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)

Although clear cell carcinomas have been described in numerous anatomic sites, their occurrence in the gallbladder and extrahepatic bile ducts (EHBD) is practically unknown. We report 10 such cases. Seven arose in the gallbladder and three in the EHBD; all patients with gallbladder tumors were females with cholelithiasis whose ages ranged from 56 to 68 years. Patients with EHBD tumors were younger (38 and 40 years of age) and had extrahepatic biliary obstruction and abdominal pain. Two patients with gallbladder carcinomas had elevated serum carcinoembryonic antigen (CEA) levels, and another without hepatic involvement had markedly elevated circulating levels of alpha-fetoprotein (AFP). Histologically, nine tumors were adenocarcinomas and one was a squamous cell carcinoma. Seven adenocarcinomas consisted of cords, sheets, nests, papillae, and trabeculae of clear cells with well-defined cytoplasmic borders. Two were composed predominantly of glands and papillary structures. The cells contained PAS-positive diastase-labile granules and were cytokeratin- and EMA-positive and immunoreactive for erythropoiesis-associated antigen. One gallbladder tumor contained areas of hepatoid differentiation, a feature described in gallbladder neoplasms only once before. These areas were AFP-positive and immunoreactive for CEA. By electron microscopy, they showed hepatoid differentiation with formation of bile canaliculi. In two gallbladder tumors, neoplastic cells contained subnuclear vacuoles reminiscent of early secretory endometrium. Foci of conventional adenocarcinoma or mucinous carcinoma were recognized in all nine tumors. The squamous cell carcinoma showed only foci of squamous differentiation with keratinization. The clear cells of this neoplasm had a trabecular and solid growth pattern. These clear cell neoplasms of the gallbladder and EHBD must be differentiated from metastatic renal cell carcinoma, based upon the presence of areas of conventional adenocarcinoma or foci of squamous differentiation since results of special stains and immunohistochemistry are similar in both neoplasms. One of the patients with EHBD carcinoma is alive and symptom-free 6 years following right hepatic lobectomy. Five patients with gallbladder tumors had direct extension into the liver and died with metastases. Two are living with metastases.
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PMID:Clear cell carcinomas of the gallbladder and extrahepatic bile ducts. 780 41

Multinucleated giant stromal cells (MGSC) have been described in a variety of lesions of various anatomical sites. They are generally believed to be derived from fibroblasts or myofibroblasts. Their size and bizarre appearance may lead to an erroneous interpretation of infiltrating malignant cells, but they are regarded as reactive in nature. MGSC also seem to participate in a neoplastic process and form a part of tumors called giant cell fibroblastomas (GCF). In GCF, multinucleated giant cells are sparsely scattered throughout the tumor, which is composed of loosely arranged spindle cells. Thus far, no tumor composed of MGSC entirely, to the best of the authors' knowledge, has been reported. This study involved an 80-year-old female with an omental tumor, which is believed to represent the first case of tumor of MGSC. The patient developed abdominal pain; a large abdominal tumor measuring 18 x 15 x 5 cm by computerized tomography was found located between the left lobe of the liver, the transverse colon, and the greater curvature of the stomach. Although the tumor was adherent to the above organs and infiltrating the omentum, it was resectable. Grossly, the tumor was highly vascular and the surface was shaggy with no recognizable capsule. The cut surfaces were red to tan with frequent cystic spaces containing bloody material. Microscopically, the tumor cells were large and multinucleated (2-6 nuclei) with prominent nucleoli. The cytoplasm was abundant and stained amphophilic. These tumor cells formed moderately cellular sheets filling the spaces between the varying sized vessels. There was prominent vascularity throughout the tumor. DNA study by image analysis revealed aneuploidy peaks. On immunohistochemistry, the tumor cells were strongly positive for vimentin, moderately positive for actin along the periphery of the cytoplasm, and negative for cytokeratin, EMA, myoglobin, S-100, CEA, Factor XIIIa, HMB-45, and HAM56 and KP-1. Ultrastructurally, the cytoplasm contained rich profiles of RER with scattered lysosomes. The cell borders were slightly irregular with occasional subplasmalemmal densities facing loosely arranged collagenous stroma. The light microscopic, immunohistochemical, and electron microscopic features of tumor cells were remarkably similar to MGSC. The tumor size and gross appearance suggested a malignancy, but it was a diploid tumor and the patient remains disease free 5 years after a complete resection.
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PMID:Multinucleated giant stromal tumor of the omentum: report of a case with immunohistochemical and ultrastructural investigation. 878 15

