Malignant endocrine tumors represent only 1% of digestive cancers, according to a study Burgundian

Malignant endocrine tumors represent only 1% of digestive cancers, according to a study in Burgundy and published in the journal "Gut".
A rare disease ...
"Few data are available on the epidemiology of malignant digestive endocrine tumors. This may be due to the rarity and heterogeneity of these diseases," recalls Dr. Lepage and his colleagues of the Burgundy digestive cancer registry in Dijon (Côte -d'Or).

The researchers then conducted the "first study" to assess the population level incidence and management of malignant digestive endocrine tumors. These tumors secrete hormones, such as gastrin secreting gastrinoma or insulinoma secreting insulin. To do this, they used the Burgundy digestive cancer registry that records all new cases in Côte-d'Or since 1976 and Saone-et-Loire since 1982.

In total, between 1976 and 1999, 229 cases of malignant digestive endocrine tumors were recorded, representing 0.8% of digestive cancers in men and 1.1% in women, the authors speak of a "rare" disease with a "slight predominance" in humans.

"From these data, we can estimate the expected number of cases in France 900," said the authors had when they presented their data in a poster Francophone Days digestive pathology.

Increased incidence of tumors in 24 years
The study also shows that in 24 years, the incidence of these tumors was significantly increased for both men and women, the rate is increased from 0.68 to 1.01 per 100,000 for men and 0.20 to 0.90 per 100,000 women between the first period (1976-1987) and the second (1988-1999).

"It is difficult to explain this trend. Improvement strategies or diagnostic criteria is an unlikely explanation for lesions of the gastrointestinal tract" and those affecting the pancreas. "The causative factors probably play a role, but they are not known because of the rarity of these tumors," the authors write.

The study also showed that the most common location of these tumors was the small intestine with 38.8% of the cases before the colon (27.1% of cases) and pancreas (20.5% of cases) . The sites were also affected, but to a lesser extent were the stomach, gallbladder, liver, esophagus, peritoneum, appendix ...

The average age of diagnosis was 70.5 years, with a number of cancers diagnosed at an advanced stage, 20% of patients with metastases in the lymph nodes and 53.3% of visceral metastases or unresectable tumors.

The proportion of unresectable tumors was 74.1%. There has been no improvement in the rate or the stage of disease at diagnosis during the study period, the authors note.

No chemotherapy was performed between 1976 and 1983, and the treatment was applied in 22.7% of cases.

The overall survival rate was 66.9% at 1 year, 50.4% at 5 years and 40.6% at ten years. The stage of disease at diagnosis, age at diagnosis and the site achieved are significant and independent prognostic factors.

"Although their incidence increases, digestive endocrine malignancies remain rare cancer, representing 1% of digestive cancers" but the prognosis is bleak across the population than the reported hospital series, conclude the researchers.