Appendiceal carcinoid tumor in children: implications for less radical surgery?

Abstract

The discovery of an appendiceal carcinoid tumor found incidentally or during the course of diagnostic or therapeutic procedures is a burden to both the patient and clinician. The role of the correct surgical operation is paramount for lesions suspected to be malignant. In the pediatric population, appendiceal carcinoids continue to challenge the clinician in choosing the optimal treatment when lesions are larger than 2 cm or involve the appendical base. While the criteria used to define these distinct lesions are available in most cases, the management and treatment are still debated and controversial when considering more radical surgical intervention. The purpose of this article is to give an overview regarding the history, diagnosis, histopathology, management, and controversies associated with appendiceal carcinoid in the pediatric population.

Management of appendiceal carcinoid tumors in children

“…As a result, recommendations regarding management of these tumors in children are derived from adult data. In adults and children, patients with tumors <1.0 cm may be treated with appendectomy alone [1]. The treatment of those with a 1.0e2.0 cm tumor is less clear in both the pediatric and adult populations, and additional resection is based on patient-specific risk factors [2,3,7].…”

“…The treatment of those with a 1.0e2.0 cm tumor is less clear in both the pediatric and adult populations, and additional resection is based on patient-specific risk factors [2,3,7]. General indications for more extensive resection are based on the risk of more invasive disease and include size >2.0 cm, j o u r n a l o f s u r g i c a l r e s e a r c h x x x ( 2 0 1 5 ) 1 e4 involvement of the base, incomplete initial resection, lymphovascular or mesoappendiceal invasion, and the pathology findings of indeterminate or high grade, goblet cell or adenocarcinoid histology [1,2]. However, these indications for additional resection in children have been questioned in the pediatric literature due to the rarity of metastatic disease.…”

“…For adults, the North American Neuroendocrine Tumor Society consensus guidelines state that tumors <1.0 cm do not warrant additional dedicated follow-up, whereas 1e2 cm tumors should have follow-up based on the presence of poor prognostic factors [1,2]. Those with tumors >2 cm should undergo serial screening every 3e6 mo in the first year, and yearly for the next seven [1,2].…”

“…Appendiceal carcinoid tumors, also known as welldifferentiated neuroendocrine neoplasms, are rare, usually incidentally detected lesions in children at the time of appendectomy for acute appendicitis with a reported incidence of less than 0.1% [1]. Unlike in adults, where algorithms for additional surgery after incidental detection and postoperative surveillance are defined by a consensus guideline published in 2010 by the North American Neuroendocrine Tumor Society, no such recommendations exist in children [2].…”

“…Unlike in adults, where algorithms for additional surgery after incidental detection and postoperative surveillance are defined by a consensus guideline published in 2010 by the North American Neuroendocrine Tumor Society, no such recommendations exist in children [2]. A recent review in 2013 provided an algorithm for management in children, but these recommendations appear to be based on data from adult patients [1]. Barriers to the development of treatment algorithms in children include the rarity of the disease with limited singleinstitution experience and a lack of a defined natural history of these tumors [3].…”