Neuroendocrine tumors of the gastrointestinal tract: a decade of experience at the Princess Margaret Hospital.

OBJECTIVES

Neuroendocrine tumors (NETs) are uncommon malignancies with variable natural history and often indolent biological behavior. Over the past decade, novel treatment approaches have been developed. The purpose of this study was to review the experience at the Princess Margaret Hospital in treating patients with NET over the past decade.

METHODS

The Princess Margaret Hospital Cancer Registry identified 193 patients with NET of the gastrointestinal tract or unknown primary who had their initial visits between 1992 and 2002. A retrospective chart review was performed.

RESULTS

The median follow-up duration was 22.6 months. Demographics of the entire cohort of 193 patients were: male, 53%; mean age, 56 years; age-adjusted Charlson score 0, 71%. Anatomic distribution of the primary location revealed that 35% were from the foregut, 40% from midgut, 11% from hindgut, and 14% from unknown origin. Pathological distribution demonstrated that 72% were NET/carcinoids, 21% were islet cell tumors, and 6% were small cell carcinomas. At presentation, 53% of patients had distant metastases and 46% had loco-regional disease. Curative surgery was performed in 35% of the cohort, whereas radiotherapy and chemotherapy of any intent were delivered to 13% and 34%, respectively. For the entire cohort, 5-year survival was 58% (95% confidence interval, 50%-67%). In multivariate analysis, age, location of primary, and curative intent of surgery were independent predictors of survival. Of patients who underwent curative surgery, 86% were alive at 5 years.

CONCLUSION

Patients with NET who are offered curative surgery have a good outcome. Distant metastases are commonly found at presentation and portend a worse prognosis. These patients should be evaluated by a multidisciplinary team regarding new approaches to surgery and other therapies.