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Multicentric primary adenocarcinomas of the midgut: the first case report.

Multicentric adenocarcinomas of the midgut have not been described; even multiple adenocarcinomas limited to the small intestine are extremely uncommon, with only 14 cases reported in the literature. We report a case of multicentric synchronous involvement of the entire midgut with adenocarcinoma in a 52-yr-old Polish woman who had more than 30 lesions extending from duodenum to mid-transverse colon. There was no family history of cancer. Preoperative evaluation and intraoperative exploration were negative for primary malignancy of the lungs, breasts, ovaries, pancreas, and other parts of the gastrointestinal tract. Results of histopathological examination, immunohistochemical staining, and ras mutational analysis of the lesions uniformly support the diagnosis of multicentric poorly differentiated adenocarcinoma. The cause for this unusual presentation is unknown, although sporadic genetic alteration(s) of oncogene(s) might have been the precipitating event.

Adenocarcinoma↗

[Intestinal obstruction caused by transitional carcinoma].

OBJECTIVES: To draw attention to the possibility that distant intestinal metastasis can arise from transitional urothelial carcinoma located in the bladder. METHODS: Two female patients developed distant metastasis during treatment of the tumor by transurethral resection of the bladder. RESULTS: Following complicated transurethral resection with perforation of the peritoneal cavity, the patients developed intestinal obstruction from metastatic transitional cell carcinoma. CONCLUSIONS: Direct or distant seeding of tumor cells on previously undamaged peritoneal serosa produced with the resectoscope may not be exceedingly unlikely.

Aged↗

[The pathophysiological significance of the low triiodothyronine syndrome with increased clearance in cancer of the stomach and large intestine].

Investigations designed to study T3 pharmacokinetics under malignant tumors of the stomach and large intestine showed an augmented total T3 clearance, which fact is indicative of enhancement of the processes of 5-monodeiodezing of thyroid hormones. It is to be thought that intensification of T3 metabolism and rise in inverse T3 produced through peripheral conversion of T4 underlie the pathogenesis of that low triiodothyronine syndrome to be encountered in malignant tumors. Since the above mechanism of development of low T3 syndrome has been identified in traumata and infectious diseases, the changes in question might be considered to be one of the links in the general adaptive syndrome directed towards satisfaction of the phagocytosing leucocytes' requirements in the biocydic substrate iodine and augmentation of the production of the fat-mobilizing factor inverse T3.

Adult↗

[The correction of the thyroid status in the surgical treatment of malignant tumors of the stomach and large intestine].

We evaluated changes in thyroid status of patients with carcinoma of the stomach and large intestine in pre- and postoperative periods under the influence of triiodothyronine (T3) prescribed to make up for deficient endogenous hormone. With T3 returning to normal in preoperative period, T4 blood level gets lowered from the upper limit of the norm to the lower one. However these patients show unchanged blood T4 concentration after surgical intervention, while without administration of T3 the level of T4 decreases. Prescription of T3 promotes greater depression of thyroid-stimulating hormone in pre- and postoperative periods. Incorporation of T3 into conventional corrective therapy of metabolism tends to improve the immediate results of surgical treatment of malignant tumours of the stomach and large intestine.

Adenocarcinoma↗

[A fatal case of aggressive-phase multiple myeloma with ileus and invasion into extramedullary organs].

A 62-year-old woman with IgA-lambda type monoclonal gammopathy had been followed up since January 1988. In March 1991, multiple myeloma (IgA-lambda) was diagnosed on the basis of bone marrow biopsy findings and increased serum IgA levels. She was treated intermittently with melphalan and prednisolone over a perioa of about 6 years, but was eventually admitted due to renal dysfunction, hypercalcemia, increased serum IgA and the formation of subcutaneous masses. During chemotherapy she underwent emergency surgery for obturative ileus. Histological examination of the resected tissues revealed invasion of myeloma cells into the small intestine and peritoneum. Despite continued chemotherapy, the patient's soft tissue masses enlarged, and new lesions appeared in other organs. In the terminal stage, lower serum IgA levels were observed despite an increase in Bence-Jones protein levels in urine. The patient died five months after admission. An autopsy found infiltration by atypical myeloma cells in multiple organs. An immunohistochemical examination revealed and increase in lambda-light chain positive cells relative to the number of alpha-heavy chain positive cells. The terminal course was considered to be representative of aggressive phase multiple myeloma. The case was rare in that the patient's ileus was caused by invasion of myeloma cells into the small intestine.

