New approach to management of malignant ascites with a streptococcal preparation, OK-432. I. Improvement of host immunity and prolongation of survival.
Explore the source record for details and available documents.
SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
We present the case of a 23-year-old male with a history since early childhood of lip and tongue mucosal neuromas. At the age of 19, he was diagnosed with both medullary thyroid carcinoma and pheochromocytoma within 1 year. These findings, with his marfanoid habitus, led to the diagnosis of multiple endocrine neoplasia type 2 (MEN 2B) syndrome. This was confirmed by a positive RET proto-oncogene. On this admission, he presented with an intestinal obstruction. Abdominal exploration revealed an obstructing tumor mass requiring colectomy, which proved by biopsy to be an intestinal neuroma. This report presents a unique case of a colonic mucosal neuroma causing obstruction in MEN 2B syndrome after the diagnosis of medullary thyroid carcinoma.
A case of small intestinal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with monoclonal cryoglobulinemia is described. The patient was a woman in her mid-sixties with purpura of the bilateral lower legs and abdominal pain. An immunoserological investigation showed expression of IgM-kappa type monoclonal cryoglobulin. A renal biopsy specimen revealed proliferative glomerulonephritis with cryoglobulin deposition. Physical examination disclosed a stenosis, edematous changes and ascariasis in the small intestine. In aspiration cytology of the ascites, proliferation of the atypical lymphoid cells with plasmacytoid differentiation was observed. These cells were positive for B-lineage antigens in immunocytochemistry, and showed an immunoglobulin heavy-chain gene rearrangement in Southern blotting and chromosomal alteration in G-banded karyotype analysis. Although medicinal treatment was used, the patient died of general prostration. The diagnosis of intestinal MALT lymphoma was made at autopsy. Expression of API2-MALT1 fusion transcripts was detected by reverse transcription-polymerase chain reaction analysis using formalin-fixed, paraffin-embedded tissue. Intestinal MALT lymphomas with API2-MALT1 expression have distinctive forms of infiltration compared with those without translocation. Therefore, detection of API2-MALT1 fusion transcripts is useful for evaluating the prognosis and clinical behavior of the disease.
A retrospective study of 28 patients with primary gastrointestinal tract lymphoma is presented. There were 27 cases of non-Hodgkin's lymphoma and one case of Hodgkin's disease. The patients with non Hodgkin's lymphoma of the gastrointestinal tract represented 10% of all non-Hodgkin's lymphoma cases seen at the Royal Adelaide Hospital/Institute of Medical and Veterinary Science complex over the six year survey period, 1972-1977. Of the patients with non-Hodgkin's lymphoma, 26 cases were diffuse type, and one case was nodular type. There was a M/F sex preponderance of 2 . 8/1, and 70% of cases were aged between 40 and 69 years. The commonest site was the stomach (19 cases), followed by small intestine (7 cases), and one case involved large intestine. At initial presentation, the disease was confined to the affected viscus (Stage IE) in seven patients (25%), and in 12 patients (43%) the disease involved viscus and regional lymph nodes (State IIE). The one patient with Hodgkin's disease had involvement of the large intestine, abdominal lymph nodes and bone marrow (Stage IV). This study was retrospective, and a management protocol was not employed. However, of the seven patients presenting with Stage IE disease, six cases had diffuse poorly differentiated lymphocytic lymphoma. Five of these patients were treated by surgical resection alone, and were in complete remission at follow-up of 66 to 103 months. In order to compare realistically the survival of different groups of patients with primary gastrointestinal lymphoma, we consider that a prospective multicentre clinical trial with comprehensive staging procedures, uniform histological classification and accepted management protocol is warranted.
Intestinal lymphoma is frequently associated with sprue-like bowel patterns. A standardized approach to the problem is suggested.Intestinal lymphoma has been subdivided into four groups, depending on the extent of involvement of the anatomical region. Only cases with specific lesions are accepted as primary lymphoma. The villous pattern in 179 cases at necropsy with suitably preserved mucosa was determined to form a normal baseline for comparison with the uninvolved portions of the lymphomatous small bowel. Only 15% of all small bowel of this random material of children more than 1 year old and adults showed a flattened mucosa. The non-lymphomatous mucosa of 20 cases with definitely primary intestinal reticulum or lymphosarcoma showed severe sprue-like atrophy in 18 (90%). Two cases of intestinal Hodgkin's disease and four cases of gastric lymphoma were associated with regular mucosal patterns. It is concluded that sprue-like villous atrophy of the small bowel is definitely a triggering factor for the development of primary intestinal reticulum cell or lymphosarcoma. A hypothesis for the possible aetiological relationship of these two conditions is discussed.
