Introduction and importance: We report an extremely rare case of appendiceal neuroma, presented as an incarcerated Amyand’s hernia.
Case presentation: A 55-year-old male presented in the emergency department due to a persistent right inguinal painful swelling. Clinical examination revealed a 5cm x 3cm firm and irreducible right groin mass. Under the diagnosis of an incarcerated right inguinal hernia, an emergency operation was performed. Intraoperatively, a 5cm x 3cm firm mass originating from the distal part of the appendix was found in the hernia sac. The operation was completed with an appendectomy and a modified Bassini hernia repair. Histological examination revealed appendiceal neuroma, and no further surgical intervention was needed.
Discussion: Appendiceal neuroma is a benign stromal tumor whose diagnosis is typically made by histopathologic study. Though not clear, pathogenesis is believed to stem from the proliferation of neuroendocrine cells due to frequent inflammatory attacks. It is usually confused with the presentation of appendicitis, and the diagnosis is confirmed after the appendectomy. Amyand’s hernia, on the other hand, is an inguinal hernia whose content is either normal or diseased appendix vermiformis. In the literature, very limited case reports of Amyand’s hernia containing a carcinoid tumor, goblet cell carcinoid tumor, mucinous cystadenoma, adenocarcinoma, and villous adenoma are listed. However, we found no single reported case of Amyand’s hernia containing appendiceal neuromas in our search of English literature using Google Scholar and PubMed search engines.
Conclusion: Amyand’s hernia containing appendiceal neuroma is an extremely rare entity. We found no case report in the accessible literature. Histopathological evaluation is important to differentiate appendiceal neuroma from malignant tumors of the appendix. An appendectomy alone with hernia repair is adequate treatment.