Carcinoid syndrome

This code represents carcinoid syndrome, a paraneoplastic syndrome characterized by a collection of symptoms resulting from the systemic release of vasoactive substances, primarily serotonin, by neuroendocrine tumors (NETs), most commonly carcinoid tumors. These symptoms often include flushing, diarrhea, bronchospasm, and cardiac valvular lesions.

Use this code when documentation clearly indicates the presence of carcinoid syndrome, typically in patients with a confirmed or suspected neuroendocrine tumor. This diagnosis is supported by clinical signs and symptoms, elevated biochemical markers (e.g., 5-HIAA in urine), and imaging studies identifying the primary tumor.

• Always code the underlying neuroendocrine tumor (e.g., malignant carcinoid tumor of the small intestine) as the primary diagnosis when known, with carcinoid syndrome as a secondary diagnosis.
• Do not confuse carcinoid syndrome with carcinoid tumor itself; this code specifically describes the syndrome caused by the tumor.
• Ensure documentation specifies the symptoms present to support the diagnosis of the syndrome.