This is a real ClinQuiz case. In the app you start with 100 points, every investigation you open costs points and every wrong guess costs points too. Read the patient, open only the tests you need, then type your diagnosis. Everything below is also written out in full, so you can simply read it top to bottom.
The answer
Small intestinal neuroendocrine tumor (NET) with carcinoid syndrome
Carcinoid syndrome is a paraneoplastic syndrome caused by systemic release of vasoactive substances (serotonin, histamine, bradykinin, prostaglandins) from a neuroendocrine tumor, most commonly originating in the distal ileum with liver metastases.
Key points
- Carcinoid syndrome almost always implies hepatic metastatic disease because liver enzymes metabolize serotonin released into portal venous blood.
- Telotristat ethyl selectively inhibits serotonin synthesis by targeting tryptophan hydroxylase.
Presentation
Presents with episodic dry flushing (cutaneous vasodilation), secretory nocturnal diarrhea, abdominal cramping, bronchospasm (wheezing), and eventual carcinoid heart disease.
Pathophysiology
Circulating serotonin stimulates gastrointestinal motility and intestinal fluid secretion causing secretory diarrhea, while bradykinin and histamine cause cutaneous vasodilation (flushing) and bronchospasm.
Etiology
Primary gastrointestinal NETs release serotonin into the portal vein, where it is metabolized by hepatic monoamine oxidase. When hepatic metastases develop (or extraportal drainage occurs), vasoactive humors escape hepatic clearance directly into systemic circulation.
Diagnostics
Diagnosis is confirmed by elevated 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA) levels (end-product of serotonin metabolism), elevated serum Chromogranin A, CT/MRI localization, and 68Ga-DOTATATE PET-CT imaging.
Differential diagnosis
Differential diagnoses include menopausal hot flashes, systemic mastocytosis, pheochromocytoma, medullary thyroid cancer, and irritable bowel syndrome.
Management in this case
Initiate long-acting somatostatin analog therapy (lanreotide or octreotide LAR) and obtain an echocardiogram.
Symptom control and disease stabilization require long-acting somatostatin analogs (octreotide LAR or lanreotide), along with telotristat ethyl (tryptophan hydroxylase inhibitor) for refractory diarrhea, surgical resection, or liver-directed therapies.
Complications
Complications include carcinoid crisis (life-threatening hypotension, arrhythmia, and bronchospasm during surgery/stress), carcinoid heart disease (right-sided valvular lesions), severe bowel obstruction due to mesenteric desmoplasia, and pellagra (niacin deficiency).
Last updated 22 October 2026. Medical education only: all cases are simulated and must not guide real patient care.