Carcinoid Crisis: A Dreaded Complication of Carcinoid Syndrome

Carcinoid crisis (CC) is a life-threatening complication of carcinoid syndrome (CS), which is characterized by hypotension, bronchospasm, and flushing can be precipitated by surgery and by drugs that release catecholamine and histamines.

Case Description:

A 62-year-old female with a history of HTN and obesity presented with complaints of dyspnea, generalized edema, fatigue which had worsened over the last month. Review of system was positive for chronic diarrhea since the last 30 years. She denied flushing, wheezing, sweating, palpitations. Vitals signs were stable. Physical examination revealed decreased breath sounds at the bases, grade 3 systolic murmur, anasarca. Lab work showed elevated creatinine of 2.57, eGFR 32. Pt underwent an echocardiogram which was suggestive of normal ejection fraction, severe tricuspid regurgitation. The tricuspid valve has the appearance of carcinoid heart disease with significantly fixed and retracted tricuspid valve leaflets.

Further lab work revealed elevated chromogranin A: 1425 (RR: 0 ng/ml), serum serotonin 1140 (N: 50-200 ng/ml), 5-HIAA(urine ratio to creatinine): 239 (N: 0-14mg/g). CT without contrast was suggestive of cirrhosis, and multiple hypodense hepatic lesions were identified. Lytic lesions were visualized within the manubrium, T10, L2, left humeral head which were worrisome for metastases. Subsequently, a liver biopsy confirmed the diagnosis of NET grade 1- carcinoid, which tested positive for chromogranin, synaptophysin, CDX2. KI–67 was less than 2%.

She was started on octreotide 200mcg TID and was scheduled for tricuspid valve replacement. Given extensive metastatic disease, she was not a candidate for surgical resection. To prevent carcinoid crisis, 12h preop she was transitioned to octreotide drip at 50mcg/hour. Postop period was complicated by carcinoid crisis. Rate of octreotide drip was maximized to 200mcg/h, in addition to being on norepinephrine, vasopressin, phenylephrine, milrinone drips. Pt required urgent RVAD placement on POD2. Her condition continued to worsen despite being on all the above pressors, and care was withdrawn by family members on POD5.

Discussion:

CC is a rare, underrecognized complication of CS. It can often be mistaken for septic shock. The pathophysiology of CC appears consistent with distributive shock. Perioperative octreotide, aimed at reducing serotonin release, is the most efficacious treatment for preventing crises during surgery. Despite prophylactic therapy with octreotide, carcinoid crisis is not entirely preventable and requires prompt recognition intraoperatively and aggressive treatment. The use of β-adrenergic agonists like epinephrine and norepinephrine is still controversial as it is thought to provoke release of mediators from the tumor and may worsen the syndrome.