Disease Progression↗

Perforation through undiagnosed small bowel involvement in primary thyroid lymphoma during chemotherapy.

A case is described of fatal perforation of the small bowel through an area of undiagnosed secondary involvement from primary thyroid lymphoma during treatment by chemotherapy. There is a known association between primary thyroid lymphoma and gastrointestinal metastases. To avoid this lethal complication, a specific search should be made for gastrointestinal involvement before chemotherapy is started in patients with advanced thyroid lymphoma.

Aged↗

Spread of disease via the subperitoneal space: the small bowel mesentery.

The designation of the subperitoneal space emphasizes the continuum of the potential space of extraperitoneal and intraperitoneal areolar tissue traversed by blood vessels, lymphatics, and nerves. Across its root, the subserous connective tissue of the small bowel mesentery is anatomically continuous with that deep to the posterior parietal peritoneum. There is thereby provided an avenue of spread from multiple sites to and from the small bowel mesentery and its relationships. These include perforated lesions of the bowel, pancreatitis, lymphoma, neuroblastoma, leiomyosarcoma of small bowel, and hemorrhage of retroperitoneal and pelvic origin.

Female↗

Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database.

BACKGROUND: Malignant gastrointestinal stromal tumors (M-GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal (GI) tract. Previous studies have included limited numbers of patients, and most included malignant and benign cases from throughout the GI tract. We reviewed the experience of a single tertiary cancer care center with M-GIST of the small intestine only. METHODS: A prospective database identified all patients seen from 1989 to 1998. Clinical and pathological data, treatment, and outcome were analyzed. Overall median follow-up time was 24 months (range, 1-176 months). RESULTS: Fifty patients (31 male, 19 female) were identified. Mean age at diagnosis was 55 years. Disease was localized in 11 patients, locally advanced (invasion into adjacent organs/peritoneum) in 24 patients, perforated in 4 patients, multiple primary lesions in 2 patients, and distant metastases in 9 patients. All patients underwent resection, which was complete in 70%. Locoregional recurrence (LR) developed in 43% (median, 25 months), and distant metastases in 59% (median, 21 months) of patients at risk. At last follow-up, 14 patients were alive (6 disease-free), 2 had died disease-free, and 34 died with recurrent disease. Overall survival (OS) was similar for localized and locally advanced disease; OS also was similar for patients with multiple primaries and distant metastases at diagnosis. Patients were grouped into three stages: (I) patients with localized and locally advanced disease; (II) patients with perforated; and (III) patients with multiple primaries and distant metastases. Actuarial OS at 5 years was 41% (n = 50)--42% for those with complete resection and 8% for incomplete resection. Univariable analysis showed that earlier stage at diagnosis (P = .001) and completeness of resection (P = .004) predicted for longer OS. CONCLUSIONS: Most patients with M-GIST of the small intestine relapse following resection, but survival may be prolonged. In univariable analysis, stage at presentation and complete resection were significant prognostic variables for OS; grade was not significant. Localized and locally advanced M-GIST of the small intestine have a mean OS > 5 years. Complete resection should be the goal of initial surgical treatment.

Adult↗

Small bowel sarcoma: analysis of survival from the National Cancer Data Base.

