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Cronkhite-Canada syndrome. A case report and analytical review of 23 other cases reported in Japan.

A case study is presented of a 57-year-old male who showed typical clinical features of Cronkhite-Canada syndrome. Numerous polypoid lesions were found in the stomach, duodenum, ileum, colon and rectum accompanied with characteristic ectodermal changes. Tests indicated a protein-losing gastroenteropathy. Intestinal lactase deficiency was demonstrated by the lactose tolerance test. Scanning electronmicroscopy of the gastric and colonic mucosa revealed prominent secretion of mucoid substances and distortion in the gastric pits and colonic crypts. These abnormal findings were interpreted as having a direct relationship to the loss of protein into the gastrointestinal tract.

Adult↗

[Stomach cancer occurring after the radiation and chemical therapy of a lymphosarcoma].

A case of the development of gastric adenocarcinoma in a woman of 47 who for the previous 9 years had been treated for lymphoplasmocytic lymphosarcoma is described. The patient periodically received courses of polychemotherapy and twice radiotherapy on the stomach area because of the affection of the stomach detected by roentgenological examination and endoscopy with gastrobiopsy. In the past two years the patient underwent multiple endoscopic examinations demonstrating over time the changes in the gastric mucosa which initially showed superficial adenomatous outgrowths of the proliferating epithelium in the presence of marked atrophy and intestinal metaplasia. Gradually the signs of dysplasia increased in them and finally adenocarcinoma was established. Resection of the stomach revealed a highly differentiated adenocarcinoma in the form of an exophytic node 2 x 2 x 1 cm in size growing into the submucous layer.

Adenocarcinoma↗

Secretory immunoglobulin A in sweat gland tumors.

The presence of immunoglobulin A (IgA) and secretory component (SC) was investigated in normal human skin and in cutaneous neoplasms including a variety of sweat gland tumors. Immunohistochemistry in normal sweat glands revealed the occurrence of secretory IgA (sIgA) as indicated by reactivity for IgA and SC in serial sections. The majority of 28 cases of sweat gland tumors could be demonstrated to retain their ability to produce IgA and SC. In normal as well as in neoplastic sweat glands heaviest staining for sIgA could be found in the lumina and at the surface of lining epithelia. This is comparable with the presence of sIgA in breast or intestinal neoplasms. In contrast other epidermal cysts or solid tumors were not labelled. In view of recent immunohistochemical studies the demonstration of IgA and SC may be of differentiating value in cutaneous glandular neoplasms.

Breast Neoplasms↗

Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: a study of 17 cases.

The clinicopathologic features of neoplasms arising in gastrointestinal endometriosis have not been well characterized. In this series, we report 17 cases of gastrointestinal endometriosis complicated by neoplasms (14 cases) or precancerous changes (three cases). Four patients, one of whom also had hypermenorrhea, presented with chronic abdominal pain and five had obstructive symptoms; one of these also had rectal bleeding. One patient presented with an acute abdomen and fecal peritonitis, one had vaginal bleeding, and one had a progressive change in bowel habits. Nine patients had a long history of endometriosis, 11 patients had had hysterectomies, and eight of these had also received unopposed estrogen therapy. The lesions involved the rectum (6), sigmoid (6), colon, unspecified (2), and small intestine (3), and comprised 8 endometrioid adenocarcinomas (EA), 4 mullerian adenosarcomas (MAS), 1 endometrioid stromal sarcoma (ESS), 1 endometrioid adenofibroma of borderline malignancy (EBA) with carcinoma in situ, 2 atypical hyperplasias (AH), and one endometrioid adenocarcinoma in situ (ACIS). The tumors ranged in size from 2 to 15 cm and all involved the serosa and muscularis propria. Two tumors extended into the mucosa, with mucosal ulceration in one. Follow-up was available in 11 cases. One patient with EA was dead of disease at 1 year, one had two recurrences at 1 and 2 years, and three were alive with no evidence of disease (ANED) at 9 months to 13 years (mean, 68 mos). The patient with the EBA was ANED at 3 months. Two patients with MAS were ANED at 2 and 3 years. The patient with ESS had a recurrence at 3 years and was ANED 6 years after her original diagnosis. One woman with AH was ANED at 60 months and the patient with ACIS was ANED at 16 months. One of the carcinomas was originally misdiagnosed as a primary intestinal adenocarcinoma. The pathologist should be aware of the possibility of a tumor of genital tract type when evaluating intestinal neoplasms in females, particularly if they have a history of endometriosis and have received unopposed estrogen therapy.

Adenofibroma↗

[Gastric metastasis from melanoma. Report of 2 surgically treated cases].

