Most appendiceal carcinoids (ACs) in children present without lymph node metastasis. Lymph node metastasis is rarely present when primary tumor diameter exceeds 1 cm. We present the extraordinary case of an AC with a primary tumor diameter of 0.7 cm and infiltration of the mesentery, as well as 1 positive lymph node of the mesentery in a 14-year-old boy. Besides adding a rare case, we review the data published in the current literature on AC with lymph node metastasis in children and summarize up-to-date guidelines for diagnostic workup, therapy, and follow-up.
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