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At least 19 recordsLinked to original sources

Clinicopathological evidence of pseudomyxoma peritonei in a dog with intestinal mucinous adenocarcinoma.

An 11-year-old, intact female Pekingese dog was presented because of chronic vomiting. Clinical examination and diagnostic imaging suggested the presence of an intestinal mass. Laparotomy was performed, and a gelatinous effusion associated with a suspected jejunal neoplasm was found. Accumulation of gelatinous material was grossly evident in several abdominal organs and parietal peritoneum. Cytopathological smears from this material showed macrophages, reactive mesothelial cells, and spindle cells embedded in a mucinous basophilic background. After spontaneous death, necropsy and histopathology were performed and showed the presence of an invasive mucinous adenocarcinoma with visceral and peritoneal metastases. The clinicopathological findings of this case report closely resemble those reported in human cases of pseudomyxoma peritonei.

Adenocarcinoma, Mucinous↗

Duodeno-jejunal tumors. Report of 9 cases and literature review.

Nine patients with tumors of the duodenum and the jejunum are described herein and the Literature is reviewed. Of the six patients with a duodenal tumor, five had an adenocarcinoma and one a Brunner's gland adenoma. A predominance of inframpullary tumors was observed within the duodenum. Jaundice and abdominal pain were, respectively, the most common presenting symptoms of the tumors localized in the periampullary and inframpullary region. Treatment was curative in four and palliative in two cases. Duodenopancreatectomy was the treatment of choice for periampullary tumors whereas segmental resection was performed in the only resectable distal duodenal tumor. Of the three patients with jejunal neoplasms, one had an adenocarcinoma arising in the efferent loop of a Billroth II gastrojejunostomy performed 40 years before and two had an high malignant lymphoma. All three the tumors could be resected. According to the Literature, our results show that: 1. The diagnosis of duodeno-jejunal tumors is usually late: 2. Although of critical importance in the improvement of the overall diagnostic accuracy, endoscopy may be inconclusive or even misleading if the entire duodenum is not explored; 3. If duodenopancreatectomy is mandatory for periampullary tumors, segmental resection seems to be an adequate procedure for tumors of the distal duodenum since it does not ignore lymphatic nodes, can be easily performed and has a low postoperative complication rate.

Aged↗

Carcinoid tumors.

A retrospective study of 86 patients with carcinoid tumors was undertaken covering an 18-year period. The most common sites of tumor were the jejunoileum (28 cases), appendix (22), and rectum (16). These were followed by the bronchus, duodenum, colon, and stomach. Three tumors had such wide-spread metastasis that their primary sites of origin could not be determined. The appendiceal and rectal carcinoids were often benign and usually found as incidental tumors, whereas colon and jejunoileal carcinoids were often metastatic. The jejunoileal carcinoids were commonly associated with multiple tumor sites and had a high frequency of secondary neoplasms. Symptomatic tumors were often metastatic. Chronic intermittent intestinal obstruction was often present with mesenteric metastasis, and liver metastasis was associated with the carcinoid syndrome. The patients' survival was good if aggressive surgical therapy was undertaken for palliation of the tumors.

Adolescent↗

Mutations of the Ki-ras, p53 and APC genes in adenocarcinomas of the human small intestine.

In contrast to the origins of colorectal carcinomas, the mechanisms of carcinogenesis in the small intestine remain unclear. We therefore analyzed the mutational status of the Ki-ras, p53, and adenomatous polyposis coli (APC) genes in primary carcinomas of the small intestine and compared the mutation patterns with those established for colorectal cancers. DNA was extracted from 15 formalin-fixed, paraffin-embedded lesions. Codons 12, 13 and 61 of the Ki-ras gene, exons 5-8 of the p53 gene, and codons 1268-1569, which contain the mutation cluster region (MCR) of the APC gene, were amplified by means of PCR, subcloned and sequenced. Mutations of the Ki-ras and p53 genes were observed in 8 (53.3%) and 4 lesions (26.7%), respectively. The mutational frequency of the Ki-ras gene in the present series of small intestinal carcinomas was similar, while that of the p53 gene was slightly lower than the reported frequencies for colorectal carcinomas. Only one case showed a mutation of the APC gene, involving an insertional mutation of an adenine at codons 1554-1556 with formation of a stop codon immediately downstream. Since the occurrence of an APC mutation is considered an early event in colorectal carcinogenesis, our findings indicating an extremely low frequency of such changes in and around the MCR suggest that carcinomas of the small intestine arise via a genetic pathway distinct from that involved in the development of carcinomas of the colorectum.