Metastatic disease involving the gastrointestinal tract is a rare pathology and melanoma is the extra-intestinal neoplasm more frequently concerned. Two cases of gastric metastases, revealed by symptoms of upper gastrointestinal bleeding, are reported. In the first case the disease-free interval, after excision of the primary lesion located in the right lower limb, was 13 years; in the second case the primary lesion remained unknown, although it probably originated from a giant congenital nevus of the left foot. Both patients had been affected before by inguinal nodes metastases, treated by radical groin lymphadenectomy; the concomitant multiple metastases to other sites (adrenal glands, retro-peritoneum, liver, lung, small bowel, brain, ovaries) limited surgery to a likely prospect of palliation, conditioning an unfavourable prognosis. Resection of gastrointestinal metastases is justified for the relief of intestinal hemorrhage (as in these reported cases), perforation and obstruction, even if treatment of single non-complicated lesions can have a curative intent. The conclusion is drawn that more aggressive diagnostic and staging procedures are indicated for the early detection of gastrointestinal metastases whenever non-specific abdominal symptoms and a history of melanoma are present.

Aged↗

Immunohistochemical localization of heparan sulfate proteoglycan in human gastrointestinal tract.

Heparan sulfate proteoglycans (HS-PGs) are associated with important cell functions, for example, cell motility, cell adhesion, and oncogenesis. We examined the localization of HS-PGs in normal and carcinoma tissues of the gastrointestinal tract to help elucidate their roles in these organs. Fresh surgical materials from 134 patients with carcinoma of the stomach or large intestine and 26 patients with various diseases of the small intestine were immunostained after fixation with 10E4 (an antibody against the HS of HS-PG) as a primary antibody. Immunoelectron microscopy (immunogold method) was also performed. The basolateral surfaces of normal tissues of the large and small intestines were strongly stained with antibody confirmed by electron microscopy. In the stomach, lesions with intestinal metaplasia showed the same staining as the intestines, although normal gastric tissue showed staining only in some parts of the basal layer of fundic and pyloric glands. Carcinoma tissues in all cases examined showed staining with antibody. Better results were obtained after fixation in acetic alcohol or zinc-containing solutions than in ordinary formalin. These characteristic localizations of HS-PG in intestines and stomachs suggest that this kind of HS-PG staining could be a hallmark characteristic of the intestine.

Adult↗

Incidence of small bowel cancer in the United States and worldwide: geographic, temporal, and racial differences.

OBJECTIVE: To examine the demographic and geographic patterns of small bowel cancer incidence in the United States and worldwide. METHODS: Incidence data from the Surveillance, Epidemiology, and End Results (SEER) program between 1973 to 2000 were used to analyze the four histologic types of small bowel cancer, adenocarcinomas, carcinoid tumors, lymphomas, and sarcomas. International comparisons were made using data from Cancer Incidence in Five Continents (CIVIII). Geographic correlations between small bowel and both large bowel and stomach cancer incidence, were performed. RESULTS: Men had higher rates than women for all types of small bowel cancer. Blacks had almost double the incidence of carcinomas and carcinoid tumors compared to whites (10.6 vs. 5.6 per million people; 9.2 vs. 5.4 per million people, respectively). Small bowel cancer incidence has risen, with the greatest increase for carcinoid tumors (21%) and black men (120%). A geographic correlation between small and large bowel cancer incidence, but not small bowel and stomach cancer, were observed. CONCLUSIONS: Small bowel cancer incidence in the U.S. is higher in blacks compared to whites, particularly for carcinomas and carcinoid tumors. Small bowel cancer incidence is rising, particularly in black men. The geographic correlation between large and small bowel cancer suggests shared etiologies.

Adenocarcinoma↗

Chemopreventive activity of crude hydroxsymatairesinol (HMR) extract in Apc(Min) mice.

We studied the effects of a lignan, hydroxymatairesinol (HMR), and rye bran on intestinal tumor development in adenomatous polyposis colimultiple intestinal neoplasia (Apc)(Min) mice. HMR showed a strong chemopreventive effect in this animal model. The mean number of adenomas in the small intestine was significantly lower (26. 6+/-11.0, P<0.05) in mice fed the inulin and HMR when compared with the inulin and inulin/rye bran fed mice (39.6+/-8.9 and 36.0+/-7.4, respectively). HMR resulted in normalization of beta-catenin levels in adenoma tissue, indicating that HMR mediates its chemopreventive effect through the Apc-beta-catenin pathway. In the cytosolic fraction, beta-catenin level in adenoma tissue was significantly elevated (P=0.008-0.013) in all the diet groups as compared with that of the surrounding mucosa. In the nuclear fraction, beta-catenin in the inulin (3.15+/-2.9 relative units) and inulin/rye (5.17+/-6.94 relative units) groups was also significantly higher (P=0.003-0.009) in the adenoma tissue when compared with the surrounding mucosa (0.5+/-0.5 and 0.35+/-0.39 relative units). However, HMR was able to restore nuclear beta-catenin level of the adenoma tissue (0.41+/-0.25 relative units) to the level found in the surrounding mucosa (0.36+/-0.28 relative units).

