The use of a long-acting depot octreotide,
which can be administered on a monthly basis,
has largely obviated the need for patients to inject
themselves on a daily basis. However, patients
may also use short-acting octreotide injections for
PREVENTION AND MANAGEMENT OF
Carcinoid crisis is a life-threatening form of carcinoid syndrome triggered by specific events, presumably stimulating release of an overwhelming
amount of biologically active compounds such as
catecholamines. Specific symptoms include flushing, diarrhea, tachycardia, arrhythmias, hypertension or hypotension, bronchospasm, and altered
mental status. Symptoms are generally refractory
to fluid resuscitation and administration of vasopressors.
Carcinoid crisis may be precipitated by chemotherapy, anesthesia, or surgery; intraoperative
complications have been reported in 11% of patients who have carcinoid syndrome.45 Subcutaneous administration of octreotide 300 µg periopera-tively reduces the incidence of carcinoid crisis, and
intraoperative octreotide should be readily available during any surgical procedure. A continuous
intravenous drip of octreotide may also be used
during carcinoid crisis.33
The patient presents to his oncologist to
discuss options for managing his recur-
rent disease and symptoms of carcinoid syndrome.
The patient is started on therapy with short-acting
subcutaneous octreotide. After a 2-week trial of the
subcutaneous short-acting octreotide, he is transi-
tioned to the long-acting release depot formulation
given every 4 weeks. Shortly after starting therapy,
the patient’s symptoms of flushing and diarrhea
resolve. Restaging CT scans performed 3 months
after his diagnosis of metastatic carcinoid tumor
demonstrate overall stable disease. His disease is
radiographically stable and his symptoms related to
carcinoid syndrome are well controlled for approxi-
mately 1 year. However, at that point, he develops
symptoms of increasing right upper quadrant pain
and worsening diarrhea. Laboratory testing reveals
increases in his CgA level to 316.8 ng/mL and
24-hour urine 5-HIAA level to 40.3 mg/24 hour.
Additionally, restaging CT scans demonstrate an
increase in both the size and number of his liver
• What treatment options are available for pa-
tients with progressive metastatic disease?
Although patients with metastatic NETs may
pursue various treatment options, there is little consensus on a single, standard treatment approach.
The following section discusses the various treatment approaches that may be used.
In selected cases, metastatic liver disease can
be surgically resected. However, a high number of
liver metastases may preclude hepatic resection.
Several retrospective surgical series have suggested that patients who undergo either complete
resection or aggressive “debulking” of hepatic
metastases have improved quality of life and improved survival times compared with patients who
do not undergo surgery.46–50 The lack of formal ran-domization and potential for selection bias make
definitive interpretation of these results difficult.
Orthotopic liver transplantation (OLT) has been
attempted in few patients who have liver-isolated
metastatic disease.51–53 The impact of transplanta-