1/34. Primary linitis plastica of the rectosigmoid in a thirteen year old boy.Childhood malignant neoplasms of the gut are extremely rare. The reported incidence of colorectal cancers in patients under 20 years of age is 1 in 10 million. The low index of suspicion for this tumour in children results in advanced disease at diagnosis and subsequently a poor prognosis. A rare case of a primary linitis plastica of the rectum occurring in a 13 year old boy is reported with review of pertinent literature.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/34. Evidence for a recessive inheritance of Turcot's syndrome caused by compound heterozygous mutations within the PMS2 gene.Turcot's syndrome is a genetic disease characterized by the concurrence of primary brain tumors and colon cancers and/or multiple colorectal adenomas. We report a Turcot family with no parental consanguinity, in which two affected sisters, with no history of tumors in their parents, died of a brain tumor and of a colorectal tumor, respectively, at a very early age. The proband had a severe microsatellite instability (MIN) phenotype in both tumor and normal colon mucosa, and mutations in the TGFbeta-RII and APC genes in the colorectal tumor. We identified two germline mutations within the PMS2 gene: a G deletion (1221delG) in exon 11 and a four-base-pair deletion (2361delCTTC) in exon 14, both of which were inherited from the patient's unaffected parents. These results represent the first evidence that two germline frameshift mutations in PMS2, an MMR gene which is only rarely involved in HNPCC, are not pathogenic per se, but become so when occurring together in a compound heterozygote. The compound heterozygosity for two mutations in the PMS2 gene has implications for the role of protein PMS2 in the mismatch repair mechanism, as well as for the presymptomatic molecular diagnosis of at-risk family members. Furthermore, our data support and enlarge the notion that high dna instability in normal tissues might trigger the development of cancer in this syndrome.- - - - - - - - - - ranking = 0.011608453460478keywords = brain (Clic here for more details about this article) |
3/34. An unusual cause of thigh pain in colonic cancer. Inflammatory oncotaxis?Colonic carcinoma metastasis in order of frequency to the liver, lung, bone and brain. Metastases elsewhere are unusual. We report a very rare case of metastatic spread to skeletal muscle and skin. There follows a review of the theories of anomalous metastatic localization. Inflammatory oncotaxis is offered as the most likely explanation of this phenomenon. The importance of good history taking in colorectal cancer follow up clinics is emphasised.- - - - - - - - - - ranking = 0.0058042267302391keywords = brain (Clic here for more details about this article) |
4/34. microsatellite instability and expression of hMLH-1 and hMSH-2 in sebaceous gland carcinomas as markers for muir-torre syndrome.Sebaceous gland carcinomas (SGCs) are rare malignant skin tumors occurring sporadically or as a phenotypic feature of the muir-torre syndrome (MTS). A subset of patients with MTS have a variant of the hereditary nonpolyposis colorectal cancer syndrome caused by mutations in mismatch repair (MMR) genes, which lead to microsatellite instability (MSI). We evaluated the value of MSI and loss of expression of the MMR genes, hMLH-1 and hMSH-2, as a marker to identify and distinguish MTS from sporadic SGC. Using a nationwide pathology report database system, we identified patients with the MTS phenotype. SGCs from 10 MTS patients and the colorectal carcinomas from 3 additional MTS patients were collected. In addition, SGCs from eight patients without a history of visceral neoplasm were collected. MSI was detected in 9 of 13 MTS-associated tumors (69%) versus 0 of 8 sporadic SGCs (P = 0.002). Except for the age of onset of colorectal carcinoma [58 years in the MSI-positive group versus 69.8 years in the MSI-negative group (P = 0.17)], no differences were seen between the MSI-negative and the MSI-positive MTS patients. Loss of expression of hMLH-1 (n = 4) or hMSH-2 (n = 4) was found in MSI-positive patients only. MSI and loss of expression of MMR genes can be used as markers for MTS in patients with SGC. Consequently, MSI and loss of MMR gene expression in a patient presenting with SGC as the initial malignancy have important consequences for the patient and family. There are at least two variants of MTS with different molecular genetic mechanisms because 31% of the patients with the MTS phenotype had no MSI.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/34. Colorectal adenocarcinoma as a second malignant neoplasm following rhabdomyosarcoma of the urinary bladder: a case report.Following improvements in therapy for childhood malignancies, the striking increase in survival rate over the past 30 years has led to the increase risk of developing second malignant neoplasms (SMNs). We report a case of colorectal carcinoma as a SMN, following treatment for rhabdomyosarcoma. The patient was diagnosed with rhabdomyosarcoma of the urinary bladder at his age of three years, and developed adenocarcinoma in the colon 13 years later. Histologic examination of the surgical specimen revealed adenocarcinoma involving the rectosigmoid area with radiation colitis in its background. The tumor cells showed strong immunoreactivity for p53 protein, suggesting the role of irradiation and p53 mutation in carcinogenesis. This case emphasizes the need for dose observation in survivors of early childhood malignancies treated with radiation and multiagent chemotherapy.- - - - - - - - - - ranking = 5keywords = neoplasm (Clic here for more details about this article) |