Adult↗

Small intestinal adenocarcinoma complicating regional enteritis.

Three new cases of small intestinal adenocarcinoma complicating Crohn's disease are reported. Seventy-five other cases have been reported to date. Analysis of these cases in comparison with de novo adenocarcinoma of the small bowel shows that they: (1) occur at a younger age; (2) occur in distal small bowel; (3) show a male predilection; and (4) have a worse prognosis. The increasing number of these cases suggests that small intestinal regional enteritis predisposes to adenocarcinoma. Improved prognosis can only be achieved with earlier diagnosis.

Adenocarcinoma↗

Adenocarcinoma of the small bowel in lynch syndrome II.

Adenocarcinoma of the small bowel is rare and accounts for about 1% of all gastrointestinal tract cancer. This disorder has been identified in association with Crohn's disease, celiac disease, Peutz-Jegher's syndrome, and familial adenomatous polyposis. We report adenocarcinoma of the small bowel in nine patients from eight Lynch syndrome II extended pedigrees. Each affected patient was in the direct genetic lineage or manifested multiple primary cancers (stomach, colon, endometrium, and ovary) consonant with the tumor spectrum of Lynch syndrome II. The average age of onset for small bowel cancer was 47 years (range 31 to 56 years), versus the general population peak occurrence after the sixth decade. We conclude that small bowel cancer may be an integral component of the tumor spectrum of Lynch syndrome II.

Adenocarcinoma↗

Tumours of the small intestine.

Tumours of the small intestine are a relatively unusual group of benign and malignant tumours of both epithelial and supporting tissue origin. Their pathology, associated syndromes, clinical features, diagnosis, and management are discussed.

Adenocarcinoma↗

Evaluation of gastrointestinal hemorrhage in patients with neurofibromatosis.

Over a 3-year period, two patients with neurofibromatosis were referred to our medical center for evaluation of repeated episodes of melena. Upper endoscopy was unrevealing in each case, as was colonoscopy. Arteriography during active hemorrhage was helpful in localizing the source of bleeding in one patient but not in the other. The source of bleeding in each patient was obvious at surgical exploration. Large neurofibromas protruded from the serosal surface of a short region of jejunum in both cases and an additional segment of ileum in one case. Hemorrhage had occurred as a result of erosion of mucosa stretched over these tumors. Local resection of the involved segments produced long-term control of the hemorrhage. Since these tumors were grossly visible on the serosal surface of the involved intestinal segments, laparoscopic evaluation could have been used to hasten diagnosis in each case.

Adult↗

Dedifferentiated leiomyosarcoma of the intestinal tract: histological, ultrastructural and immunohistochemical examinations.

Six cases of dedifferentiated leiomyosarcoma of the small and large bowel are presented with histological, ultrastructural and immunohistochemical examination. One case arose in the jejunum, two in the ileum, and the other three in the large intestine. The tumours were submucosal in four cases with large areas of ulceration; two were polypoid. Four tumours showed typical leiomyosarcomatous appearance with dedifferentiated components and two were typical leiomyosarcomas at the primary site with differentiated components only in metastatic foci. By immunohistochemistry, typical leiomyosarcomatous areas showed a positive reaction for muscle-specific actin (MSA), MB1, MB2 and myosin. In contrast, desmin-positive cells were scattered throughout the tumour or were not present. Tumour cells in dedifferentiated components were positive for alpha-1-antitrypsin and alpha-1-antichymotrypsin in all cases but one; neuron specific enolase, MB1, MB2 and myosin were positive with variety. MSA was faintly positive in only a few tumour cells of two cases and desmin was not detected in any of the cases studied. Ultrastructurally, tumour cells in typical leiomyosarcomatous areas demonstrated evident smooth muscle features, although in dedifferentiated areas they lacked such features except in one case. Our results indicate that dedifferentiated elements may derive from ordinary leiomyosarcoma and loose muscle features due to dedifferentiation.

