Neuroendocrine tumor of the small bowel and carcinoid syndrome: difficulties in diagnosis: a case report

Abstract

Background. Neuroendocrine tumors (NETs) of the small bowel are rare and slow-growing tumors arising from intraepithelial endocrine cells that synthesize serotonin. Diagnosis of these tumors poses a signifcant challenge because they are often not diagnosed until an advanced stage, since the tumor may be asymptomatic or accompanied by non-specifc gastrointestinal complaints. Approximately 40 % of patients develop carcinoid syndrome due to hormonal activity of NETs. Surgery is the mainstay treatment of locoregional small bowel NETs. The fve-year survival rate of patients is about 85 %, with a median rate of 9.3 years.Case description. The female patient complained of facial redness and, to a lesser extent, redness of the skin of the trunk, accompanied by a feeling of heat, severe headache, lacrimation, and general feeling of weakness. The patient unsuccessfully received symptomatic treatment prescribed by various specialists (gynecologist, therapist, psychiatrist, endocrinologist, etc.) for 14 years. Based on the comprehensive examination, NET of the small bowel was diagnosed. The patient underwent radical surgery (pT2N1M0, stage IIIB, G2), but taking into account the unfavorable prognostic factors (metastases in the mesenteric lymph node, presence of carcinoid syndrome, elevated biochemical markers, Ki67 level = 6 %, presence of somatostatin receptors of 2 and 5 types in 60 % of tumor cells), the patient was further treated with somatostatin analogues.Conclusion. When small bowel NETs are suspected, especially with the evidence of carcinoid syndrome, every effort should be made to confrm the diagnosis using a combination of anatomical and functional tumor imaging with biochemical markers.

Keywords

• net
• neuroendocrine biomarkers
• small bowel nets
• carcinoid syndrome
• carcinoid heart disease