Clinical features, frequency and prognosis of Dukes' A colorectal carcinoma: a population-based investigation.

The main aim of this study was, through the data of a population-based Registry, to establish the incidence of Dukes' A lesions by year of registration and the main clinical features, and to assess cancer-specific survival. One hundred and eighteen Dukes' A colorectal tumours were diagnosed (in 117 patients) out of 1337 registered between 1984 and 1992 in the Health Care District of Modena, Northern Italy; 94 patients were treated with surgery and 23 with endoscopic polypectomy. The frequency of Dukes' A tumours ranged between 4.8% and 18% by year of registration. Dukes' A carcinomas were significantly more frequent in the distal colon. Only 5 patients (4%) died of their cancer, and in all patients the tumour was localised in the rectum. Carcinomas associated with a poor prognosis did not show any of the biological variables usually associated with an unfavourable outcome, but, our data suggest the possibility of incomplete removal of tumours at surgery.

Certification of an emergency department according to UNI EN ISO 9002 criteria.

Over the past decade, the philosophy of total quality has appeared in all fields of industry in Japan and the USA. This philosophy is now present all over Europe as well, Italy included. In 1993 the Arcispedale Santa Maria Nuova of Reggio Emilia, a town in Northern Italy, began to apply the total quality management philosophy, thus embarking on a course of continuous quality improvement. The present article describes the main steps which led to an external accredited team granting the emergency department of this hospital a quality system certificate in 1997 according to the international quality system requirements UNI EN ISO 9002. The importance of this work lies in the fact than an industrial quality standardization system, whose requirements have little in common with a traditional view of medical practice, can nevertheless be applied to a public health care department.

Clinical and biologic features of adenomatosis coli in Northern Italy.

BACKGROUND: Familial adenomatous polyposis (FAP) is a hereditary disease characterized by more than 100 adenomas scattered in the large bowel and by various extracolonic manifestations. We proposed a) to establish the frequency of the disorder in Northern Italy, b) to describe the most relevant clinical findings, and c) in a subgroup of 21 patients (from 8 families), to evaluate the spectrum of mutations of the APC gene. METHODS AND RESULTS: Patients with FAP diagnosed between 1961 and 1991 were referred to our Study Group from surgery and gastroenterology units of the region Emilia-Romagna. The incidence of FAP was in the order of 1 in 16,500, with about a third of patients being 'single' cases. Colorectal malignancies were present in 75.6% of symptomatic patients but absent in most (93.75%) of the asymptomatic family members ('call-up' individuals). Gastric, duodenal, and jejunal adenomas were found in 8.2%, 30.6% and 53.3% of the investigated patients, respectively. Congenital hypertrophy of the retinal pigment epithelium and occult jaw lesions were seen in 64.7% and 39.5% of FAP patients but only in 0.5% and 2.5% of a matched, by age and sex, control population. These two clinical markers had a specificity of 99% and 97%, although their sensitivity was 64% and 39%. Finally, mutations of the APC gene were detected in 6 families (16 affected individuals) of the 8 families (21 affected individuals) tested; no correlation could be found between genotype and phenotype. CONCLUSIONS: This study confirms that early diagnosis is essential for an appropriate management of FAP patients, although this aim remains elusive in single cases. High-risk individuals are ideal candidates for APC gene mutation analysis, which should be offered to all first-degree relatives of affected patients.

Small bowel carcinoma in hereditary nonpolyposis colorectal cancer.

A 53-yr-old man, a member of a hereditary nonpolyposis colorectal cancer (HNPCC) family, with previous colonoscopic polypectomies, presented for persisting vomiting and marked signs of dehydration. Previous radiological and endoscopic examinations of the upper digestive tract were negative, with the exception of the presence of a duodenal adenomatous polyp. Enteroclysis led to a diagnosis of obstruction at the Treitz angle due to a moderately differentiated adenocarcinoma. Microsatellite instability was demonstrated in the DNA extracted from the tumor. The patient was the carrier of a mutation in the intron 13 of the hMLH1 gene, one of the four mismatch repair genes known to be responsible for HNPCC.

