A Rare Case of Neuroendocrine Tumor Presenting as Lethal Hepatic Bleeding

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Introduction: Neuroendocrine tumors (NETs) of the gastrointestinal (GI) tract occur in about 5.25 individuals per 100,000 people. The distribution of NETs based on organ involvement is as follows: ileum with distal jejunum (25%), duodenum with proximal jejunum (22%), appendix (22%), colon (20%), rectum (10%), stomach (2 - 4%) and esophagus 0.05 %. Common symptoms of NETs include abdominal pain, diarrhea, cutaneous flushing, and sweating. Most NETs originated from appendix are indolent without symptoms. Here we presented a rare case of primary appendiceal NET presenting as lethal hepatic bleeding, leading to the demise of the patient.

Case Presentation: A 78-year-old woman presented with sudden onset of abdominal pain and somnolence. Physical examination revealed exquisite diffuse abdominal tenderness as well as distention. Vitals included a blood pressure 56/37 mmHg and she was immediately taken to the operating room for exploratory laparotomy secondary to suspicion of ischemic bowel. Exploratory laparotomy revealed a mesenteric mass approximately 3 cm in size adjacent to the mid jejunal area. The mass was excised and approximately 1 L of sanguineous fluid was drained. In the right upper quadrant of her abdomen, about 700 mL of blood clot surrounding the liver was found. Her liver was micronodular and cirrhotic and the source of bleeding was found to be in the right hepatic lobe. Biopsy of the mesentery nodule and liver taken were strongly positive for chromogranin and synaptophysin. The Ki-67 proliferative index was greater than 90 % consistent with the diagnosis of neuroendocrine carcinoma. Her postoperative course was complicated by stress induced gastric ulcers, disseminated intravascular coagulation, acute kidney injury, shocked liver, and fungemia with Candida glabrata and Candida albicans. The patient expired 12 days after massive hepatic hemorrhage.

Conclusion: Despite its generally indolent nature, primary appendiceal neuroendocrine tumors may rarely pursue an aggressive clinical course which must make clinicians highly aware and cautious during treatment and management.