A case of well-differentiated neuroendocrine tumor of the appendix in a 14-year-old male adolescent clinically presented with features of acute appendicitis: A case report and literature review.

INTRODUCTION AND

IMPORTANCE

Neuroendocrine neoplasms (NENs) of the appendix are rare in children even though they are the second commonest gastrointestinal tumor next to lymphoma. NENs are usually diagnosed incidentally in appendectomies done for appendicitis, occasionally resulting in a second operation to ensure adequacy of the management. This case report is aimed at emphasizing the significance of subjecting any appendectomy specimen to histopathologic examination, irrespective of the indication for appendectomy.

PRESENTATION OF CASE: Here we presented a case of a 14-year-old male adolescent who presented with right lower quadrant abdominal pain of 16 h duration. He had leukocytosis with left shift, and the abdominal ultrasound examination concluded acute appendicitis, for which an emergency appendectomy was done. The histopathologic examination of the appendix along with the immunohistochemistry findings for synaptophysin concluded well differentiated neuroendocrine tumor WHO grade 1, AJCC stage PT3NxMx. Subsequent right hemicolectomy with lymphadenectomy was done due to the stage of the tumor.

CLINICAL DISCUSSION: Appendiceal NENs are extremely uncommon in the global pediatric population and have benign behavior. The tip of the appendix is the commonest site of origin. Surgery is the mainstay of treatment, and size is the most determinant factor in determining the extent of surgery.

CONCLUSION

Since most appendiceal neuroendocrine tumors are diagnosed incidentally, every appendectomy tissue should be submitted for histopathologic examination to identify the presence of a tumor and determine its stage.