Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030794 (pelvic pain)
 4,056 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gastrointestinal stromal tumors (GISTs) are c-kit-positive tumors that may arise anywhere in the tubular gastrointestinal tract. Around 5% of the cases arise elsewhere in the abdominal cavity. Tumors originating in the omentum and mesentery have been reported.A 31-year-old woman presented with pelvic pain, a palpable pelvic mass, and elevated CA-125. Imaging showed innumerable pelvic and abdominal masses. Histopathology showed a GIST that was positive for c-kit and vimentin and negative for desmin and smooth muscle actin. The patient was started on imatinib mesylate. Six months after diagnosis the tumor has remained stable.GI stromal tumors (GIST) may initially present as pelvic mass with elevated CA-125. Imatinib mesylate is the current mainstay therapy for GISTs after surgery.
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PMID:Gastrointestinal stromal tumor presenting as a pelvic mass. 1467 90

Nonsmall cell neuroendocrine carcinoma (NSCNEC) of the ovary is a rare and aggressive tumor commonly associated with other surface epithelial and germ cell neoplasms. In this study, we present the clinicopathologic and immunohistochemical features of 11 such cases seen at The University of Texas M.D. Anderson Cancer Center in a 16-year period (1990 to 2005). Patients ranged in age from 22 to 63 years (mean 46.7). The most common presentation was abdominal/pelvic pain (6 cases), followed by ascites (2 cases), pelvic mass, vaginal bleeding, and abdominal bloating (1 case each). Tumors were mostly unilateral, cystic, or solid/cystic and ranged in size from 5 to 26 cm (mean 16.2). In 8 cases, NSCNEC was associated with other epithelial neoplasms, including mucinous neoplasms of low malignant potential, mucinous carcinoma, endometrioid carcinoma, mixed endometrioid and mucinous carcinoma, and a high-grade carcinoma, not otherwise specified. In 2 cases, the tumor was associated with a mature cystic teratoma; one of them also containing an invasive moderately differentiated adenocarcinoma. A single case was associated with a benign ovarian cyst. The latter case had a dermoid cyst in the contralateral ovary. NSCNEC represented anywhere from 10% to 90% of the ovarian tumor. Microscopically, the neuroendocrine component was usually composed of large and/or intermediate oval to round cells. In 2 cases, the intermediate cells were intermixed with small cells. Three cases had also spindle cells. The neoplastic cells were mostly arranged in a solid pattern, nests, or trabeculae. All tumors had a brisk mitotic activity. Immunoperoxidase studies for keratin cocktail, cytokeratin (CK) 7, CK20, CAM 5.2, chromogranin A, synaptophysin, NSE, CD56, and c-kit were performed and the cases stained as follows: keratin cocktail 6/6, CK7 4/5, CK20 3/5, CAM 5.2 3/3, chromogranin A 8/11, synaptophysin 9/9, NSE 1/1, CD56 4/8, and c-kit 5/7. According to the International Federation of Gynecology and Obstetrics staging system, 4 cases were stage I tumors, 3 cases were stage III tumors, and 4 cases were stage IV tumors. Seven patients were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by chemotherapy. One patient had a bilateral salpingo-oophorectomy with omentectomy and appendectomy followed by chemotherapy; 1 patient had a total abdominal hysterectomy with right salpingo-oophorectomy followed by chemotherapy; one had a bilateral salpingo-oophorectomy followed by chemotherapy, and one had a right salpingo-oophorectomy with appendectomy followed by chemotherapy. Five patients died of disease at 2, 3, 9, 20, and 36 months. One patient is alive with disease at 8 months and 5 are alive without evidence of disease at 11, 28, 37, 66, and 68 months. Four of 5 patients who died of disease had either stage III or IV tumors and 3 of 5 patients who are alive without evidence of disease have stage I tumors. In summary, ovarian NSCNEC is an aggressive tumor with a tendency to present at advanced stage and cause death within a mean of 17 months after diagnosis; however, some patients, particularly those with stage I disease and/or those who have received platinum-based therapy, may have a more favorable prognosis.
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PMID:Ovarian nonsmall cell neuroendocrine carcinoma: a clinicopathologic and immunohistochemical study of 11 cases. 1746 Apr 63

The author reports a very rare case of a gastrointestinal stromal tumor in the uterus. A 74-year-old woman was admitted to our hospital because of pelvic pain. Imaging modalities showed a large tumor of the posterior aspect of the uterus, and enucleation was performed. The tumor was attached to the posterior uterus, similar to subserosal leiomyoma. No attachment to the gastrointestinal organs was recognized. The tumor was soft, tan, and measured 13 x 15 x 12 cm. The tumor consisted of cellular spindle cells with focal necrotic areas. Mitotic figures were noted in 3 of 50 high-power fields. The tumor cells were positive for KIT, CD34, platelet-derived growth factor receptor alpha, and vimentin, but negative for alpha-smooth muscle actin, S100 protein, p53 protein, HMB45, and desmin. Ki-67 labeling was 3%. Five normal uteruses used as controls showed KIT-positive Cajal-like mesenchymal cell scattering in the myometrium. Genetic analyses of the c-kit gene (exons 9, 11, 13, and 17) and platelet-derived growth factor receptor alpha gene (exons 12 and 18) revealed a point mutation at codon 559 (GTT-->GAT) of exon 11 of the c-kit gene. Other exons showed no abnormalities. This case shows that gastrointestinal stromal tumor may occur in the uterus.
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PMID:Gastrointestinal stromal tumor of the uterus: a case report with genetic analyses of c-kit and PDGFRA genes. 1904 11

Gastrointestinal stromal tumours (GIST) are rare mesenchymal tumours which represent 1% to 3% of gastrointestinal neoplasm. Rectal location of GIST is extremely rare reaching 5% of GIST and only 0.1% of rectal tumours. They usually metastases to the liver (65%) and exceptionally to the bone (3%). We reported a case of rectal stromal tumour with an exceptional metastasis located in the rib. A 40-year-old man who presented with pelvic pain, associated with rectal syndrome, rectal bleeding and subocclusive episodes. Physical examination objectified a tough, budding rectal mass, with a smooth wall, localized 3cm above of anal margin. A Thoraco-abdominal computed tomography showed a large heterogeneous tissue mass, taking the whole pelvis, coming from the right-side wall of the rectum of 17.3 x 14cm. It was associated with liver and bone secondary locations. Biopsies confirmed the secondary locations of an intermediate risk GIST. Immunohistochemical study showed an overexpression of c-kit protein (CD117) and Dog1. Imatinib was prescribed to reduce the tumour size. Stromal metastatic rectal tumours in bone level are extremely rare conditions. The diagnosis is confirmed by histological examination with immune histochemical analysis. The prognosis remains poor in metastatic forms but it has been improved since the introduction of Imatinib.
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PMID:Rectal stromal tumor with an exceptional liver and bone metastatic locations. 3130 6