Adenoma↗

Elevated (> or = 10%) MIB-1 proliferative index correlates with poor outcome in gastric stromal tumor patients: a study of 35 cases.

Mitotic activity and tumor size are currently regarded as the most powerful prognostic indicators for patients with gastrointestinal stromal tumor (GIST). This retrospective study evaluated the prognostic accuracy of MIB-1 proliferative index (PI) in combination with these two indicators in 35 GIST patients. Within a high-risk group, determined initially by tumor size and mitotic count, overall survival was significantly shorter for patients whose tumors had PI > or = 10% MIB-1 positive cells. When tumor location (gastric versus small intestine) was taken into account, a combination of tumor size, mitotic count, and PI > or = 10% identified a subgroup of patients with significantly shorter survival for gastric (but not small intestinal) GIST. Based on our results, MIB-1 immunostaining, when used in combination with tumor size and mitotic count, appears to be a powerful tool for identifying patients, especially those with gastric tumors, at high risk of recurrence and early tumor-related death.

Adult↗

Malignant melanoma: patterns of metastasis to the small bowel, reliability of imaging studies, and clinical relevance.

OBJECTIVES: The aims of this study were to determine the frequency of different patterns of melanoma metastases to small bowel on radiological examination, and to assess the reliability of the most commonly used radiological methods for detecting these lesions. METHODS: The records of cases archived as melanoma metastatic to the small bowel of the Armed Forces Institute of Pathology were reviewed. The clinical information, type of imaging procedure performed, and radiological features were analyzed and compared to the findings at surgery and at autopsy. RESULTS: A total of 32 patients had clinical and surgical data with pathological confirmation. Seven patients had metastasis involving the duodenum, 22 had jejunal involvement, and 11 had ileal involvement. Metastases were categorized as polypoid, cavitary, infiltrating, or exoenteric. The polypoid pattern was seen in 20 patients (63%), six of whom showed multiple polypoid lesions (>10), referred to as polyposis. The "target lesion," a discrete polypoid mass with a central ulceration, was observed in only three (9%) of the 32 patients. Eight patients (25%) demonstrated a cavitary pattern, a circumferential mass with inner marginal necrosis, and five (16%) showed an infiltrating pattern. One patient (3%) had an exoenteric lesion with a fistulous tract. The small bowel follow-through demonstrated 32 of 55 metastases (sensitivity 58%). Contrast-enhanced CT demonstrated 32 of 48 masses (sensitivity 66%). Of the six cases of malignant polyposis, none were identified using CT, and only two were diagnosed by small bowel follow-through. CONCLUSIONS: The polypoid pattern, equally distributed between the jejunum and ileum, is the most common manifestation of metastatic melanoma to the small bowel. The target lesion was infrequently seen in this series. Small bowel follow-through and conventional CT seem to be unreliable in demonstrating melanoma metastases to the small bowel.

Adult↗

AgNOR counts and determination of malignancy in stromal tumours of the stomach and small intestine.

Twenty four primary stromal tumours of the stomach and small intestine were investigated by silver staining of interphase nucleolar organiser regions (AgNORs) in an attempt to obtain an objective criterion for prediction of malignant tumour behaviour. Malignant tumours tended to have higher AgNOR counts than their benign counterparts, but this increase was small and there was some overlap between the two groups. There was a correlation between the mean AgNOR count and the mitotic count. There was no correlation between tumour size and these two measurements. This study suggests that in these stromal tumours the AgNOR count is not a useful independent predictor of malignant behaviour.

Adolescent↗

An unusual cause of subcutaneous emphysema, pneumomediastinum and pneumoperitoneum.

A 62 year old female with subcutaneous emphysema, pneumomediastinum and pneumoperitoneum, was observed. Pneumothorax, however, was not present. Laparotomy revealed a large infiltrate in the left lower abdomen, which had penetrated the anterior abdominal wall. Microscopically, a recurrence of previously diagnosed vulval carcinoma was demonstrated. Despite intensive treatment the patient died two months later.

Carcinoma, Squamous Cell↗