Aged↗

Primary jejunoileal tumors: a review of 45 cases.

We reviewed 45 patients who underwent surgery for primary jejunoileal tumors over a 15-year period. There were 16 benign and 29 malignant tumors, which included 13 lymphomas, 7 adenocarcinomas, 7 carcinoid tumors, and 2 leiomyosarcomas. Eighteen patients, 13 of whom had benign tumors, presented with intestinal bleeding and 5 tumors were found incidentally at laparotomy. Benign lesions were more frequently sited in the jejunum while malignant lesions were more common in the ileum (p less than 0.001). Lesions presenting with hemorrhage were more likely to be benign than malignant (p less than 0.001) and were more commonly sited in the jejunum than in the ileum (p less than 0.05). Visceral perforation (31%), intestinal obstruction (21%), and an abdominal mass (17%) were other presenting features in patients with malignant tumors. In spite of a wide variety of investigations, the correct diagnosis was reached preoperatively in only 31% of patients. Surgical management included either limited bowel resection or segmental resection with regional lymphadenectomy. Operative mortality was 13% and morbidity was 36%. Actuarial 5-year survival for all malignant tumors was 24%, being 64% at 5 years for carcinoid tumors, 20% at 30 months for adenocarcinomas, and 10% at 42 months for lymphomas. These results reemphasize the need for a high index of suspicion and early laparotomy in patients with obscure intestinal symptoms if the prognosis of small bowel tumors is to improve.

Adult↗

Radiology in primary small bowel adenocarcinoma.

In a retrospective study, films from radiologic examinations of 13 patients with primary adenocarcinoma of the small bowel were reviewed. All patients had a barium examination and in 5 cases angiography was also performed. The barium studies disclosed strictures with overhanging edges in 11 patients, in 4 of whom there was a pronounced prestenotic dilation. Angiographically the predominant findings were in all cases hypovascularity and displacement of vessels. In 4 patients there was also encasement. Neovascularity was present in 2 patients. One patient showed angiographic changes due to intussusception.

Adenocarcinoma↗

Angiographic demonstration of gastrointestinal neurofibromas in von Recklinghausen's disease.

Two cases of intestinal neurofibromas which were demonstrated during mesenteric arteriography are described. In 1 patient, tumors located in the jejunum and distal ileum were the apparent source of gastrointestinal bleeding. In another case, a neurofibroma of the proximal jejunum was an incidental finding during evaluation for severe pancreatitis and bleeding from esophageal varices.

Adult↗

Crescent-shaped necrosis: a new imaging sign suggestive of stromal tumor of the small bowel.

We report three cases of unsuspected stromal tumors of the small bowel (STSB). These cases were diagnosed preoperatively by sonography, the imaging was completed for some with barium studies, computed tomography, magnetic resonance imaging, or angiography. To our knowledge, the use of this noninvasive technique for detecting this type of tumor has not been described in the literature. Moreover, we believe that some sonographic findings such as crescentic tumoral necrosis may appear highly suggestive of STSB.

Adult↗

131I-meta-iodobenzylguanidine in the management of metastatic midgut carcinoid tumors.