BACKGROUND: The diagnosis of digestive bleeding in some cases can require a diagnostic laparotomy when other methods have been ineffective. Video-laparoscopy can provide such cases with a certain diagnosis and the possibility of performing a simultaneous surgical treatment. METHODS: Our experience using video-laparoscopic to treat digestive hemorrhages of obscure origin involved 16 patients. The examined patients presented with the following diseases: Meckel's diverticulum (8 cases), gastric leiomyoma (1 case), small bowel leiomyoma (4 cases), jejunum leiomyosarcoma (1 case), small bowel melanoma (1 case), and Peutz-Jeghers syndrome (1 case). RESULTS: In all these patients the laparoscopic approach allowed us to identify the origin and site of the hemorrhages, and subsequently to treat the identified lesions. In one patient, an intraperitoneal resection of the diverticulum was performed using Endo-Gia (TM30NIF; Tyco Auto-Suture). In the remaining cases, a minilaparatomy was performed for resection of the disordered loop. CONCLUSIONS: Video-laparoscopy is very useful for avoiding explorative laparatomies on patients affected by digestive hemorrhages of obscure origin. In these cases, the video-laparoscopic approach allows full and meticulous explorations of the small bowel to be performed, and seems to be useful for diagnosis through direct observation of the lesions. Moreover, if necessary, video-laparoscopy can be used for the effective treatment of the diseases.
Explore the source record for details and available documents.
This article has summarized associations of gastrointestinal disorders with cutaneous findings. An understanding of the cutaneous manifestations of gastrointestinal disease can raise the clinician's index of suspicion and provide clues to the successful diagnosis of internal disease.
Explore the source record for details and available documents.
CONTEXT: Gastrointestinal stromal tumors (GISTs) are specific, generally Kit (CD117)-positive, mesenchymal tumors of the gastrointestinal tract encompassing a majority of tumors previously considered gastrointestinal smooth muscle tumors. They are believed to originate from interstitial cells of Cajal or related stem cells. OBJECTIVE: To review current clinicopathologically relevant information on GIST. DATA SOURCES: Literature in Medline and authors' own experience. CONCLUSIONS: GISTs usually occur in older adults (median age 55-60 years) and rarely in children in the second decade (<1%) throughout the gastrointestinal tract: 60% in stomach, 35% in small intestine, and less than 5% in rectum, esophagus, omentum, and mesentery; most GISTs in the latter 2 sites are metastatic. Five percent of GISTs occur in patients with neurofibromatosis type 1 syndrome (multiple small intestinal tumors) and in Carney triad (gastric epithelioid GISTs in young females). Familial GISTs occur in patients with inheritable germline Kit or platelet-derived growth factor receptor alpha (PDGFRA) mutations. Histologically GISTs vary from spindle cell tumors to epithelioid and pleomorphic tumors. Most GISTs (95%) express Kit (CD117), CD34 (70%), and heavy caldesmon (80%), whereas 25% are positive for smooth muscle actin and less than 5% for desmin. Tumor size and mitotic activity are best predictive prognostic features; small intestinal tumors behave more aggressively than gastric tumors with similar parameters. Mutually exclusive gain-of-function Kit or PDGFRA mutations occur in a majority of GISTs representing in-frame deletions, point mutations, duplications and insertions. Mutations in Kit juxtamembrane domain (exon 11) are the most common in GISTs of all sites, whereas rare Kit extracellular domain (exon 9) Ala502-Tyr503 duplication is specific for intestinal GISTs. Mutations in PDGFRA have been identified in juxtamembrane (exon 12) and tyrosine kinase domains (exons 14 and 18), nearly exclusively in gastric GISTs, mostly in epithelioid variants. Some Kit and PDGFRA mutations have a prognostic value. Kit/PDGFRA tyrosine kinase inhibitor imatinib has been successfully used in the treatment of metastatic GISTs for more than 5 years. However, primary and acquired secondary resistance linked to certain types of Kit and PDGFRA mutations is limiting long-term success necessitating the use of alternative treatments.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Mutations in DNA mismatch repair (MMR) genes are associated with increased genomic instability and susceptibility to cancer. Mice rendered deficient in either Mlh1 or Pms2 as a result of gene targeting are prone to tumorigenesis, particularly, lymphomas. In addition, although Mlh1-/- mice also develop small intestinal adenomas and adenocarcinomas, Pms2-/- animals remain free of such tumors. To establish whether this phenotypic dichotomy might be associated with a quantitative and/or qualitative difference in genomic instability in these mice, we determined small intestinal epithelial cell DNA mutant frequency and mutation spectrum using a transgenic lambda-phage lacI reporter system. Mutant frequencies obtained from both Mlh1-/- and Pms2-/- mice revealed elevations of 18- and 13-fold, respectively, as compared to their wild-type littermates. Interestingly, we found that C : G-->T : A transitions were significantly elevated in Mlh1-/- mice, accounting in large measure for the 1.5-fold lacI mutant frequency increase seen in these animals. We hypothesize that the increased level of C : G-->T : A mutations may explain, in part, why Mlh1-/- mice, but not Pms2-/- mice, develop small intestinal tumors. Furthermore, the difference in the lacI mutational spectrum of Mlh1-/- and Pms2-/- mice suggests that other MutL-like heterodimers may play important roles in the repair of G : T mispairs arising within murine small intestinal epithelial cells.
