Treatment of Gastrointestinal Neuroendocrine Tumors of the Appendix

Appendectomy.

The Italian Tumori Rari in Etá Pediatrica project performed a prospective registry study that evaluated 113 pediatric patients with appendiceal neuroendocrine tumors.[9][Level of evidence C1] Primary re-excision was not recommended for completely excised tumors smaller than 2 cm, except for microscopic/macroscopic residual tumor on the margins of the appendix. In these cases, cecum resection and pericecal node biopsy was recommended. Decisions about tumors larger than 2 cm were made at the discretion of the primary physicians. However, physicians were discouraged from performing right hemicolectomy unless margins were positive. Of the 113 study participants, 108 had tumors smaller than 2 cm. Thirty-five patients had extension of tumor beyond the appendiceal wall. Five tumors invaded the serosa, and 28 tumors invaded the periappendiceal fat. Margins were clear in 111 of 113 patients. The study concluded that appendectomy alone should be considered curative for most pediatric patients with appendiceal neuroendocrine tumors. The procedure of choice is a resection of the appendix without hemicolectomy.

The Italian Tumori Rari in Etá Pediatrica project performed a prospective registry study that evaluated 113 pediatric patients with appendiceal neuroendocrine tumors.[9][Level of evidence C1] Primary re-excision was not recommended for completely excised tumors smaller than 2 cm, except for microscopic/macroscopic residual tumor on the margins of the appendix. In these cases, cecum resection and pericecal node biopsy was recommended. Decisions about tumors larger than 2 cm were made at the discretion of the primary physicians. However, physicians were discouraged from performing right hemicolectomy unless margins were positive. Of the 113 study participants, 108 had tumors smaller than 2 cm. Thirty-five patients had extension of tumor beyond the appendiceal wall. Five tumors invaded the serosa, and 28 tumors invaded the periappendiceal fat. Margins were clear in 111 of 113 patients. The study concluded that appendectomy alone should be considered curative for most pediatric patients with appendiceal neuroendocrine tumors. The procedure of choice is a resection of the appendix without hemicolectomy.

At 41 months of follow-up, all 113 patients were alive.

The Italian Tumori Rari in Etá Pediatrica project performed a prospective registry study that evaluated 113 pediatric patients with appendiceal neuroendocrine tumors.[9][Level of evidence C1] Primary re-excision was not recommended for completely excised tumors smaller than 2 cm, except for microscopic/macroscopic residual tumor on the margins of the appendix. In these cases, cecum resection and pericecal node biopsy was recommended. Decisions about tumors larger than 2 cm were made at the discretion of the primary physicians. However, physicians were discouraged from performing right hemicolectomy unless margins were positive. Of the 113 study participants, 108 had tumors smaller than 2 cm. Thirty-five patients had extension of tumor beyond the appendiceal wall. Five tumors invaded the serosa, and 28 tumors invaded the periappendiceal fat. Margins were clear in 111 of 113 patients. The study concluded that appendectomy alone should be considered curative for most pediatric patients with appendiceal neuroendocrine tumors. The procedure of choice is a resection of the appendix without hemicolectomy.

The five patients with tumors larger than 2 cm did well.

The Italian Tumori Rari in Etá Pediatrica project performed a prospective registry study that evaluated 113 pediatric patients with appendiceal neuroendocrine tumors.[9][Level of evidence C1] Primary re-excision was not recommended for completely excised tumors smaller than 2 cm, except for microscopic/macroscopic residual tumor on the margins of the appendix. In these cases, cecum resection and pericecal node biopsy was recommended. Decisions about tumors larger than 2 cm were made at the discretion of the primary physicians. However, physicians were discouraged from performing right hemicolectomy unless margins were positive. Of the 113 study participants, 108 had tumors smaller than 2 cm. Thirty-five patients had extension of tumor beyond the appendiceal wall. Five tumors invaded the serosa, and 28 tumors invaded the periappendiceal fat. Margins were clear in 111 of 113 patients. The study concluded that appendectomy alone should be considered curative for most pediatric patients with appendiceal neuroendocrine tumors. The procedure of choice is a resection of the appendix without hemicolectomy.

One patient had resection of the cecum; no residual tumor was found.

The Italian Tumori Rari in Etá Pediatrica project performed a prospective registry study that evaluated 113 pediatric patients with appendiceal neuroendocrine tumors.[9][Level of evidence C1] Primary re-excision was not recommended for completely excised tumors smaller than 2 cm, except for microscopic/macroscopic residual tumor on the margins of the appendix. In these cases, cecum resection and pericecal node biopsy was recommended. Decisions about tumors larger than 2 cm were made at the discretion of the primary physicians. However, physicians were discouraged from performing right hemicolectomy unless margins were positive. Of the 113 study participants, 108 had tumors smaller than 2 cm. Thirty-five patients had extension of tumor beyond the appendiceal wall. Five tumors invaded the serosa, and 28 tumors invaded the periappendiceal fat. Margins were clear in 111 of 113 patients. The study concluded that appendectomy alone should be considered curative for most pediatric patients with appendiceal neuroendocrine tumors. The procedure of choice is a resection of the appendix without hemicolectomy.

One patient had a right hemicolectomy (tumor was <2 cm with clear margins, but an octreotide scan was possibly positive), and no tumor was found.

