Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Desmoplastic small round cell tumors (DSRCT) are highly aggressive tumors typically involving the serosal surfaces of the peritoneum. Patients often present with abdominal pain, an abdominal mass, ascites or signs of intestinal obstruction. Cytogenetic and molecular studies have identified a characteristic t(11;22)(p13;q12) translocation within the tumor cells. The fused gene product apparently aligns the NH2-terminal domain (NTD) of the EWS gene to the zinc finger DNA-binding domain of the WT1 gene. This product could lead to loss of the tumor suppressor effect of the WT1 gene as well as to an increase in EWS driven expression of growth factors normally repressed by WT1. We investigated this latter possibility by performing immunohistochemical studies on formalin fixed tissue from 10 cases of DSRCT and five Wilms' tumors using-antibodies to insulin-like growth factor (IGF)-II, the latency associated peptide of transforming growth factor (TGF)-beta 1, platelet-derived growth factor (PDGF)-AB chain and PDGF-alpha receptor, respectively. In general, tumor cells were strongly positive for these growth factors in DSRCT, while stromal cells were negative for IGF-II and positive for the other growth factors in parallel with the tumor cells. Wilms' tumor cells were essentially negative for PDGF-AB chains, but positive for IGF-II, and the latency associated peptide of TGF-beta 1 and variably positive for PDGF-alpha receptor. These findings support the proposed molecular mechanism of tumorigenesis for DSRCT and may help explain this tumor's poor prognosis.
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PMID:Intra-abdominal desmoplastic small round cell tumor: immunohistochemical evidence for up-regulation of autocrine and paracrine growth factors. 984 6

Desmoplastic small round cell tumors (DSRCT) are highly aggressive tumors typically involving the serosal surfaces of the peritoneum. Patients often present with abdominal pain, an abdominal mass, ascites or signs of intestinal obstruction. Cytogenetic and molecular studies have identified a characteristic t(11;22)(p13;q12) translocation within the tumor cells. The fused gene product apparently aligns the NH2-terminal domain (NTD) of the EWS gene to the zinc finger DNA-binding domain of the WT1 gene. This product could lead to loss of the tumor suppressor effect of the WT1 gene as well as to an increase in EWS driven expression of growth factors normally repressed by WT1. We investigated this latter possibility by performing immunohistochemical studies on formalin fixed tissue from 10 cases of DSRCT and five Wilms' tumors using antibodies to insulin-like growth factor (IGF)-II, the latency associated peptide of transforming growth factor (TGF)-beta1, platelet-derived growth factor (PDGF)-AB chain and PDGF-alpha receptor, respectively. In general, tumor cells were strongly positive for these growth factors in DSRCT, while stromal cells were negative for IGF-II and positive for the other growth factors in parallel with the tumor cells. Wilms' tumor cells were essentially negative for PDGF-AB chains, but positive for IGF-II, and the latency associated peptide of TGF-beta1 and variably positive for PDGF-alpha receptor. These findings support the proposed molecular mechanism of tumorigenesis for DSRCT and may help explain this tumor's poor prognosis.
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PMID:Intra-abdominal desmoplastic small round cell tumor: immunohistochemical evidence for up-regulation of autocrine and paracrine growth factors. 1007 70

Desmoplastic spindle cell tumor of the liver is a recently described and extremely unusual neoplasm that affects children and young adults. We report 1 case in a 33-year-old man. The patient had abdominal pain and dyspepsia. Abdominal examination showed that the liver was enlarged and palpable until umbilical region. Laboratory studies demonstrated positive serologic markers to hepatitis B virus. All other analytical studies were irrelevant. Computed tomography revealed a large tumor mass in left hepatic lobe showing heterogeneous densities, with hyperdense peripheral areas, as multiple nodular calcifications of less than 1 cm. In the central part of the mass, a big hypodense area was observed. There was no evidence of extrahepatic disease. Grossly, the tumor was well circumscribed with multiple nodular calcifications. The tumor was characterized by the presence of cohesive nests of bland spindle cells arranged in short fascicles and surrounded by desmoplastic stroma, intermixed with epithelioid cells. Mitotic activity was very low. Extensive osteoid formation was seen inside the cell nests. The tumor cells showed cytoplasmic reactivity for vimentin and pan-cytokeratin. The cells of desmoplastic stroma were immunoreactive for actin. The biologic behavior is still uncertain with only 5 published cases, but current information suggests that they are low-grade tumors with an indolent course. The clinical and morphologic features of this tumor are very similar to those of tumors previously reported as "nested stromal-epithelial tumor of liver" and "ossifying stromal-epithelial tumor of liver." We describe the histologic, immunohistochemical, and molecular genetic features of a case of desmoplastic spindle cell tumor of the liver and review the literature.
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PMID:Desmoplastic nested spindle cell tumor of the liver in an adult. 2012 57

