Low-grade appendiceal mucinous neoplasms (LAMNs) have been a very controversial tumor, and there is a lack of standardization for the optimal surgical procedure due to the infrequency of this disease. This is the first case report of duplicate appendix complicated by LAMN. The preoperative imaging examinations revealed that the mucinous tumor was shrinking spontaneously, allowing for safe laparoscopic resection. The histopathological findings indicated a dilated common base of the duplicated appendix, suggesting that the mucinous content drained spontaneously to the cecum. Further studies of the various complications of LAMNs are needed to establish the optimal surgical approach for LAMNs.
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