Thalamic hyperdensity on computer-associated tomography (CT) has been reported as a disease-specific finding for GM2-gangliosidosis. However, a boy with the typical clinical symptoms and enzyme defect of infantile GM1-gangliosidosis had hyperdense thalamic lesions at 12 months of age. At the age of 34 months, the thalamus showed atrophy and the hyperdensity was restricted to the medial part of the thalamus, which exhibited increased intensity on T1-weighted and decreased intensity on T2-weighted images. Thalamic hyperdensity may be specific not only to GM2-gangliosidosis but also to GM1-gangliosidosis.

Atrophy↗

PubMedDate not supplied

Merosin-negative non-Fukuyama-type congenital muscular dystrophy: a case report.

We report a female infant with non-Fukuyama-type congenital muscular dystrophy with merosin deficiency. She manifested marked hypotonia and muscle weakness from the neonatal period, with an elevated creatine kinase concentration. Her motor developmental milestones were markedly delayed; however, her intellectual development was normal. Although cranial computed tomography (CT) at 3 months of age was normal, subsequent CT at 16 months of age demonstrated diffuse, abnormal white matter lucencies. Muscle biopsy findings at 16 months of age were compatible with those of congenital muscular dystrophy. In addition, no muscle fibers were immunostained by the merosin antibody. The patient died of pneumonia at 23 months of age. These clinical symptoms and CT findings are similar to those described in patients with merosin-negative congenital muscular dystrophy in European countries.

Brain↗

PubMedDate not supplied

Angiogenesis inhibitor, TNP-470, suppresses growth of peritoneal disseminating foci.

BACKGROUND/AIMS: Angiogenesis is critical not only for growth of primary tumors but also for cells established at distant organs. We investigated the effects of angiogenesis inhibitor, TNP-470, on the establishment and growth of intraperitoneally inoculated human gastric cancer cell line, MKN-45, and survival of nude mice with this tumor. METHODOLOGY: Human gastric cancer cell line, MKN-45, were injected into the peritoneal cavity of an ICR nude mouse and a model of peritoneal dissemination was developed. TNP-470 was injected subcutaneously every other day from day 1 until sacrifice or death. The effects of TNP-470 on MKN-45 cells were also examined in vitro. RESULTS: Although the number of disseminated foci was not significantly different, the maximum size was significantly smaller in a TNP-treated group than those of a control. Survival time was significantly longer in a TNP-treated group. TNP-470 demonstrated no growth inhibition of MKN45 cells in vitro. CONCLUSIONS: Those results suggested that anti-angiogenic agent, TNP-470, might be effective in treating peritoneal dissemination of gastric cancer by inhibiting growth of the seeded tumor cells on the peritoneum.

Animals↗

PubMedDate not supplied

Clinicopathologic differences between early gastric remnant cancer and early primary gastric cancer in the upper third of the stomach.

BACKGROUND/AIMS: This study was designed to clarify the clinicopathologic characteristics and survival in early gastric remnant cancer and compare with early primary cancer in the upper third of the stomach. METHODOLOGY: Twenty-five patients with early gastric remnant cancer, who underwent resection at Kanagawa Cancer Center and First Department of Surgery, Yokohama City University between 1974 and 1996 were evaluated in this study. Various clinicopathologic characteristics, such as age, sex, symptoms, size of tumor, depth of invasion, lymph node metastasis, cell differentiation, and survival were investigated and early gastric remnant cancer was compared with early primary cancer in the upper third of the stomach. RESULTS: According to the macroscopic type, protruded type such as I or II type accounted for a great majority in early gastric remnant cancer, while II c depressed type was common in early primary cancer in the upper third of the stomach, comprising 64.2% of all cases. Pathological examination disclosed that well-differentiated carcinoma and mucosal carcinoma were more frequently observed in early gastric remnant cancer than in early primary cancer in the upper-third of the stomach. The 5-year survival rate was 83.5% for early primary cancer in the upper-third of the stomach. In contrast, no patients experienced recurrence after operation for early gastric remnant cancer. CONCLUSIONS: From the view point of clinicopathological evaluation, gastric remnant cancer is a special from of gastric cancer. A follow-up program is important in order to detect early gastric remnant cancer. A low incidence of lymph node metastasis suggests that endoscopic mucosal resection of the tumor or limited operation could be performed under strict indication.

