We pick up on carcinoid syndrome by simply looking at what the patient's telling us. The signs that may accompany it may range from excess tearing, or lacrimation; periorbital edema; telangiectasia that usually is bimalar, making some patients look like they may have lupus; and then the flush. The flush is generally upper body. If it's a foregut carcinoid, it might involve the stomach; histamine release is something that may give rise to a rash that might be more systemic. Other things accompanying the syndrome might be palmar erythema or plantar erythema. There can be hepatic distention or abdominal distention related to the liver that would sometimes give rise to ascites. There can be edema. Carcinoid syndrome is relatively infrequent as it relates to the occurrence of the neoplasm. So it's a smaller number of patients that come to clinical attention than what we see in the pathology or the anatomical context.

The diagnosis of carcinoid disease comes about from looking at the clinical features and then following that up with the biochemical parameters, the 24-hour urinary 5-hydroxyindoleacetic acid or the blood level for chromogranin A. Then that can be extended into pancreatic polypeptide, neuron-specific enolase, and then localization of the disease can be done either on physical exam if there's a palpable mass-specifically, hepatomegaly-or an abdominal mass, pelvic exam, in the female, particularly. Then, ordering a test such as an abdominal CT scan, an OctreoScan, or another type of confirmatory tests might direct a physician to biopsy a mass someplace.

The treatment for carcinoid needs to center around what is going on with the patient. If there's nausea and vomiting, the treatment has to address those issues, so an NG tube would come before the specific diagnosis and potentially be done at the time of exploratory laparotomy. The medical options initially would be the somatostatin analogs. This would be where the disease is specifically being targeted with this form of treatment. The somatostatin analogs will bind to the either 1, 2, 3, 4 or 5 subtypes of the somatostatin receptors. Octreotide sees mostly subtype 2 and 5, a little bit of type 3, but we're left right now with no good binding to type 1 and type 4.

So if we can suppress the syndrome, we can suppress the flushing, the diarrhea, potentially the bronchoconstriction. If we can relieve the problems of the primary tumors, then we're well on our way to allowing these patients to live a long time, with a high quality of life.
From a physician's standpoint it's very rewarding, because these patients are coming back to you. They really feel that their treatment has made a difference in their lives. It's almost magic: we can now do things to control a disease process that otherwise looks hopeless.

Lowell Anthony MD