Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Smooth muscle tumors of the alimentary tract are uncommon. A retrospective study was made of 7 patients treated for leiomyosarcoma of the small and large bowel to identify prognostic factors that influence results. The symptoms associated with these tumors varied according to the anatomic sites of the lesions and the position of the growth in relation to the intestinal lumen but the most common presenting clinical signs are bleeding or obstruction. The differential diagnosis between benign and malignant smooth muscle tumors is sometimes quite difficult. Clinical behavior of the myosarcomas of the gastrointestinal tract can be predicted to a large extent by the site of the tumor, the presence or absence of invasion of adjacent vital organs, and the histopathologic grade of malignancy. Although the mitotic activity of a gastrointestinal stromal tumor remains the most critical prognostic factor, tumors have been seen to recur locally and to metastasize even with rare or absent mitotic figures. Further studies are needed to pinpoint the factors that may be correlated to the prognosis.
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PMID:[Leiomyosarcoma of the small intestine and the colorectum. A case contribution]. 908 27

Neoplasms of the small bowel are rare lesions that account for less than 5% of all gastrointestinal tumors. Although the differential diagnosis for a small bowel tumor is extensive, various small bowel neoplasms have characteristic features at computed tomography (CT) that may aid in making a diagnosis. Small bowel adenocarcinoma may appear at CT as an annular lesion, a discrete nodular mass, or an ulcerative lesion. Non-Hodgkin lymphoma may appear as a segmental bulky mass that gradually merges into the normal bowel wall. Lymphoma is characteristically associated with marked luminal dilatation. Carcinoid tumor may appear as an ill-defined homogeneous mass that displaces bowel loops. Calcification and desmoplastic reaction in a mesenteric mass suggest the diagnosis of carcinoid tumor. Gastrointestinal stromal tumors (GISTs), both benign and malignant, may be submucosal, subserosal, or intraluminal. The CT appearance of a GIST may include a sharply defined mass with homogeneous attenuation, sometimes with calcification. Lipoma appears at CT as a well-circumscribed, intraluminal homogeneous mass with fat attenuation. Most malignant small bowel tumors are actually metastases that have spread intraperitoneally, hematogenously, or by local extension. Intraperitoneal seeding usually manifests at CT as multiple small nodular metastases along the small bowel serosa, mesentery, and omentum. In patients with Peutz-Jeghers syndrome, nonneoplastic lesions may mimic small bowel neoplasms.
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PMID:CT evaluation of small bowel neoplasms: spectrum of disease. 953 85

Gastrointestinal stromal tumour (GIST) is the designation used here to identify the most common subset of gastrointestinal mesenchymal tumours specific to those sites. These tumours have unique histological, immunophenotypic and molecular genetic features that set them apart from typical smooth muscle tumours and schwannomas; however, by tradition, they have been classified as GI-smooth muscle tumours, or stromal tumours/smooth muscle tumours. GISTs occur predominantly in persons over 40 years of age with an equal sex incidence. Benign GISTs outnumber the malignant ones by a margin of 10:1. GISTs occur throughout the gastrointestinal tract, but are most common in the stomach (60-70%) and small intestine (30%). GISTs are rare in esophagus, colon and rectum. Histologically they may show a spindle cell or epithelioid pattern (the former largely corresponds with the designation of cellular leiomyoma and the latter with that of leiomyoblastoma). Immunohistochemically most GISTs are positive for CD34 and c-kit protein (CD117); the latter is quite specific for GISTs among mesenchymal tumours. Genetically GISTs commonly show DNA losses in the long arm of chromosome 14, and c-kit gene mutations occur at least in some cases. c-kit is also expressed in the interstitial cells of Cajal, the gastrointestinal pacemaker cells, and relationship of GISTs to these cells has been proposed recently. GISTs differ histologically, immunohistochemically and genetically from typical (esophageal) leiomyomas that are negative for c-kit and CD34 and neither show DNA-losses in 14q nor c-kit mutations. Evaluation of malignancy of GISTs is based on mitotic count, tumour size and extra-gastrointestinal spread. Tumours with mitotic counts higher than 5/10 high power fields or larger than 10 cm have a significant risk for recurrence and metastasis and are considered histologically malignant; however, some tumours with mitotic activity < 1/10HPF may metastasize indicating some uncertainty in malignant potential of GISTs, especially those larger than 5 cm.
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PMID:Gastrointestinal stromal tumours. 989 65