A French multicenter study of children younger than 18 years with neuroendocrine tumors of the appendix was carried out by surveying pediatric surgeons from 1988 to 2012. A total of 114 patients were identified. Risk factors for secondary right hemicolectomy were tumor extension into the mesoappendix, positive margins, size larger than 2 cm, and high proliferative index. Eighteen patients met the above criteria and were observed.[10]The investigator's recommendation was that appendectomy alone is sufficient treatment for neuroendocrine tumors of the appendix.

A French multicenter study of children younger than 18 years with neuroendocrine tumors of the appendix was carried out by surveying pediatric surgeons from 1988 to 2012. A total of 114 patients were identified. Risk factors for secondary right hemicolectomy were tumor extension into the mesoappendix, positive margins, size larger than 2 cm, and high proliferative index. Eighteen patients met the above criteria and were observed.[10]The investigator's recommendation was that appendectomy alone is sufficient treatment for neuroendocrine tumors of the appendix.

All patients were alive and had no disease at follow-up.

A French multicenter study of children younger than 18 years with neuroendocrine tumors of the appendix was carried out by surveying pediatric surgeons from 1988 to 2012. A total of 114 patients were identified. Risk factors for secondary right hemicolectomy were tumor extension into the mesoappendix, positive margins, size larger than 2 cm, and high proliferative index. Eighteen patients met the above criteria and were observed.[10]The investigator's recommendation was that appendectomy alone is sufficient treatment for neuroendocrine tumors of the appendix.

In addition, follow-up radiological studies and biological tests were not helpful.

A systematic review and meta-analysis of 38 studies of appendiceal neuroendocrine tumors identified 958 patients with a mean age at presentation of 11.6 years. Tumor size was 2 cm or smaller in 85% of the cases. Of the 24 papers that reported the status of the resection margin, 97% of tumors had negative margins. Nodal involvement was reported in ten series and was present in 1.4% of cases, with higher rates seen in patients whose tumors were larger than 2 cm (35%). Vascular involvement was seen in 11% of 510 patients, and invasion of the mesoappendix or periappendiceal fat was reported in 29% of 910 patients.[8]

A systematic review and meta-analysis of 38 studies of appendiceal neuroendocrine tumors identified 958 patients with a mean age at presentation of 11.6 years. Tumor size was 2 cm or smaller in 85% of the cases. Of the 24 papers that reported the status of the resection margin, 97% of tumors had negative margins. Nodal involvement was reported in ten series and was present in 1.4% of cases, with higher rates seen in patients whose tumors were larger than 2 cm (35%). Vascular involvement was seen in 11% of 510 patients, and invasion of the mesoappendix or periappendiceal fat was reported in 29% of 910 patients.[8]

According to the European and American Neuroendocrine Tumor Societies, 189 patients met the criteria for a secondary procedure after initial appendectomy, but only 69 patients underwent a secondary procedure (n = 43, hemicolectomy; n = 2, ileocecectomy; n = 1, cecectomy; n = 2, ileocolectomy; n = 21, not specified).

A systematic review and meta-analysis of 38 studies of appendiceal neuroendocrine tumors identified 958 patients with a mean age at presentation of 11.6 years. Tumor size was 2 cm or smaller in 85% of the cases. Of the 24 papers that reported the status of the resection margin, 97% of tumors had negative margins. Nodal involvement was reported in ten series and was present in 1.4% of cases, with higher rates seen in patients whose tumors were larger than 2 cm (35%). Vascular involvement was seen in 11% of 510 patients, and invasion of the mesoappendix or periappendiceal fat was reported in 29% of 910 patients.[8]

Of the 120 patients who did not have a secondary procedure, 91 patients had tumors extending to the mesoappendix, 5 patients had vascular invasion, 4 patients had positive margins, 12 patients had tumors 2 cm or larger, 1 patient had a high proliferative index, and 7 patients had positive lymph nodes.

A systematic review and meta-analysis of 38 studies of appendiceal neuroendocrine tumors identified 958 patients with a mean age at presentation of 11.6 years. Tumor size was 2 cm or smaller in 85% of the cases. Of the 24 papers that reported the status of the resection margin, 97% of tumors had negative margins. Nodal involvement was reported in ten series and was present in 1.4% of cases, with higher rates seen in patients whose tumors were larger than 2 cm (35%). Vascular involvement was seen in 11% of 510 patients, and invasion of the mesoappendix or periappendiceal fat was reported in 29% of 910 patients.[8]

No disease recurrences were reported in patients who had a secondary procedure or in those who were observed.

A systematic review and meta-analysis of 38 studies of appendiceal neuroendocrine tumors identified 958 patients with a mean age at presentation of 11.6 years. Tumor size was 2 cm or smaller in 85% of the cases. Of the 24 papers that reported the status of the resection margin, 97% of tumors had negative margins. Nodal involvement was reported in ten series and was present in 1.4% of cases, with higher rates seen in patients whose tumors were larger than 2 cm (35%). Vascular involvement was seen in 11% of 510 patients, and invasion of the mesoappendix or periappendiceal fat was reported in 29% of 910 patients.[8]

Preoperative and postoperative imaging was not helpful in managing patients.