Case Presentation: A 51 year old female recently diagnosed with metastatic goblet cell carcinoid mixed adenocarcinoma of the appendix with metastasis to the peritoneum, mesentery, small and large bowel status post ileo-cecectomy and ileostomy presented with acute onset anxiety and mania. Patient presented with a two-day history of decreased sleep, flight of ideas and increased anxiety despite using anxiolytics. She began having worsening psychotic symptoms including hallucinations of her family watching her through the TV and tracking her on the Internet; inappropriate laughter, noises and gestures. Patient required hand restraints and intramuscular Haloperidol due to increased mania. CT head unremarkable. MRI of the brain without intracranial mass or metastatic lesions. Labs remarkable for elevated white blood cell count to 14.1. Chemistry and urinalysis were unremarkable. Stool studies negative and blood cultures remained without growth. Pancreatic polypeptide was notably elevated. EEG was normal. Given the negative workup outlined above and history of metastatic carcinoid tumor as well as elevated pancreatic polypeptide, there was concern for psychosis related to carcinoid syndrome. Olanzapine and Octreotide were started and symptoms improved significantly with decreased agitation, and cessation of hallucinations. Patient was discharged home on the above medications with return to baseline mental status.
Discussion: Carcinoids are neuroendocrine malignancies derived from cells characterized by their ability to produce and secrete vasoactive substances such as serotonin. Carcinoids are divided into foregut (respiratory tract, stomach, duodenum, and pancreas) midgut (ileum and appendix) and hindgut (left colon and rectum). Midgut tumors are especially active serotonin producers. Excess serotonin can cause carcinoid syndrome, which can present as diarrhea, skin flushing, heart valve dysfunction, bronchospasm, pellagra and uncommonly with psychiatric symptoms. Only two cases in the literature have reported the presence of psychosis with carcinoid tumor. We present a case report of a 51-year-old female who presented with psychosis in the context of metastatic neuroendocrine carcinoid cancer of the appendix. Neuroendocrine malignancies of the midgut such as appendix are especially active serotonin producers. Carcinoid syndrome due to excess serotonin production is more likely with metastatic carcinoid cancer. The mechanism precipitating psychosis in carcinoid syndrome is not fully understood but includes hormonal effect from the tumor; niacin deficiency due to metabolic diversion of its precursor tryptophan to form serotonin. Diagnosis and treatment of psychosis associated with carcinoid syndrome are challenging. Treatment options include tumor resection, antipsychotics, somatostatin analogue and avoidance of SSRI(Selective serotonin reuptake inhibitors).
Conclusions: We present an extremely rare case of psychosis associated with neuroendocrine carcinoid cancer of the appendix. The recognition of psychosis as a presenting and unusual symptom of carcinoid syndrome is essential for proper treatment and management of this complicated and uncommon condition.