Malignant primary cardiac tumors are very rare.Desmoplastic small round cell tumors are also rare tumors and,when present, are generally found in the abdomen. We report a case of an adolescent male who presented with chest pain,abdominal pain, and difficulty in breathing, who was found to have a primary cardiac sarcoma with several characteristic features of a desmoplastic small round cell tumor.
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PMID:A primary cardiac sarcoma with features of a desmoplastic small round cell tumor in an adolescent male. 2038 63

Desmoplastic small-round-cell tumor (DSRCT) is an uncommon type of malignancy, which primarily occurs in adolescent males and develops in the abdominal cavity. The present study reports the case of two manifestations of DSRCT complicated with other diseases, which involved the invasion of the pelvis or abdominal vessels. The first case was of a 25-year-old man with repeated diarrhea and abdominal distension for 9 months; the second case was of a 68-year-old man who presented with persistent abdominal pain for 20 days. In each patient, a mass was identified in the abdomen and biopsies were performed in order to reach a diagnosis. Immunohistochemical staining of the biopsy material revealed a diagnosis of DSRCT in each case. In addition, the present study aimed to discuss the use of imaging techniques in providing evidence for the early diagnosis of DSRCT.
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PMID:Desmoplastic small-round-cell tumor of the abdomen: A report of two rare cases. 2662 57

Desmoplastic Small Round Cell Tumour (DSRCT) is an uncommon malignant type of Small Round Blue Cell tumours with less than 200 cases reported in literature. Notorious for its aggressiveness, its commonest site of affectation is the abdomen, with a predilection for peritoneal cavity and prone to spread to multiple organs. We report a case of a 26-year old male undergraduate who presented with a 6-month history of progressive abdominal swelling associated with abdominal pain, weight loss, and change in bowel habits. He was cachectic with dyspnoea, bilateral pitting pedal oedema, a grossly distended and a palpable huge firm mass measuring 20 x 15cm. Immunohistochemisttry confirmed a diagnosis of DSRCT. An abdominopelvic Computed Tomography (CT) scan showed extensive abdominal and pelvic disease with pulmonary and liver metastases. He could only tolerate one course of chemotherapy due to deteriorating renal function. It ran an aggressive course of 9 months from onset of symptoms to eventual demise of the patient.
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PMID:DESMOPLASTIC SMALL ROUND BLUE CELL TUMOUR OF THE ABDOMEN - A CASE REPORT. 2773 22

Desmoplastic small round-cell tumour is a very rare neoplasm, which usually arises from the abdominal or pelvic peritoneum of adolescents and young adults. Early diagnosis is difficult, because most tumours present with non-specific gastrointestinal symptoms after a long asymptomatic period. It is generally a very aggressive tumour, which grows rapidly with poor prognosis and an overall five-year survival rate of 15% despite multimodal treatment. Despite multiple treatment strategies, the management of desmoplastic small round-cell tumour still remains a clinical challenge and no consensus about a therapeutic protocol has been established. A 35-year-old man presented with mild abdominal pain, constipation and weight gain, and was eventually diagnosed with desmoplastic small round-cell tumour, which was shown to be limited to the abdomen. After incomplete debulking surgery, radiotherapy and chemotherapy, he developed multiple metastatic nodular foci in chest and the pleura and, unfortunately, he died due to disease progression.
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PMID:Desmoplastic small round-cell tumour of the peritoneal cavity: case report and literature review. 3197 62