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Malakoplakia of the gastrointestinal tract is a rare condition, with only 38 cases reported in the world literature so far. In Papua New Guinea 2 cases have been seen in young patients. These cases are described and the literature briefly reviewed.
Forty patients with neuromuscular disorders of the gastrointestinal tract were evaluated histologically and radiologically. Eighteen patients with progressive systemic sclerosis had predominant circular muscle thinning and fibrosis. Visceral myopathy (11 patients) was characterized by vacuolar degeneration of the smooth muscle cells with thinning and fibrosis typically affecting the longitudinal layers. Visceral neuropathy (five patients) had degeneration of myenteric plexus neurons with various patients having intranuclear inclusions, Schwann cell proliferation, or inflammatory cell infiltration. Radiologically, these syndromes had diffuse abnormalities of gastrointestinal motor function manifested by small and large intestinal dilatation, esophageal hypomotility (progressive systemic sclerosis and visceral myopathy), or disordered hypercontractility (visceral neuropathy). Marked duodenal enlargement typified visceral myopathy, and although all types may have dilated small intestine, only progressive systemic sclerosis has packing of valvulae. Colonic sacculations were found in progressive systemic sclerosis, lack of haustrations and increased colonic caliber in visceral myopathy, and hypercontractility in visceral neuropathy. Complete barium contrast examination will assist in differentiation of true obstruction from pseudoobstruction, will define the diffuse nature of the syndrome, and will help establish an accurate diagnosis by identifying features specific for these entities.
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Transcatheter arterial embolization was performed in three patients with cystic hepatic metastases from leiomyosarcoma of gastrointestinal tract. In one patient who had been alive and examined by CT scanning for more than 3 years, it was considered as valid for evaluating efficacy of TAE that the size reduction of cystic lesions followed the reduction of mural nodules and smoothing of marginal irregularity.
Mantle cell lymphoma (MCL) comprises 2.5%-7% of all non-Hodgkin's lymphomas, and the gastrointestinal tract is involved in about 20% of cases. Multiple lymphomatous polyposis (MLP) is an uncommon disease that is regarded as the intestinal form of MCL. We present a rare case of gastrointestinal MCL without MLP, and demonstrate that rituximab was effective for the treatment of this patient. A 61-year-old man presented with continuous diarrhea and hematochezia for a period of 5 months. Superficial lymph nodes were not palpable, but both tonsilla were enlarged. The level of soluble interleukin (IL)2-receptor was 3480 U/ml (normal <500 U/ml). Colonoscopy showed diffuse redness with erosion, without observation of any venous capillary, with these findings continuing from the rectum to the ileum. Upper gastrointestinal endoscopy showed a slightly rough gastric mucosal surface, and chicken-skin like mucosa was observed in the second portion of the duodenum. Small-to-medium size lymphoma cells were seen histologically from the tonsilla to the rectum. The lymphoma cells were immunohistochemically positive for CD5, CD20, CD79a, and cyclin D1. Polymerase chain reaction analysis revealed a chromosomal translocation t(11;14)(q13;q32) in the bcl-1 gene. We diagnosed this as a case of MCL from these findings. For treatment, the patient received a total of ten courses of combination chemotherapy consisting of cyclophosphamide (1000 mg), doxorubicin (70 mg), vincristine (2 mg) and prednisolone (50 mg) (CHOP), which led to a partial remission. However, 2.5 years later, massive infiltrations of the lymphoma cells were found in the colon and stomach. As the infiltrating lymphoma cells expressed CD20 molecules on their surfaces, the patient was treated with a chimeric anti-CD20 monoclohal antibody, rituximab, which showed significant efficacy, and a second partial remission was achieved.
Tumors of the gastrointestinal tract were induced in white non-inbred rats exposed to MNNG in various doses. Gastric tumors appeared in the dosage of 153 mg, with its 2 and 3.3 times increase no change in the frequency of gastric tumors was noted. The frequency of jejunal tumors was higher with increased MNNG dosage.
The bombesin group of hormonal peptides produced in the amphibian skin have powerful actions on the mammalian gastrointestinal tract. In man a bombesin-like material was found in an endocrine cell which is widely distributed in the gastrointestinal mucosa. This has tentatively been identified as belonging to the non-enterochromaffin cell groups. The large number of cells containing a bombesin-like peptide suggests that this substance may be important in human physiology.
Clinical data on photodynamic diagnosis for the detection of premalignant and malignant lesions in the gastrointestinal tract are encouraging so far. A major benefit of using autofluorescence is the lack of side effects because no sensitizer has to be applied.However, highly sophisticated detection systems are needed to enhance the weak autofluorescence-based fluorescent signal. New prototypes of autofluorescence video endoscopes are under way and will be decisive for further clinical use, especially because results of recently published studies have been disappointing.
We report a rare case of multiple lymphomatous polyposis of the gastrointestinal tract, with emphasis on the radiological, colonoscopic, and morphological findings. The underlying condition proved to be a poorly differentiated lymphocytic lymphoma. This is the first case of multiple lymphomatous polyposis in which immunologic marker studies are described and B cell origin confirmed. The different types of lymphoid polyps of the colon and the role of immunohistology are also discussed.
A combination of approaches has begun to elucidate the mechanisms of gastrointestinal development. This review describes progress over the last 20 years in understanding human gastrointestinal development, including data from both human and experimental animal studies that address molecular mechanisms. Rapid progress is being made in the identification of genes regulating gastrointestinal development. Genes directing initial formation of the endoderm as well as organ-specific patterning are beginning to be identified. Signaling pathways regulating the overall right-left asymmetry of the gastrointestinal tract and epithelial-mesenchymal interactions are being clarified. In searching for extrinsic developmental regulators, numerous candidate trophic factors have been proposed, but compelling evidence remains elusive. A critical gene that initiates pancreas development has been identified, as well as a number of genes regulating liver, stomach, and intestinal development. Mutations in genes affecting neural crest cell migration have been shown to give rise to Hirschsprung's disease. Considerable progress has been achieved in understanding specific phenomena, such as the transcription factors regulating expression of sucrase-isomaltase and fatty acid-binding protein. The challenge for the future is to integrate these data into a more complete understanding of the physiology of gastrointestinal development.
