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To evaluate the genetic factors of familial predisposition to gastric cancer, genetic alterations in the surgically resected stomach samples from gastric-cancer-prone families were investigated. Familial gastric cancer (FGC) was defined as gastric cancer occurring in a family with 3 or more gastric cancer patients over at least two successive generations. We examined replication error (RER) of six microsatellite markers and screened mutations of the 10-(A) repeat sequence in the transforming growth factor-beta receptor type II (TGF-betaRII) gene in individuals from seven unrelated FGC families. Three cases showed RER at one of the six (CA)n microsatellite markers but the other 4 cases showed no RER at any of these loci. No mutation was found in the 10-(A) repeat of the TGF-betaRII gene. Additionally, no germline mutation was found by polymerase chain reaction-single strand conformation polymorphism in exons 1-16 of E-cadherin, exons 5-8 of p53 and in the mutation cluster region of APC. These results indicate that disorders in the DNA mismatch repair system, E-cadherin, p53 and APC may be infrequently involved in the carcinogenesis of Japanese FGC.

Primary carcinoid tumor of the jejunum is rare, and is an unusual cause of massive gastrointestinal bleeding. A case of primary jejunal carcinoid tumor in a 39-year-old woman who presented with massive hematochezia is described. Both upper and lower gastrointestinal endoscopies showed no abnormalities. An abdominal computed tomographic scan, small-bowel barium contrast studies, and small-bowel endoscopy showed a subserosal mass, of 5 × 4 cm, with a cavity suggesting central necrosis, and a deep mucosal ulceration, located in the proximal jejunum. Although these clinical presentations were strongly suggestive of a leiomyosarcoma, histologic examination of biopsy samples obtained by enteroscopy confirmed the diagnosis of jejunal carcinoid tumor. The patient underwent radical jejunal resection and recovered uneventfully. In spite of the large size of the tumor, there was one solitary lymph node metastasis, but no evidence of liver metastases. This kind of jejunal carcinoid tumor, presenting with massive gastrointestinal bleeding and a subserosal bulky growth mimicking a leiomyosarcoma, has not been reported previously. Moreover, this is a rare case of a jejunal carcinoid which was diagnosed preoperatively by small bowel-endoscopic biopsy.

BACKGROUND In patients with thoracic esophageal carcinoma, radical dissection of the upper mediastinal lymph nodes often leads to complications such as recurrent laryngeal nerve palsy and subsequent pulmonary disorders. Intraoperative radiation therapy (IORT) to the upper mediastinum and nerve-sparing three-field lymphadenectomy followed by external beam radiotherapy has been developed to improve the locoregional control rate without resulting in these major postoperative complications. METHODS Three-field lymphadenectomy, including cervical, mediastinal, and abdominal lymph node dissection, was performed. Dissection of the upper mediastinum was conservative to preserve recurrent laryngeal nerve function. IORT of 12-25 grays (Gy) was applied to the upper mediastinum. Postoperative radiation therapy (PORT) of 45 Gy in 16 fractions over 4 weeks was applied to the entire neck and upper mediastinum using an external X-ray beam. Between 1989-1996, 121 patients with thoracic esophageal carcinoma underwent surgery and received IORT, and 103 of these patients underwent PORT as part of their treatment schedule. RESULTS The surgical mortality rate was 0.8% (1 of 121 cases). The overall 5-year survival rate was 34.4% and the cause specific 5-year survival rate was 54.8%. The cause specific 5-year survival rate for pN0 tumors was 79.4% and was 43.8% for pN1 tumors. No patients died with locoregional recurrence in the mediastinal lymph nodes. Recurrent laryngeal nerve palsy was observed in 25 patients (21%), but the palsy remained for > 1 month in only 13 patients (11%). Mechanical ventilation support for > 48 hours was required for 22 patients (18.2%). Fatal tracheal ulcers occurred in 4 of 18 patients who received the highest IORT dose of 25 Gy. CONCLUSIONS Three-field lymphadenectomy to preserve recurrent laryngeal nerves and IORT using 12-20 Gy followed by 45-Gy PORT effectively reduced locoregional recurrence, recurrent laryngeal nerve palsy, and pulmonary complications caused by radical surgical dissections. The minimally effective dose of IORT appears to be ≤ 15 Gy, a factor that will be further evaluated with longer follow-up. Cancer 1999;86:6–13. © 1999 American Cancer Society.

Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40 °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.

achieved in 42(50%): a mean of 4.8 2.8 sessions overall; 3.9 2.5 for RFA, 5.8 2.8 for RF EMR/ESD, 10 for RF CA pt, 3 for RFA EMR/ESD CA pt. By histology, 50% of patients with HGD achieved CE-IM; 80% of patients with LGD achieved CE-IM, 60% of patients with EC achieved CE-IM, 93% of patients with IM achieved CE-IM. In patients who completed therapy, CE-D was achieved in 35/43(81%) overall: 85%(mean 4.5 2.3 sessions) in the RFA group, 81%(mean 7.5 3.7 sessions) in the RF EMR/ESD group, 100% (mean 10 sessions) in the RFA CA group. One pt with HGD developed a nodule after RFA, underwent EMR revealing invasive cancer and was referred for esophagectomy. Positive correlation was seen between length of BE and number of sessions for CE-IM (R .52, p .01). SF occurred in 17 (20%) pts; 4 in the RFA only group, 13 receiving combination therapy, all treated endoscopically. 28 (33%) of patients have not achieved eradication but are still undergoing therapy. 3 patients (4%) died of unrelated causes during treatment. 11 (13%) patients were lost to followup or discontinued therapy. Conclusions: 1. RF is overall safe, alone or in conjunction with other modalities. 2. Complete ablation is most frequent in the IM and LGD patients as per previous studies. 3. Progression to cancer not amenable to endoscopic therapy is low in our experience. 4. Stricture formation is relatively common in multimodality therapy compared to RF alone, but is amenable to endoscopic therapy. 5. Overall success rate appears lower with proposed numbers by multicenter study group and may need prolonged treatment sessions to eradicate BE.

We evaluated the efficacy and tolerability of a dual therapy with rabeprazole and amoxicillin (AMX) as an empiric third-line rescue therapy. In patients with failure of first-line treatment with a proton pump inhibitor (PPI)-AMX-clarithromycin regimen and second-line treatment with the PPI-AMX-metronidazole regimen, a third-line eradication regimen with rabeprazole (10 mg q.i.d.) and AMX (500 mg q.i.d.) was prescribed for 2 wk. Eradication was confirmed by the results of the 13C-urea breath test (UBT) at 12 wk after the therapy. A total of 46 patients were included; however, two were lost to follow-up. The eradication rates as determined by per-protocol and intention-to-treat analyses were 65.9% and 63.0%, respectively. The pretreatment UBT results in the subjects showing eradication failure; those patients showing successful eradication comprised 32.9 ± 28.8 permil and 14.8 ± 12.8 permil, respectively. The pretreatment UBT results in the subjects with eradication failure were significantly higher than those in the patients with successful eradication (P = 0.019). A low pretreatment UBT result (≤ 28.5 permil) predicted the success of the eradication therapy with a positive predictive value of 81.3% and a sensitivity of 89.7%. Adverse effects were reported in 18.2% of the patients, mainly diarrhea and stomatitis. Dual therapy with rabeprazole and AMX appears to serve as a potential empirical third-line strategy for patients with low values on pretreatment UBT.