In view of the morbidity and frequency in familial cases an effective screening programme should aim at an early diagnosis of GEP particularly when truncating, especially nonsense mutations are found.
Carcinoid tumors occur most frequently in the gastrointestinal tract. Most of these tumors are clinically silent and the diagnosis is not made before surgery. The surgical treatment depends on the localization and the size of the tumor. Small bowel carcinoid tumors metastasize in 20-30% of the cases if the tumor is smaller than 1 cm. Therefore, the primary tumor should always be resected widely including the regional lymph nodes. Carcinoid tumors of the appendix less than 1 cm in size do not metastasize. For such patients an appendectomy is the treatment of choice. For tumors larger than 2 cm, a right hemicolectomy should be performed. If the tumor is between 1 and 2 cm, the surgical treatment depends on several factors (positive lymph nodes, extension of the tumor into the mesoappendix or subserosal lymphatic invasion, age of the patient). In young patients, an aggressive treatment is preferred. Carcinoid tumors of the colon and rectum less than 2 cm in size rarely metastasize. The surgical treatment for patients with tumors less than 2 cm is local excision, whereas for patients with tumors larger than 2 cm a wide resection is advocated.
Two cases of carcinoid tumors in Meckel's diverticula are reported. Additionally, data of 104 published cases of carcinoids in this rare location are analyzed. The average age of the patients is 56.6 years and corresponds to the age of patients with ileal carcinoids. Carcinoids in Meckel's diverticula also resemble ileal carcinoids in their biological behavior more than they do appendiceal carcinoids. Tumors larger than 5 mm have a marked risk to metastasize. By the time symptoms are present, 77 percent of these tumors have already metastasized. Men are affected by this tumor 2.5 times more often than women. Carcinoids in Meckel's diverticula metastasize twice as often in female as in male patients. More than 70 percent of carcinoids in Meckel's diverticula are found at the tips of the diverticula. An aggressive surgical management of tumors larger than 5 mm is recommended.
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