A case of primary gastric T-cell lymphoma, which was positive for granzyme B, is reported. The patient was a 47-year-old Japanese female who complained of a dull upper abdominal pain. Radiographic and endoscopic examinations revealed an ulcerative infiltrative lesion in her stomach. Following the confirmation of a high-grade malignant lymphoma, a distal gastrectomy with regional lymph nodal dissection was performed. The histology of the gastric lesion revealed a malignant lymphoma of the diffuse pleomorphic type without lymph nodal involvement. Immunohistochemistry revealed that the tumor cells were positive for LCA, CD3, TIA-1 and granzyme B, but were negative for CD4, CD8, CD56, CD30, L-26, EMA, TCR alpha/beta and TCR gamma/delta. Because the tumor cells showed T cell nature with cytotoxic activity proved by TIA-1 and granzyme B, and without evidence of further maturation of T cell, a malignant lymphoma originating from extrathymic-derived T cells was suggested.
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PMID:Granzyme B-positive primary gastric T-cell lymphoma: gastric T-cell lymphoma with the possibility of extrathymic T cell origin. 1110 59

Celiac disease is an autoimmune gastrointestinal disorder characterized by mucosal atrophy of the jejunum on exposure to gluten, a protein found in grains. The purpose of our study was to determine the prevalence of celiac disease in children with Downs syndrome in a U.S.-based Caucasian population. The 97 Downs syndrome children were screened for celiac disease using serum IgA-anti-endomysial antibody testing, which is highly specific and sensitive for the disorder. Children with titers greater than 1:5 (using the IgA endomysial antibody [EMA] test; EMA+) were considered affected. Ten children (10.3%) were EMA+. We examined their HLA DQA1 DQB1 genotype, karyotype, clinical characteristics, and the prevalence of celiac disease in their first-degree relatives. The nine available karyotypes were trisomy 21. Downs syndrome-specific mean height percentile was 64%+/-26% (range <5-99%) and weight percentile was 43%+/-28% (range 5-95%). Presence of diarrhea, constipation, vomiting, and abdominal pain was similar for children with and without celiac disease. Only bloating symptoms were significantly more frequent in those with celiac disease (EMA+). Seven of eight (88%) genotyped EMA+ children had the celiac disease-associated high-risk HLA DQA1*0501 DQB1*0201 genotype as compared with 13/ 80 (16%) of EMA- children. Five of 48 (10%) first-degree relatives of the celiac disease (EMA+) children were EMA+. In conclusion, celiac disease, as diagnosed by positive endomysial antibody tests, has an increased prevalence in children with Downs syndrome in the U.S. as compared with the general population (1/250). Clinical and growth characteristics do not distinguish between children with and without celiac disease. Based on these observations, it is recommended that children with Downs syndrome be screened for celiac disease.
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PMID:Prevalence and clinical characteristics of celiac disease in Downs syndrome in a US study. 1142 58

The aim of this study is to report the experience of a case of benign multicystic mesothelioma of the peritoneum presented with acute appendicitis symptomatology. A 28 years old man with right lower and upper abdominal pain was admitted into hospital. Because of the clinical picture, the symptomatology and the leukocytosis a diagnosis of acute appendicitis was made and the patient underwent appendicectomy according to Mc Burney. At laparotomy some cc of purulent fluid were sucked and a cystic mass that contained clear fluid was revealed. In consequence of the incidental diagnosis a following middle laparotomy was made with a careful surgical excision of the mass and of the appendix. Macroscopically the lesion was identified like a neoplastic mass 25 centimeters in diameter, with a multicystic and fibrous-adipose aspect, with cysts 5 centimeters in diameter. The cystic spaces were lined by a layer of eptelial cells which presented positive reaction for cytokeratin and EMA, whereas endothelium markers were absent. The ultrastructural, morphological and immunohistochemical findings were diagnostic of a benign multicystic mesothelioma.
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PMID:Benign multicystic mesothelioma of the peritoneum: a case report. 1223 71

Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma (DLBCL) is a rare, recently defined tumor distinct in many aspects from ALK-positive anaplastic large cell lymphoma (ALCL). We present two additional cases of ALK+DLBCL recently diagnosed in our department and a review of literature. A 48-year old man presented with a large upper neck mass growing slowly over 18 months. Histologically the tumor was diagnosed as an ALK-positive diffuse large B-cell lymphoma. with plasmablastic features. Large, frequently intrasinusoidal tumor cells expressed CD138, EMA, weakly IgA and kappa, but were negative for other B-cell markers, T-cell markers and CD30. The ALK staining was cytoplasmic with the increased intensity in the Golgi area. At the diagnosis the patient manifested with the stage IIIB. Three courses of CHOP resulted in partial and only transient remission. The patient died of massive bleeding from his decomposing tumor 3 months after the diagnosis. A 49-year old man complaining of abdominal pain revealed abdominal lymphadenomegaly and a gastric infiltrate, involving the deep portions of the gastric wall. The tumor showed immunoblastic/anaplastic morphology, with some Reed-Sternberg-like cells positive for ALK. ALK immunostaining was cytoplasmic, weak in a routine immunostain, enhanced with double (proteinase + pressure cooker) antigen retrieval. FISH was consistent with the t(2;5)/nucleophosmin(NPM)-ALK rearrangement. The tumor demonstrated similar "null" B/T phenotype with positivity for IgA, lambda, EMA and LCA. The patient (stage IVB) currently undergoes chemotherapy. ALK-positive DLBCL affects mostly middle-aged men, shows generally poor but stage-dependent prognosis (at least 60% mortality rate), presents typically as a lymph node-based disseminated disease, and very rarely involves the bone marrow. Genetic studies showed that the majority of ALK+DLBCL cases are characterized by the clathrin (CLTC)-ALK fusion and in a few cases the NPM-ALK rearrangement has been found.
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PMID:ALK-positive diffuse large B-cell lymphoma: two more cases and a brief literature review. 1592 Oct 12

A 28-year-old woman presented with abdominal pain. Ultrasonograhic examination showed a pre-sacral mass, with complex structure and well delimitated cystss with thick walls. The resected specimen was 7.5 x 6 x 4 cm in size, well circumscribed and yellow in colour, with cysstic change containing mucoid-like material. Histologically, the lesion was composed of spindle cells with high cellularity and rich vascularization with a haemangiopericytoma-like pattern. The diagnosis of solitary fibrous tumour (SFT) was made. The differential diagnosis for SFT of the pre-sacral spaace involves haemangiopericytoma, GIST, malignant mesothelioma, synovial sarcoma, leiomyomatous tumours and granulosa cell tumour. Immunohistochemical studies revealed reactivity for CD34, CD99 and Bcl-2, but no staining for desmin, inhibin, c-kit, EMA, CK, SMA, S-100 and CD31, confirming a diagnosis of SFT. Although SFT is usually associated with a favourable prognosis, close follow-up is recommended because of the limited information on its long-term behaviour.
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PMID:A rare case of solitary fibrous tumour of the pre-sacral space: morphological and immunohistochemical features. 1599 48