From May 1993 to May 1998 at our hospital 12 classic gastric resections were operated on by laparoscopic procedure. A 2/3 distal Billroth resection was performed in 5 patients, for gastric ulcer (2) and for GIST (3). Other 7 patients were curatively operated on for a malignant disease. Any case was resected by oncosurgical gastrectomy including a D2-lymphadenectomy. The histological diagnoses were an adenocarcinoma in 6 cases, and one highly malignant maltoma. The mean operating time was 230 min in Billroth I resections and 295 min in total gastrectomies. As a complication, we saw postoperatively a duodenal leakage in one case, we could successfully manage also laparoscopically on the day after the operation. All the courses were extremely uncomplicated, connected with rapid mobility, early gut motility, little pain, and a comfortable cosmetic result. The resection result in any case of malignoma was R0, the mean lymphonode amount was 34. Tumour cell dissemination and trokar site metastases we could'nt note either. Oncosurgically, there are no disadvantages in comparison to the open approach.
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PMID:[Initial experience with laparoscopic gastrectomy in benign and malignant tumors]. 1035 92

To identify genetic changes related to tumor progression and find out diagnostic and prognostic genetic markers in gastrointestinal stromal tumors (GISTs), 95 tumor samples (24 benign GISTs, 36 malignant primary GISTs, and 35 GIST-metastases) from 60 patients were studied using comparative genomic hybridization. DNA copy number changes were detected in all samples. Benign GISTs had a mean of 2.6 aberrations/ sample (losses:gains, 5:1) and significantly fewer DNA copy number changes and fewer gains than malignant primary and metastatic GISTs (P < 0.01). High-level amplifications were not seen in benign GISTs. Malignant primary GISTs had a mean of 7.5 aberrations/tumor (losses: gains, 1.6:1), whereas the mean number of aberrations/metastatic GIST was 9 (losses:gains, 1.8:1). Frequent changes observed in all GIST groups included losses in chromosome arms 1p (51%), 14q (74%), and 22q (53%). Gains and high-level amplifications at 8q and 17q were significantly more frequent in metastatic GISTs (57 and 43%) than in benign GISTs (8 and 0%; P < 0.001) and malignant primary GISTs (33 and 25%; P < 0.05). Gains and high-level amplifications at 20q were only seen in malignant primary and metastatic GISTs (P < 0.01), and gains at 5p were not detected in benign GISTs (P < 0.01). Losses in chromosome arm 9p were never seen in benign tumors (P < 0.001), and they were more frequent in metastatic GISTs than in malignant primary GISTs (63 and 36%; P < 0.05). Losses in 13q were less frequent in benign GISTs than in malignant primary (P < 0.05) and metastatic (P < 0.01) GISTs. Our results show that several DNA copy number changes are related to the behavior of GISTs and can be used as prognostic markers for tumor progression.
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PMID:DNA sequence copy number changes in gastrointestinal stromal tumors: tumor progression and prognostic significance. 1091 66

