I have yet to walk away from a conversation about the management of incidentally found carcinoid tumors of the appendix satisfied. Today, I was determined to find some kind of an answer.
My basic understanding is that an incidentally found lesion which is < 2 cm and with negative margins is essentially treated with the appendectomy. Otherwise, a hemicolectomy is needed.
If we were to extrapolate form the adult literature, the simplest recommendation is that of the NCCN version 2.2014 (National Comprehensive Cancer Network). Simply stated, a tumor < or = 2cm completely removed with the specimen is essentially cured with no further operative management needed. Surveillance is performed "as clinically indicated". The document does acknowledge the fact that the management of tumors between 1 and 2 cm with poor prognostic factors such as mesoappendiceal invasion, lymphovascular invasion, or atypical histologic features, is controversial.
The NANETS (North American Neuro Endocrine Tumor Society) guidelines officially take into account local invasion. They recommend right hemicolectomy for patients with evidence of tumor invasion to the base of the appendix, if the tumor is > 2 cm, if tumor size cannot be determined, if tumor is incompletely resected, if there is lymphovascular invasion or invasion of the mesoappendix, in patients with intermediate or high grade tumors, and in patients with mixed histology tumors.
A recent paper, Kim et al reported their experience with the management of incidentally found carcinoid tumors. The authors noted that out of their 13 cases, only one tumor was larger than 2 cm (2.1 cm) and that was the only patient, out of three who had a right hemicolectomy, who was found to have regional metastatic disease.
As is the overall case with carcinoid of the appendix, the numbers are just too small to make solid recommendations, especially for tumors in the 1 to 2 cm range. A good conversation with the patient and family about the numbers and management options is central to decision making.
So much for finding an answer today.
If we were to extrapolate form the adult literature, the simplest recommendation is that of the NCCN version 2.2014 (National Comprehensive Cancer Network). Simply stated, a tumor < or = 2cm completely removed with the specimen is essentially cured with no further operative management needed. Surveillance is performed "as clinically indicated". The document does acknowledge the fact that the management of tumors between 1 and 2 cm with poor prognostic factors such as mesoappendiceal invasion, lymphovascular invasion, or atypical histologic features, is controversial.
The NANETS (North American Neuro Endocrine Tumor Society) guidelines officially take into account local invasion. They recommend right hemicolectomy for patients with evidence of tumor invasion to the base of the appendix, if the tumor is > 2 cm, if tumor size cannot be determined, if tumor is incompletely resected, if there is lymphovascular invasion or invasion of the mesoappendix, in patients with intermediate or high grade tumors, and in patients with mixed histology tumors.
A recent paper, Kim et al reported their experience with the management of incidentally found carcinoid tumors. The authors noted that out of their 13 cases, only one tumor was larger than 2 cm (2.1 cm) and that was the only patient, out of three who had a right hemicolectomy, who was found to have regional metastatic disease.
As is the overall case with carcinoid of the appendix, the numbers are just too small to make solid recommendations, especially for tumors in the 1 to 2 cm range. A good conversation with the patient and family about the numbers and management options is central to decision making.
So much for finding an answer today.
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