Management of Neuroendocrine Tumors in Children, Adolescents, and Young Adults

Abstract

Neuroendocrine tumors, often referred to as carcinoid tumors, are relatively rare within the pediatric and young adult populations. However, when they do occur, the more aggressive tumors can be associated with significant morbidity and even mortality in this younger age group. This article reviews the history of pediatric neuroendocrine tumors, typical clinical presentation, appropriate diagnostic studies, staging, and treatment of this unusual cancer.

Appendiceal carcinoids in children—management and outcomes

“…The 2010 NANETS guidelines stated that "small (<1 cm) well-differentiated carcinoids confined to the tip of the appendix that are completely excised can be regarded as cured, if there is no evidence of lymphovascular invasion or invasion into the mesoappendix" [2,6] They recommended that patients with tumors <2 cm generally require no follow-up; however, given rare reports of regional lymph node disease in tumors <2 cm, other tumor characteristics should be taken into consideration in addition to size. Patients with mesoappendiceal invasion, high-grade tumors, or any tumors requiring right hemicolectomy need more intense follow-up according to the NANETS guidelines [6].…”

“…The outcomes of roughly 200 documented cases have guided the recommendations at this point [2]. Extended resections and colectomies are currently recommended for tumors >2 cm in adults, but the need for that in the pediatric age group is unclear as there are anecdotal reports of children with tumors >2 cm who have remained disease free for 10 y after appendectomy alone [4,5].…”

“…They are classified by embryologic origindforegut (airway), midgut (small bowel, appendix), or hindgut (rectum). Of these locations, the appendix is the most common site of disease [2]. Appendiceal carcinoids are most commonly discovered incidentally after appendectomy for acute appendicitis.…”

Well-differentiated neuroendocrine tumor of the stomach

“…Present recommendations for diagnosis, treatment, and long-term follow-up vary and are primarily derived from The North American Neuroendocrine Tumor Society (NANETS) adult guidelines. [3–7] …”

“…[10] The annual incidence of NET both in adults and children is estimated to be roughly 2 to 5 cases per 100,000 population. [6,7,10–12] …”