CONTEXT: Many diffuse large B-cell lymphomas (DLBCLs) of the stomach are believed to represent high-grade transformation of low-grade marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type, which is of memory B-cell origin, displaying evidence for positive antigen selection and a low level of ongoing somatic mutation of the rearranged immunoglobulin heavy-chain variable region (V(H)) genes. The pattern of somatic mutation has been studied little in intestinal DLBCLs. OBJECTIVE: To assess evidence for antigen selection and the levels of ongoing mutation, we analyzed the ratio of replacement to silent mutations, as well as the frequency of intraclonal sequence variation in gastric and small intestinal DLBCLs that showed no concomitant low-grade component. DESIGN: Genomic DNA was extracted from formalin-fixed paraffin blocks of gastric (n = 6) and small intestinal (n = 6) DLBCLs. The complementarity-determining region 2 and framework region 3 sequences (<200 base pairs) of the rearranged immunoglobulin V(H) gene were obtained from polymerase chain reaction-amplified product, and the ratio of replacement-to-silent mutations and the frequency of intraclonal sequence variation were determined. RESULTS: Clustering of replacement mutations in complementarity-determining region 2 with a high (>2.9) ratio of replacement-to-silent mutations was observed in 5 gastric DLBCLs, whereas it was recognized in only 1 intestinal DLBCL. Intraclonal sequence variation was observed in 6 intestinal and 5 gastric DLBCLs. The frequency of ongoing mutation was much higher in the intestinal (median, 0.33%) than in the gastric DLBCLs (median, 0.13%), but the difference was not statistically significant (P =.09). CONCLUSIONS: The mutation pattern was consistent with positive antigen selection in gastric DLBCLs, but not in the intestinal tumors. Ongoing mutation was much more frequent in the intestinal than in the gastric DLBCLs. These findings suggest that positive antigen selection plays a major role in a significant proportion of gastric tumors, whereas germinal center reaction with aberrant mutation is important in small intestinal DLBCLs.
Small bowel obstruction is most frequently due to postoperative or inflammatory adhesions, intestinal neoplasms, hernias, or bezoars. Intermittent small bowel obstruction may be secondary to a Crohn's disease stricture or to chronic adhesive peritonitis. Enterolithiasis, usually associated with jejunal diverticulosis or with a Meckel diverticulum, should be considered in patients who have not previously undergone abdominal surgical procedures. X-ray evidence of stones in the abdominal field, outside the common sites, i.e. gallbladder, kidney, bladder, should suggest a diagnosis of enterolithiasis. The authors report a case of multiple enteroliths in a patient with a segmental ileal stricture and ulcerations (diagnosed as Crohn's disease) causing frequent, intermittent occlusive symptoms, treated by segmental ileal resection.
OBJECTIVES: To review the outcomes of the ureteroenteric strictures treated by endourological techniques in our department, and to compare our long-term results with other reported series with similar follow-up and number of patients. METHODS: We retrospectively reviewed 27 ureteroenteric strictures treated from March 1994 to June 2003, with a mean follow-up of 30.2 months (1 day-53 months). 13 cases underwent ballon dilation + permanent double J catheter (3 of them antegrade) 8 patients underwent endoscopical incision + double J catheter (5 of them with Acucise). RESULTS: 12/21 (57,14%) renal units improved and/or remained stable. We emphasize the absence of peroperative complications except 1 case that had a very poor oncological prognosis and died of septicemia 1 day after balloon dilation. CONCLUSIONS: Endourological treatment of ureteroenteric strictures has demonstrated to provide good fuctional results on the short and mid-term in patients that open surgery, although being the treatment of choice, would be too aggressive due to their disease, age, morbid conditions,....