines, Ad.IkappaBalpha-SR enhanced apoptosis by both flow cytometry and caspase 3/7 assay as compared with chemotherapy alone. CONCLUSIONS: NF-kappaB is activated in human gastric cancer in response to chemotherapy and may result in inducible chemoresistance. Inhibition of NF-kappaB by Ad.IkappaBalpha-SR enhances the antitumor effects of chemotherapy and has potential as a novel antineoplastic strategy.     

Metastatic potential of dermatofibrosarcoma protuberans with fibrosarcomatous change

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon fibrohistiocytic tumor that is locally aggressive. DFSP is associated with frequent local recurrences but rarely metastasizes. It has been suggested that fibrosarcomatous change within a dermatofibrosarcoma protuberans (DFSP-FS) is associated with increased risk of local recurrence and distant metastases. Metastases remain rare, however, and are typically preceded by multiple local recurrences. We report a rare case of a DFSP-FS metastasis to the head of the pancreas without any preceding local recurrence.     

Significance of micrometastases in colorectal cancer

Conclusion The combined effort of pathologists, molecular biologists, and surgeons has allowed a very focused examination on select lymph nodes within a specimen. As such, clinicians are afforded the pathologic diagnosis with a smaller and smaller extent of disease. At present, the detection threshold has exceeded the clinical knowledge: that is to say, the mere presence of disease in minute amounts has not yet been studied extensively enough to identify prognostic information from such or to alter the current therapy offered to patients. Continued efforts to define “clinically relevant micrometastases” should persist.