Appendiceal neuroendocrine tumors in single center literature review and a case series

Abstract

A 13-year-old Indian boy presented with right iliac fossa pain, vomiting, diarrhea and fever for the past 4 days. On further history taking, he also complained of weight loss about 7 kg in 1 month. Otherwise, there was no constitutional symptom. Patient has a strong family history of malignancy. Patient's mother was diagnosed breast cancer and patient's grandmother was diagnosed lingual cancer. Initial diagnosis was acute appendicitis. We proceeded with emergency open appendicectomy and intraoperative finding was inflamed retrocecal appendicitis, Tip of appendix was thickened and adhered deeply to the posterior peritoneum, otherwise caecal base was healthy and no Meckel's diverticulum found. Histopathology examination (HPE) result showed carcinoid tumor with tumor size 0.5cm and base was free. Immunohistochemistry staining for Synaptophysin: Positive, Chromogranin: Positive and CD56: Positive. Urine 5-HIAA result was normal. CT Thorax, Abdomen and Pelvis showed no evidence of distant metastasis. We plan to follow up this patient every 6 monthly clinically. Case 2: A 24-year-old Malay gentleman presented with right iliac fossa pain, diarrhea and fever for one day duration. Abdominal examination demonstrated tenderness over the right iliac fossa. Abdominal X-ray shows only fecal loaded over the ascending colon. Blood parameters were normal limits. Our initial diagnosis was acute appendicitis. We proceeded with emergency open appendicectomy. Intraoperative finding was perforated tip of appendix with 10cc pus but base was healthy. Patient was discharge home well at post operative day three. Patient was reviewed back in clinic in 1 month and the HPE result showed carcinoid tumor with tumor size 0.5cm, base and body is free. Immunohistochemistry staining for Synaptophysin: Positive, Chromogranin: Positive and CD56: Positive. Urine 5-HIAA and result was normal. CT Thorax, Abdomen and Pelvis showed no evidence of distant metastasis. We plan to follow up this patient every 6 monthly clinically.