Aged↗

PubMedDate not supplied

Neoadjuvant chemotherapy with a combination of irinotecan and cisplatin in advanced gastric cancer--a case report.

We report a case of advanced gastric carcinoma successfully treated with a combination of irinotecan and cisplatin as neoadjuvant chemotherapy. The patient, a 78-year-old man, had type 2 gastric cancer, which had metastasized to the paraaortic lymph nodes. He was treated with irinotecan, 70 mg on day 1 and day 15, and cisplatin, 80 mg on day 1. The course was repeated every 4 weeks. Two courses of treatment resulted in a marked reduction of both the primary tumor and lymph nodes. Subsequently, the patient underwent curative surgery consisting of total gastrectomy, splenectomy, and D3 lymph node dissection. No surgical complications were observed. On microscopic examination, only a few tumor cells were detected in the granulation tissues of the resected stomach and in the lymph nodes. This would be the first case to demonstrate the effectiveness and the safety of irinotecan and cisplatin used in the neoadjuvant setting for treatment of advanced gastric carcinoma.

Aged↗

PubMedDate not supplied

Should scirrhous gastric carcinoma be treated surgically? Clinical experiences with 233 cases and a retrospective analysis of prognosticators.

BACKGROUND/AIMS: The prognosis of patients with scirrhous gastric carcinoma has been poorest. METHODOLOGY: To clarify the role of surgical treatment, 233 patients with a primary scirrhous gastric carcinoma were retrospectively analyzed. RESULTS: Of the 233 patients, 182 underwent surgical resection, while the other 51 did not. The median survival time of those with unresectable tumors was 88.0 +/- 15.3 days and that of those who underwent resection was 380.0 +/- 41.8 days. In the 182 patients who underwent resection, multivariate analysis revealed four significant factors; lymphatic invasion, serosal invasion, curability, and lymph node dissection. Of these, curability was the most significant. The median survival time of patients whose tumor were curatively resected was 727.0 +/- 116.3 days, significantly longer than 272 +/- 34.9 days for those whose resection ended noncuratively. In 65 patients whose tumor was curatively resected, subset analyses of factors by multivariate analyses revealed an absence of serosal invasion as the single significant prognosticator. The 5-year survival rate was 55.6% in patients with scirrhous cancer without serosal invasion. CONCLUSIONS: For patients with scirrhous gastric carcinoma, palliative resection should not be attempted for poor outcome. However, if curative resection seems feasible, radical surgery would be justified, especially for tumors without serosal exposure.

Adenocarcinoma, Scirrhous↗

PubMedDate not supplied

Clinicopathologic differences between gastric remnant cancer and primary cancer in the upper third of the stomach.

BACKGROUND: The prognosis of gastric remnant cancer is considered to be poor compared with that of primary cancer. MATERIALS AND METHODS: This study was conducted to elucidate the differences of clinicopathologic findings and treatment results between 59 patients with gastric remnant cancer (GRC) and 579 patients with primary proximal gastric cancer (PGC). RESULTS: There was no significant difference in the incidence of lymph node metastasis between GRC and PGC. However, changes in the metastatic pattern to lymph nodes were observed in GRC. In GRC, the tumor easily invaded the neighboring organs due to the adhesions around the remnant stomach, resulting in a low resectability with curative intent. The 5-year survival rate after curative resection for advanced GRC was 50.9%. GRC patients without serosal invasion had a good prognosis. CONCLUSION: Although the survival rate after curative resection for GRC patients was similar to that of PGC, GRC patients without serosal invasion had a better prognosis. Therefore, early detection is an important way to improve overall survival in GRC.

Adult↗