Tumors of the small intestine are relatively rare. The diagnosis is difficult to establish because the symptoms are vague and non-specific. Although the small intestine constitutes 75% of the length and over 90% of the mucosal surface area of the gastrointestinal tract, only 1 to 2% of gastrointestinal malignancies occur in this segment. Metastases are usually present at the time of diagnosis. The outcome of these patients can be improved if the possibility of a malignant small bowel tumor is considered in all cases of unexplained abdominal pain or gastrointestinal bleeding, especially in younger age. Malignant tumors occur with increasing frequency in distal small bowel with a preponderance of malignant lesions in the ileum compared with the jejunum and the duodenum. Adenocarcinoma is the most common tumor of the primary malignant small bowel tumors, followed by carcinoid, lymphoma and leiomyosarcoma. Mesenchymal tumors of the gastrointestinal tract, traditionally regarded as smooth muscle tumors, have demonstrated different cellular differentiations based on immunohistochemical and ultrastructural features. Therefore the terms leiomyoma and leiomyosarcoma have been replaced by a more encompassing term, gastrointestinal stromal tumor (GIST). The majority of GISTs occurs in the stomach; stromal tumors involving the small intestine (SISTs) are far less common but seem to have greater malignant potential. The clinical a case of a small intestinal stromal tumor (SIST), localised in the jejunum and characterised by an uncertain histological aspect, is presented and a review of the literature is made.
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PMID:[Gastrointestinal stromal tumors. A case of small intestine stromal tumor (SIST) with an uncertain biological aspect]. 1095 71

We report herein the case of a 70-year-old woman found to have a gastrointestinal stromal tumor (GIST) of the stomach. Preoperative X-ray and endoscopic examination revealed a hemispheric submucosal tumor with central depression in the anterior wall of the gastric fornix. The tumor, which was 3 cm in diameter, was resected by a laparoscopy-assisted procedure. Histologic examination revealed that it was composed of spindle-shaped cells with elongated nuclei, and few mitoses. Most of the tumor cells showed immunoreactivity for vimentin and CD34, but not for alpha-smooth muscle actin, desmin, or S-100 protein. The PCNA index was 40.5%. Thus, the GIST did not show differentiation toward smooth muscle or neural cells. A gastrectomy was not performed because the small size of the tumor, and the paucity of the mitoses indicated that it was benign. Nevertheless, careful and long-term follow-up is needed to monitor for signs of possible local recurrence or distant metastases.
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PMID:Gastrointestinal stromal tumor of the stomach: report of a case. 1132 47

Gastrointestinal stromal tumors (GISTs), the specific KIT-positive mesenchymal tumors of the gastrointestinal tract, have been sporadically reported in the rectum, but there are few clinicopathologic series. In this study we analyzed the clinicopathologic features of 133 anorectal GISTs, 3 intramural leiomyomas (LMs), and 8 leiomyosarcomas (LMSs) from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki. Ninety-six GISTs were documented as KIT-positive and three additional ones as CD34-positive. Thirty-four tumors were included by their histologic similarity to KIT- or CD34-positive cases. GIST-specific c-kit gene mutations, mostly in exon 11, were documented in 18 of 29 cases (62%). The GISTs occurred in adults with the age range of 17-90 years (median 60 years) with a significant male predominance (71%). The tumors ranged from small asymptomatic intramural nodules to large masses that bulged into pelvis causing pain, rectal bleeding, or obstruction. They were mostly highly cellular spindle cell tumors; four tumors had an epithelioid morphology. The tumors coexpressed CD34 and KIT and were rarely positive for smooth muscle actin or desmin and never for S-100 protein. Seventy percent of patients with tumors >5 cm with more than 5 mitoses/50 high power fields (HPF) (n = 31) died of disease, whereas only one tumor <2 cm with <5 mitoses/50 HPF (n = 21) recurred and none caused death. Long latency was common between primary operation and recurrences and metastases; either one occurred in 60 of 111 patients with follow-up (54%). Distant metastases were in the liver, bones, and lungs. Three benign actin- and desmin-positive and KIT-negative intramural LMs, similar to those seen in the esophagus, were identified. There were eight LMSs, six of which formed a polypoid intraluminal mass and were actin-positive and KIT-negative. Despite high mitotic counts, only one LMS patient died of disease. A great majority of rectal smooth muscle and stromal tumors are GISTs, which have a spectrum from minimal indolent tumors to overt sarcomas. Intramural LMs are exceptional, and true LMSs are rare, and similar to colonic ones, often present as intraluminal polypoid masses that appear to have a better prognosis than GISTs with similar mitotic rates.
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PMID:Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases. 1168 71