6/34. Histopathology of early colorectal cancer.The histopathologic spectrum of early colorectal cancer (ECC) is compared from the Western and Japanese perspective. In the West, most ECC presents as a malignant adenoma, whereas superficial ECC with no adenomatous component is well described in the Japanese literature. Furthermore, superficial ECC is thought to progress rapidly and disseminate early. The discrepant viewpoints are considered in the light of different approaches to histopathologic interpretation. Lesions reported by Japanese pathologists as superficial carcinomas limited to the mucosa are diagnosed as flat adenomas in the West. In one Japanese series only 4 of 218 (1.8%) flat or small sessile neoplasms removed by endoscopic mucosal resection were associated with submucosal invasion by carcinoma. Therefore superficial ECC with submucosal spread is uncommon. Most ECC in hereditary nonpolyposis colorectal cancer evolves through the adenoma-carcinoma pathway (including flat adenoma). However, adenoma removal in the context of population-based screening programs has not lowered the incidence of colorectal cancer; flat colorectal neoplasia may be important in clinical practice.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/34. brain metastasis: first sign of colorectal carcinoma.The purpose of this study was to describe the detection of a brain metastasis as the first sign of colorectal carcinoma, without any liver and/or lung involvement. This finding demonstrates primarily the need for regular central nervous system investigation in diagnosing colorectal cancer and secondly that further, more thorough research into the biomolecular events involved in metastases from colorectal cancer is warranted.- - - - - - - - - - ranking = 0.0058042267302391keywords = brain (Clic here for more details about this article) |
8/34. Basaloid carcinoma of the colon arising at the splenic flexure.AIMS: Basaloid carcinomas typically arise in the anal canal and there are only three well-documented cases of this neoplasm reported outside the anal canal, none more proximally than the sigmoid colon. The first occurrence of a basaloid colonic carcinoma arising outside the sigmoid colon, at the splenic flexure, is presented. methods AND RESULTS: A splenic flexure mass was resected from a 54-year-old man with a 3-week history of abdominal discomfort, diarrhoea and weight loss. This tumour, like typical anal canal basaloid carcinomas, was composed of islands of basaloid cells with peripheral nuclear palisading; within many islands there was central necrosis and focal squamous differentiation. Ultrastructural and immunohistochemical studies confirmed the basaloid nature and focal squamous differentiation within this neoplasm. Basaloid carcinoma of the anal canal has been associated with human papilloma virus. Using in-situ hybridization, HPV dna was not detected in this case. CONCLUSIONS: Outside the anal canal, it has been postulated that basaloid colonic carcinomas may arise from cloacogenic embryologic rests, squamous metaplastic epithelium, or totipotential basal cells. The location and pathological findings of this tumour suggest that this rare colonic neoplasm arises from a totipotential basal cell.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
9/34. Adenosquamous carcinoma of the colorectum: report of two cases.Adenosquamous carcinomas of the colorectum are rare neoplasms. Our experience with two cases is presented in this paper. One patient, who complained of bloody stool, was found to have adenocarcinoma in the sigmoid colon. He received a laparoscopy-assisted sigmoidectomy. The histological examination revealed that the tumor was adenosquamous carcinoma. To date, he has survived six months post operatively without evidence of recurrence. The other patient, who complained of anal bleeding, was found to have rectal adenocarcinoma and received a low anterior resection. Histological examination revealed that the tumor was an adenosquamous carcinoma. He remains alive, with no evidence of recurrence, nine years post operatively. Both cases showed paracolic lymph node metastasis. Because of its very low incidence, the histogenesis, malignancy and prognosis of this disease remain unclear. Thus, further clinical and histological study of this disease entity is required.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/34. Synchronous primary malignant neoplasms of colon and bladder.Extracolonic and synchronous malignancies are rare in colorectal carcinomas. We report a 68-year-old man with complaints of rectal bleeding and hematuria. Endoscopic biopsies revealed synchronous adenocarcinoma of the colon and transitional cell carcinoma of the bladder. The patient was started on chemotherapy, and is alive (with disease) nine months later.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
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