Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gastrointestinal stromal tumor (GIST) is very rare in infancy. Most of the reported cases in the literature are in adults; some are in children but there are a few reported cases in the literature. The present case is a 6-day-old female neonate presenting with lethargy, poor feeding, constipation, abdominal distention, and rectal bleeding. She was operated on with the impression of intestinal obstruction, and right hemicolectomy was performed on her. Surgical specimen showed a well-defined and round 3-cm mass in the cecal area. Diagnosis was made by histologic and immunohistochemical studies which showed a GIST. The tumor showed positive vimentin and c-kit but negative for all other markers (desmin, actin, S100, NSE, and CD-34). So the case was an undifferentiated GIST. After 1 year of follow-up the patient was completely normal.
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PMID:Neonatal gastrointestinal stromal tumor. Report of a case and review of literature. 1579 38

Ectopic human chorionic gonadotropin production has been described in a wide variety of non-germ cell tumors, particularly in epithelial tumors, but rarely in sarcomas. In this report, we describe the case of 49-year-old woman with a history of "uterine fibroids," who presented with vaginal bleeding and a positive urine pregnancy test. After pregnancy was ruled out by ultrasound, the patient underwent a laparotomy and hysterectomy for a presumptive diagnosis of "fibroids" and was found to have carcinomatosis at the time of the surgery. Therefore optimal debulking of tumors was performed. Two weeks later, the patient developed a small bowel obstruction, which apparently was due to rapid recurrence of tumors in the abdomen, and soon afterwards she died. Microscopically, the resected pelvic mass was composed of highly atypical and pleomorphic spindle cells admixed with many multinucleated giant cells. The tumor had a high mitotic rate along with areas of hemorrhage and necrosis. Immunohistochemically, the tumor cells were positive for vimentin, desmin, smooth muscle actin, and beta-human chorionic gonadotropin, and were negative for epithelial membrane antigen, keratin AE1/3, S-100, CD31, CD117, Ber-EP4, WT-1, estrogen and progesterone receptors. The majority of cells, including the multinucleated giant cells, were strongly immunoreactive for beta-human chorionic gonadotropin. Only three cases of leiomyosarcomas with beta/human chorionic gonadotropin production have been described in the literature, and all three cases had extrauterine origin. Our case, to the authors' best knowledge, is the first uterine leiomyosarcoma with prominent beta/human chorionic gonadotropin production.
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PMID:A high-grade uterine leiomyosarcoma with human chorionic gonadotropin production. 1681 64

The nature of the distinctive ovarian lesion often associated with sclerosing peritonitis, initially considered a variant of luteinized thecoma in the paper describing this phenomenon, remains uncertain, as does its long-term prognosis. We describe the features of 27 cases, including immunohistochemical analysis of 13 cases. Sclerosing peritonitis was documented in 25 cases. Patients ranged in age from 10 months to 85 years, and typically presented with abdominal distension and pain with ascites and sometimes bowel obstruction. The ovarian lesions, clinically bilateral in 24 cases, ranged from 2 to 31 cm and often had a striking cerebriform aspect. Microscopically, mitotically active spindle cells with weakly luteinized cells, variable edema, and entrapped follicles were typical. The spindle cells were focally positive with calretinin in 2 cases, CD56 in 2, AE1/3 in 4, smooth muscle actin in 12, and desmin in 8 cases, and negative with alpha-inhibin, epithelial membrane antigen, beta-catenin, CD34, and transforming growth factor-beta, with focal nuclear positivity for estrogen receptor in 5 and progesterone receptor in 11 cases. Luteinized cells were positive with alpha-inhibin, calretinin, and/or CD56. The peritoneal lesions were strongly positive with AE1/3 and exhibited focal weak or moderate positivity with estrogen receptor or progesterone receptor in 4 of 8 cases each. Follow-up in 20 cases (mean: 5.9 y) disclosed no evidence of spread of the ovarian lesion, but 3 patients died of sclerosing peritonitis. The findings fail to allow definitive classification of the ovarian lesions, and we prefer at present to retain their current designation as a subtype of luteinized thecoma, but to allow for the possibility of a non-neoplastic nature, feel it reasonable to have the designation "thecomatosis" as a parenthetical alternative. We have documented for the first time that sclerosing peritonitis is not invariably associated with the distinctive ovarian pathology present in these cases.
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PMID:Luteinized thecomas (thecomatosis) of the type typically associated with sclerosing peritonitis: a clinical, histopathologic, and immunohistochemical analysis of 27 cases. 1863 18

Gastrointestinal stromal tumors (GISTs) are rare in the childhood period. The authors reported a case who was admitted to the neonatal intensive care unit (NICU) on a suspicion of intestinal obstruction. She was operated and a mass in a size of 6 x 4.5 x 4 cm was resected from the ileum. Histologic and immunohistochemical studies showed a GIST. CD34, small muscle actin (SMA), and desmin were positive. The baby was discharged on the 13th day after operation.
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PMID:A rare cause of intestinal obstruction: neonatal gastrointestinal stromal tumor. 2129 43

Neuromuscular and vascular hamartoma (NMVH) is an unusual lesion presenting as intestinal obstruction by stricture formation. It is characterized by a hamartomatous mass comprising haphazardly arranged mesenchymal tissue native to the intestinal mucosa and submucosa. We aimed to characterize the clinicohistopathological spectrum of NMVH in adult subjects with a search for an etiological clue. We reviewed 84 resected specimens (adult cases) of intestinal obstruction in our institute and diagnosed 4 cases with NMVH. A panel of special stains (Masson trichrome, Verhoeff-van-Gieson, and periodic acid-Schiff) and immunohistochemistry (smooth muscle actin, S100, Bcl2, CD34, vimentin, desmin, CD117, and CD3) were performed in all cases. All cases of NMVH showed characteristic hamartomatous mounds comprising haphazardly arranged smooth muscle, nerves, ganglia, vessels, and collagen with overlying mucosal ulceration. Adjacent bowel showed submucosal fibrosis, muscularis mucosae thickening, and duplication along with vasculopathy. A typical vasculopathy ("vessel-in-vessel" appearance) was seen in the submucosal and/or subserosal veins. Besides, different other forms of vasculopathic changes like obliterative venopathy and concentric myohypertrophy were also seen. One case had vasculitis and the patient died despite successful surgery. One other case was associated with lymphocytic ganglioneuronitis and granulomatous etiology. We conclude that NMVH can be multifactorial in origin although ischemia resulting from vasculopathy appears to be directly causative. The characteristic vasculopathy in the submucosal location may aid in the diagnosis of NMVH in small biopsy samples.
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PMID:Histopathologic Spectrum of Neuromuscular and Vascular Hamartoma With Special Reference to the Vasculopathic Phenomenon. 3176 Aug 40