Involvement of the inferior vena cava (IVC) by hepatic tumors, although uncommon, is considered to be unresectable by standard surgical techniques. Recent advances in hepatic surgery have made combined hepatic and vena caval resection possible. The purpose of this study is to describe the surgical techniques and early results of combined resection of the liver and IVC. From 1997 to 2000, 11 patients underwent resection of the IVC along with four to seven liver segments. Resections were carried out for hepatocellular carcinoma (four); colorectal metastases (four); and hepatoblastoma, gastrointestinal stromal tumor metastases, and squamous cell carcinoma in one patient each. Ex vivo procedures were performed twice, and total vascular isolation was used in the nine other cases. The IVC was reconstructed with ringed Gore-Tex tube graft (five), primarily (five), or with Gore-Tex patches (one). There were two early deaths: one from liver failure at 3 weeks and one from sepsis secondary to a perforated segment of small bowel 4 months postresection. One patient with a gastrointestinal stromal tumor died at 32 months of recurrent tumor and one patient with hepatocellular carcinoma is alive with recurrent tumor at 16 months. The remaining patients are alive and disease free with follow-up ranging from 3 to 40 months without evidence of IVC occlusion. Combined resection of the liver and IVC is a formidable undertaking with substantial surgical risk. However, this aggressive surgical approach offers a chance for cure in patients with tumors involving the IVC that would otherwise have a dismal prognosis.
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PMID:Resection of the inferior vena cava for hepatic malignancy. 1173 Feb 25

Glomus tumors usually occur in the peripheral soft tissues, but similar tumors have also been reported in the stomach and occasionally in the intestines. However, the relationship of these tumors to peripheral glomus tumors and gastrointestinal stromal tumors has not been fully clarified because previous series of gastrointestinal glomus tumors predate availability of immunohistochemistry. This clinicopathologic study examined 32 gastrointestinal glomus tumors. All but one of the tumors were located in the stomach and the remaining tumor was from the cecum. The tumors occurred with a strong female predominance (23 females and 9 males) and a median age of 55 years (range 19-90 years). The gastric tumors typically presented with gastrointestinal bleeding or ulcer-like symptoms, and 14 tumors had mucosal ulceration. Five tumors were incidental findings. The tumor sizes varied from 1.1 to 7 cm (median 2 cm), and most were located in the antrum. Histologically, the tumors typically had a solid pattern of sharply demarcated, round glomus cells with prominent, mildly dilated pericytoma-like vessels. Vascular invasion and focal atypia were relatively common (seen in 11 and 13 cases, respectively), and low mitotic activity (1-4 per 50 high power fields), was seen in 10 cases. Immunohistochemically, all tumors were positive for alpha-smooth muscle actin and calponin, and nearly all had a net-like pericellular laminin and collagen type IV positivity. All tumors were negative for desmin and S-100 protein. Three tumors had focal synaptophysin positivity, but none was positive for chromogranin. All tumors lacked KIT expression and the GIST-specific mutations in the c-kit gene. Follow-up revealed one patient death of metastatic disease to liver at 50 months; this tumor had 1 mitosis per 50 high power fields, but had spindle cell foci, mild atypia, and vascular invasion. Thirteen patients were well and alive after long-term follow-up. Gastrointestinal glomus tumors occur almost exclusively in the stomach, and they have a good overall prognosis, but a small, unpredictable potential for malignant behavior exists. These tumors are phenotypically similar to peripheral glomus tumors and differ from epithelioid GISTs.
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PMID:Gastrointestinal glomus tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. 1185 1


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