BACKGROUND: Small bowel sarcomas (SBS) are rare, accounting for 10% of small bowel cancers. As a result, few studies of SBS have had enough patients to accurately define their natural history and to determine the factors that have an impact on patient survival. The objective of this study was to examine patient and tumor factors in SBS and to determine prognostic factors for disease-specific survival (DSS) using the National Cancer Data Base. METHODS: Data from the National Cancer Data Base for patients diagnosed with primary SBS between 1985 and 1995 were analyzed. The chi2 statistic was used to determine significant differences between groups of patient, tumor, and treatment factors. DSS was calculated for patients diagnosed between 1985 and 1990. Significant differences in survival were determined using the Wilcoxon statistic for univariate analyses and by Cox regression in multivariate analyses. RESULTS: Of 14,253 small bowel tumors diagnosed between 1985 and 1995, sarcomas represented 10.1%. Overall, 5-year DSS was 38.9%, with a median survival of 34.1 months (n = 590). By univariate analysis, patient age, sex, tumor size, tumor grade, histologic type, general summary stage, nodal status, and whether cancer-directed surgery was performed were significantly correlated with DSS. In multivariate analysis, tumor size <5 cm, leiomyosarcoma histology, and localized disease were found to be significant favorable prognostic factors for DSS. CONCLUSIONS: SBS are rare tumors that are challenging in terms of their histopathologic classification, grading, and staging. Patients with SBS were treated predominantly by surgery, with a minority receiving adjuvant therapy. Tumor size, histologic type, and general summary stage were independent prognostic factors for 5-year DSS in patients with SBS, which is improved relative to 5-year DSS seen in patients with small bowel adenocarcinoma.

Adult↗

Development of spontaneous tumours and intestinal lesions in Fhit gene knockout mice.

The fragile histidine triad (FHIT) gene is frequently inactivated in various types of tumours. However, the system-wide pathology caused by FHIT inactivation has not been examined in detail. Here we demonstrate that Fhit gene knockout mice develop tumours in the lymphoid tissue, liver, uterus, testis, forestomach and small intestine, together with structural abnormalities in the small intestinal mucosa. These results suggest that Fhit plays important roles in systemic tumour suppression and in the integrity of mucosal structure of the intestines.

Acid Anhydride Hydrolases↗

Transgenic mouse models that explore the multistep hypothesis of intestinal neoplasia.

SV-40 T antigen (TAg), human K-rasVal12, and a dominant negative mutant of human p53 (p53Ala143) have been expressed singly and in all possible combinations in postmitotic enterocytes distributed throughout the duodenal-colonic axis of 1-12-mo-old FVB/N transgenic mice to assess the susceptibility of this lineage to gene products implicated in the pathogenesis of human gut neoplasia. SV-40 TAg produces re-entry into the cell cycle. Transgenic pedigrees that produce K-rasVal12 alone, p53Ala143 alone, or K-rasVal12 and p53Ala143 have no detectable phenotypic abnormalities. However, K-rasVal12 cooperates with SV-40 TAg to generate marked proliferative and dysplastic changes in the intestinal epithelium. These abnormalities do not progress to form adenomas or adenocarcinomas over a 9-12-mo period despite sustained expression of the transgenes. Addition of p53Ala143 to enterocytes that synthesize SV-40 TAg and K-rasVal12 does not produce any further changes in proliferation or differentiation. Mice that carry one, two, or three of these transgenes were crossed to animals that carry Min, a fully penetrant, dominant mutation of the Apc gene associated with the development of multiple small intestinal and colonic adenomas. A modest (2-5-fold) increase in tumor number was noted in animals which express SV-40 TAg alone, SV-40 TAg and K-rasVal12, or SV-40 TAg, K-rasVal12 and p53Ala143. However, the histopathologic features of the adenomas were not altered and the gut epithelium located between tumors appeared similar to the epithelium of their single transgenic, bi-transgenic, or tri-transgenic parents without Min. These results suggest that (a) the failure of the dysplastic gut epithelium of SV-40 TAg X K-rasVal12 mice to undergo further progression to adenomas or adenocarcinomas is due to the remarkable protective effect of a continuously and rapidly renewing epithelium, (b) initiation of tumorigenesis in Min mice typically occurs in crypts rather than in villus-associated epithelial cell populations, and (c) transgenic mouse models of neoplasia involving members of the enterocytic lineage may require that gene products implicated in tumorigenesis be directed to crypt stem cells or their immediate descendants. Nonetheless, directing K-rasVal12 production to proliferating and nonproliferating cells in the lower and upper half of small intestinal and colonic crypts does not result in any detectable abnormalities.

Analysis of Variance↗