Frequency and clinical features of multiple tumors of the large bowel in the general population and in patients with hereditary colorectal carcinoma.

BACKGROUND: Reports on the frequency of multiple carcinomas of the colon and rectum have varied from 3-4% to more than 10% of all tumors of the large bowel. METHODS: We reviewed the files of a specialized colorectal cancer registry with the following objectives: a) to determine the frequency of multiple tumors (synchronous or metachronous) in the general population; b) to compare these values with those observed in patients with hereditary nonpolyposis colorectal carcinoma (HNPCC); and c) to evaluate the clinical outcome of patients with multiple tumors and the role of other clinical parameters in the development of these neoplasms. RESULTS: From 1984 to 1992, 53 patients with multiple tumors (of 1298 registered patients, 4%) had large bowel carcinoma; 33 (2.5%) were synchronous and 20 (1.5%) metachronous. The total number of multiple colorectal carcinomas was 95, which was 7% of all registered colorectal carcinomas (1337 carcinomas in 1298 patients). Multiple tumors occurred significantly more often in patients with HNPCC than in those with sporadic carcinomas (P < 0.001); this increased prevalence was more marked for metachronous lesions, which occurred almost 4 times more often in patients with HNPCC (5.8% vs. 1.3%; P < 0.001). The average interval of time between the first and the second malignancy was 8.7 years; there was no significant difference between hereditary and sporadic tumors. Patients with synchronous tumors did not show appreciable differences in survival when compared with individuals who had single neoplasms. In contrast, a poor clinical outcome was observed in patients with metachronous tumors after the development of the second carcinoma. Finally, polypoid adenomas of the large bowel were found significantly more often in patients with multiple primary tumors than in those with a single tumor. CONCLUSIONS: These results emphasize the importance of preoperative pancolonoscopy for the identification of possible synchronous tumors (both benign and malignant) and long-lasting endoscopic follow-up for the detection of recurrent or metachronous lesions. The conclusions are even more pertinent for patients with HNPCC, whose risk of metachronous tumors is significantly higher than that of patients with sporadic carcinoma.

Colorectal carcinoma in different age groups: a population-based investigation.

Although colorectal cancer is a disease of the older population, these tumors are not infrequent before the age of 55. Through the data of a population-based registry, we proposed giving a description of the clinical features of three groups of patients in whom the disease occurred at a relatively early age of onset (group I: < 40 yr; group II: 41-50 yr; group III: 51-55 yr). There were only 14 patients under the age of 40 yr (1.1% of total registered patients, n = 1298 in the period 1984-1992). Group II and III represented 5.9% and 6.0%, respectively (n = 76 and 78), with minor fluctuations throughout the 9-yr period of registration. Inherited colorectal tumors [hereditary nonpolyposis colorectal cancer (HNPCC), adenomatosis coli, and suspected HNPCC] accounted for 38.4% of group I patients (5 of 14), 17.1% of group II, 10.2% of group III, and only 3.5% of individuals older than 55 (p, for trend, < 0.001). Thus, hereditary colorectal tumors were detected significantly more often in younger individuals. The majority of colorectal malignancies were localized in the left colon or rectum in all three groups, with a tendency (not significant) to a preferential localization in the right colon for tumors developed in group I (37% vs 18% and 14% in groups II and III, respectively). Pathological stage and main histological types did not differ among the three groups. Finally, life-table analysis did not show significant differences in 5-yr survival among the three groups; however, when considered together, early onset cases showed a more favorable prognosis than older individuals (log-rank 11.6; p < 0.001). In conclusion, colorectal cancer is diagnosed very rarely before the age of 40 yr, whereas about 12% of all cases belong to the age group 41 to 55 yr of age. Hereditary tumors were found more frequently in younger patients, with a well-defined inverse relationship between age of onset and frequency of genetically determined tumors. Finally, the clinical outcome was more favorable in the whole series of early onset cases than in older registered patients.