The management of metastatic neuroendocrine tumors incorporates multimodal therapy with surgery, biotherapy, and chemotherapy. Tumor-targeted therapies using radiolabeled octreotide and metaiodobenzylguanidine (mIBG) represent a novel treatment approach. The aim of this study was to evaluate the effectiveness of 131I-mIBG in the treatment of metastatic midgut carcinoid tumors. survival outcomes were assessed for patients treated at two regional cancer centers and then compared. One center used 131I-mIBG routinely in the management of metastatic carcinoid tumors (center A), and the other did not use this modality (center B). Only patients with histologically proven metastatic carcinoid tumor shown, or thought most likely, to be of midgut origin were included in the study. During the period 1980 to 2002, a series of 58 patients from center A with metastatic carcinoid tumor arising from the midgut underwent multimodality therapy with the addition of 131I-mIBG. Their median age was 64 years. The median dose of 131I-mIBG administered was 6751 MBq, and there was an average of 2.8 treatments per patient. During the same period, 58 patients with metastatic carcinoid were treated at center B with similar multimodality therapy without the use of 131I-mIBG therapy. Their median age was 65 years. Survivals at 3 and 5 years were 77% and 63%, respectively (95% CI 47-75), for group A. The 3- and 5-year survivals for group B were 56% and 47% (95% CI 34-59), respectively. The mean follow-up was 6.6 years for group A and 5.0 years for group B. Although retrospective in nature, this study suggests that the addition of 131I-mIBG therapy to the treatment protocol of patients with metastatic midgut carcinoid tumors prolongs survival.

3-Iodobenzylguanidine↗

Long-term survival of patients with small intestinal carcinoid tumors.

Midgut carcinoid tumors are rare and have a markedly better prognosis than adenocarcinoma in the small intestine. New diagnostic methods and medical as well as surgical therapies have evolved during the last decades, leading to more active care of these patients. Patients with small intestinal carcinoids diagnosed from 1960 to 2000 in the duodenum (n = 89) and jejunum/ileum (n = 2437) were identified in the Swedish Cancer Registry. Cases without histologic verification and autopsy cases were excluded. Overall, cause-specific and relative survival were calculated. The overall 5-, 10-, and 15 year survivals were, respectively, 60%, 46%, and 28% for duodenal tumors and 56%, 36%, and 23% for jejunal/ileal tumors. Cause-specific 5-, 10-, and 15-year survival was 94% for all three follow-up periods for duodenal tumors and 87%, 80%, and 77% for those in the jejunum/ileum. The corresponding relative survivals were, respectively, 72%, 67%, and 51% for duodenal tumors and 67%, 54%, and 44% for those in the jejunum/ileum. Sex did not influence overall or cause-specific survival. The age at diagnosis correlated inversely with overall and cause-specific survival for tumors in the jejunum/ileum. For tumors in the jejunum/ileum, the overall and cause-specific survival correlated with the time period of diagnosis, with a more favorable prognosis for those diagnosed in recent years. A multivariate Cox proportional hazards model showed similar results. We concluded that young age and diagnosis in recent years are positive predictors of survival for patients with midgut carcinoids. The divergence between cause-specific and relative survival implies the need for a more detailed analysis of the causes of death of these patients.

Adult↗

[Morphology, DNA cytophotometry and prognosis in gastrointestinal neuroendocrine tumours (carcinoids). A clinico-pathological study of 95 patients].

A total of 123 manifestations (97 primary tumours and 26 metastases) of neuroendocrine tumours of the gastrointestinal tract observed in 95 patients was investigated for the prognostic value of clinical, histological and DNA cytophotometric parameters. Metastases almost exclusively occurred among ileal carcinoids, which also were responsible for all 14 cases of lethal outcome observed during the follow-up period of mean 42 months. Aneuploid DNA values could be determined significantly more frequently among ileal than in non-ileal carcinoids and showed - upon analysis of the total group of gastrointestinal neuroendocrine tumours - a significant correlation to lethal course of disease. In addition, among 18 cases with primary and secondary carcinoid manifestations available for DNA cytophotometry, an association between the DNA content of metastatic neuroendocrine tumours and prognosis came to light. When applied to the group of ileal neoplasms, however, the parameter DNA content did not allow a better prognostic assessment.

Adult↗