Recently, BMI-1 was identified as a protein downregulating p16ink4a and mandatory for the continued existence of several stem cell compartments like hematopoietic and neural stem cells. In this study we investigated BMI-1 expression as a potential stem cell marker of the gastrointestinal tract. We found weak expression in the isthmus region of the stomach, and moderate expression in crypts of the intestines, whereas intestinal surface epithelial cells were weakly positive or negative for BMI-1. In addition, a variety of highly differentiated cells such as parietal cells, neuroendocrine cells of the pylorus, Paneth cells and a subset of goblet cells were moderately to strongly positive for BMI-1. Furthermore, we detected strong expression in gastrointestinal neoplasias. This expression pattern indicates a correlation of BMI-1 expression with gastrointestinal stem cells as well as numerous specialized cell types and points to a role of this protein in cellular differentiation in addition to that of stem cell maintenance. Besides, our results imply a role for BMI-1 in the tumorigenesis of gastrointestinal cancer.
Polyglucosan inclusion bodies have been described in smooth muscle of the gastrointestinal tract of aged dogs, and rarely in association with enteric dysmotility in humans. We have systematically examined the human small and large bowel for the presence of such inclusions in health and motility disorders. Systematic, blinded, dual observer analysis of colonic and ileal tissue from patients (n=80, age 20-92 years) undergoing large bowel resections for non-dysmotile conditions, principally neoplasia was performed, as well as retrospective review of all intestinal tissues referred for specialist histochemistry from patients undergoing surgery for motility disorders. All sections were stained with haematoxylin and eosin and periodic acid-Schiff stains. No polyglucosan bodies were identified in any specimen without dysmotility, regardless of age, but were a feature of 4/104 patients with diverse severe gastrointestinal motility disorders. In contrast to dogs, polyglucosan bodies are not a feature of normal ageing in human gastrointestinal smooth muscle but, in accord with previous suggestions, are seen in rare cases of human gut dysmotility. The significance of this difference is unclear.
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About one third of leiomyosarcomas of the gastrointestinal tract do develop metastasis; and nearly 90% of the metastasis are intra-abdominal. It is said that leiomyosarcoma rarely involves regional nodes, although two series reported 8% of leiomyosarcomas of the stomach and 7% of the small intestines had nodal metastasis at initial operations (mostly of limited resections). It is suggested that wider resection of primary tumor with adjacent mesentery may reveal higher frequency of occult nodal metastasis and might prevent some of the loco-regional relapse and sarcomatosis.
PURPOSE OF REVIEW: Neuroendocrine tumors of the gastrointestinal tract represent a small area within oncology, but many new data have been reported during the past year. This paper updates the recent findings. RECENT FINDINGS: An N-terminally truncated variant of heat shock protein 70 (Hsp70) has been identified in neuroendocrine tumors but not in normal pancreatic islets. CDX-2 is a homeobox gene product essential for intestinal development and differentiation that is expressed at high levels in intestinal neuroendocrine tumors, but not in other types. A new marker has been identified, neuroendocrine secretory protein-55, a member of the chromogranin family, specific for endocrine pancreatic tumors but not for intestinal neuroendocrine tumors. Positron emission tomography has significantly improved imaging of neuroendocrine tumors of the gastroenteropancreatic tract. Tracers such as C-5-hydroxy-L-tryptophan show very high sensitivity for detection of tumors, higher than for somatostatin receptor scintigraphy. A new somatostatin analogue SOM230 binding to four of five somatostatin receptors has recently come into clinical trials. SUMMARY: Increased knowledge of the molecular background for the development of neuroendocrine tumors may improve the management of these tumors in the future. New tumor markers have been developed and the localization techniques have been significantly improved. That will of course lead to earlier diagnosis and improved possibilities for surgical cure of these patients.
Two cases of lymphomatoid granulomatosis affecting the gastrointestinal tract in the form of gastric ulceration and intestinal perforation are described. This rare presentation of lymphomatoid granulomatosis is discussed in the context of previous reviews of the disease, and it is suggested that clinically overt gastrointestinal involvement represents a very poor prognostic indicator in this condition.
The distribution of endocrine cells in the gastrointestinal tract of the house musk shrew, Suncus murinus (Family Soricidae, Order Insectivora) was studied immunohistochemically. The hormones investigated were gastrin, cholecystokinin (CCK), somatostatin, secretin, glucagon, gastric inhibitory polypeptide (GIP), motilin and neurotensin. In the gastric mucosa, gastrin and somatostatin cells were only found in the pyloric regions, and no other hormonal cell-types were observed. In the intestinal mucosa, the largest number of endocrine cells belonged to the gastrin and glucagon/glicentin cell-types, whereas CCK-33/39 and secretin cells were the least numerous. Numbers of other cell-types were intermediate between these two groups. The gastrin and GIP cells were mostly localized in the proximal portion of the intestine, decreasing in number towards the distal portion. The motilin and CCK-33/39 cells were restricted to the proximal half. The glucagon/glicentin and neurotensin cells were most abundant in the middle portion. The somatostatin and secretin cells, although only present in small numbers, were randomly distributed throughout the intestine. This characteristic distribution of gastrointestinal endocrine cells is discussed in comparison with the distribution patterns of other mammals.