A Carcinoid Syndrome Case Study
The following case illustrates the importance of biochemical monitoring in carcinoid syndrome. Look here for:
History
- 46-year-old male construction worker previously in good health, except for mild chronic hypertension
- Patient had self-medicated with an OTC antidiarrheal medication with mixed results
Presentation/Diagnosis
- Patient presented with symptoms including occasional facial flushing, mild diarrhea (1 to 2 watery stools/day), and abdominal cramping
- Biochemical workup revealed urinary 5-HIAA levels of 30 mg/24 hours (normal range: 3-15 mg/24 hours) and CgA level of 148 mcg/L (normal value: 100 mcg/L)
- CT scan of the abdomen and scintigraphy revealed a tumor in the midgut
- Needle biopsy was interpreted as typical carcinoid disease
Intervention
Surgery was performed to remove a 2-cm tumor, which was resected with apparently clear margins
Follow-up
12 months postsurgery
- CT scan revealed two metastatic liver lesions (of approximately 1 cm and 4 cm)
- Urinary 5-HIAA increased to 75 mg/24 hours
- CgA level increased to 305 mcg/L
- Patient claimed there was no change in mild diarrhea, abdominal cramping, and flushing
18 months postsurgery
- CT scan revealed two additional liver lesions and slight increase in the size of the other liver lesions
- Urinary 5-HIAA levels increased to 130 mg/24 hours
- CgA level increased to 520 mcg/L
Key Opinion Leader Analysis
Case analysis
- This case exemplifies a situation where progressive disease is occurring biochemically and radiographically, but not necessarily clinically. Symptoms of carcinoid syndrome were present at the time of initial diagnosis and persisted over the next 18 months.
- Appropriate initial intervention, in the setting of metastatic disease, includes removal of a symptomatic primary tumor, as well as the consideration of a prophylactic cholecystectomy. Surveillance documents progressive but "low-bulk" disease.
- Controlling the disease becomes the challenge of clinicians who, over the last two decades, have had an increasing number of options to consider either during or after surgical intervention. Using medical therapy to control symptoms is considered a standard approach. The potential benefits include not only suppressing symptoms but also lowering biochemical markers by up to 50%.
- Consequences of progressive disease include increasing carcinoid syndrome symptoms and the risk of carcinoid heart disease. The latter usually occurs in the setting of a number of years of uncontrolled disease and 5-HIAA values exceeding 100–150 mg/24 hours.
- With the appropriate recognition and suspicion of carcinoid heart disease, intervention to control the underlying disease is the major challenge.
- Medical therapy, used with or without debulking measures, helps to control carcinoid syndrome symptoms and may either prevent or delay the onset of complications of the disease, such as tricuspid insufficiency and/or pulmonic stenosis.*
* Zuetenhorst JM, Bonfrer JM, Korse CM, et al. Carcinoid heart disease: the role of urinary 5-hydroxyindoleacetic acid excretion and plasma levels of atrial natriuretic peptide, transforming growth factor-beta and fibroblast growth factor. Cancer. 2003;97:1609-1615.
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