Perivascular epithelioid cell tumor (PEComa) is defined as a very rare mesenchymal tumor of histologically and immuno-histochemically distinctive perivascular epithelioid cells. PEComa in the colon is very rare, with only a few reported cases so far. Because of its rarity, the clinical features and biological behavior of PEComa in the colon have yet to be established. A 16-year-old female patient with PEComa in the transverse colon was referred to our hospital for rectal bleeding. Laboratory data showed a hemoglobin level of 6.6 g/dL, WBC of 8,800/mm(3), and platelet count of 191,000/mm(3). Colonoscopy, barium enema, and abdominal computed tomography revealed a 2-cm, smooth-surfaced, round tumor with focal ulceration in the proximal transverse colon. The patient complained of abdominal pain one day after endoscopic polypectomy. She underwent a segmental resection for a perforated transverse colon. Immunohistochemically, the tumor cells showed strong diffuse positivity for HMB-45 while they were negative for c-kit, smooth muscle actin, cytokeratin, S-100, vimentin, desmin, chromogranin, synaptophysin, EMA, and CD-34. The diagnosis of PEComa was based on histological and immunohistochemical staining. The patient did not receive any adjuvant therapy and was discharged on postoperative day 11 without complications. Whole-body fluorine-18 fluorodeoxyglucose fusion positron emission tomography performed 2 months after surgery showed no signs of recurrence or metastasis. There was also no recurrence or metastasis at 24 months' follow-up.
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PMID:Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. 1745 82

Malignant rhabdoid tumor, first described in the kidney of young infants, is a rare and highly aggressive neoplasm of controversial histogenesis that has been reported at many other sites, including the gastrointestinal tract. However, malignant rhabdoid tumor of the small intestine is very rare, with only seven cases published to date. We report a 70-year-old man who presented with abdominal pain and weight loss, and showed a perforated jejunal mass with disseminated metastases by imaging. The patient underwent partial jejunectomy and biopsy of a liver metastasis. Microscopically, the tumor was characterized by neoplastic cells with vesicular nuclei, large nucleoli and abundant eccentric cytoplasm with hyaline globular intracytoplasmic inclusions. Immunohistochemically, the neoplasm coexpressed vimentin and epithelial antigens (AE1/AE3, Cam 5.2, CK34betaE12, CK19 and EMA), most of them showing a peculiar immunostaining pattern in relation to the globular inclusions. Ultrastructurally, the inclusions corresponded to paranuclear whorls of intermediate filaments. The patient received postoperative chemotherapy but died 9 months after surgery. In summary, we report the exceptional case of an undifferentiated carcinoma of the jejunum with rhabdoid phenotype. As with tumors at other sites, recognition of rhabdoid morphology in small intestine neoplasms is of significance because the prognosis is extremely poor.
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PMID:Undifferentiated carcinoma of the jejunum with extensive rhabdoid features. Case report and review of the literature. 1913 90

Pure Sertoli cell tumor (SCT) is a rare sex cord tumor and a subtype of Sertoli-Leydig cell tumors according to the WHO Classification. They lack a Leydig cell component and do not contain the immature neoplastic stroma found in the neoplasms of the Sertoli-Leydig cell category. The age of the patients ranges between two and 79 years. Sertoli cell tumors occur in women of reproductive age but a few can also occur in children. The most common clinical presentation when occurring in children is isosexual pseudoprecocity. Women of reproductive age and postmenopausal women frequently present with abdominal pain, swelling and menstrual abnormalities. Occasionally SCTs occur in patients who have Peutz-Jeghers syndrome. The tumors are hormone functional in 40-60% of cases. They are often estrogenic, occasionally also androgenic or rarely both. Grossly they are usually yellow to brownish, solid or with several cystic areas. Microscopically they show always almost a tubular growth pattern, but they may also have other growth patterns which can be extensive, making the correct diagnosis difficult. These histologic patterns may result in SCTs mimicking other ovarian tumors. The immunohistochemical panel which usually includes EMA, inhibin, chromogranine, CD99 and calretinin is often helpful in establishing the diagnosis. Most SCTs are Stage I, unilateral, cytologically bland, and clinically benign, but occasional examples are high stage. About 11% of Stage I tumors have worrisome histologic features that may portend an adverse outcome.
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PMID:Pure Sertoli cell tumor. a case report and review of the literature. 2034 97


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