Patients with widely metastatic disease require lifelong medical management. The role of debulking operations is controversial, and, in general, patients with metastatic disease should not be subjected to surgery unless the tumor can be completely resected for potential cure. Palliative operations may be appropriate for carefully selected patients.
Various chemotherapy regimens have been used in the treatment of metastatic enteropancreatic neuroendocrine tumors. Streptozocin plus 5-fluorouracil, with
Figure 16-12. Specimen photograph after the "Thompson operation." This 36-year-old man had a single small duodenal wall tumor that was removed through a duodenotomy (as in the patient in Figure 16-11). Adjacent to the duodenum was a large lymph node (6 cm) containing a neuroendocrine tumor that stained strongly for gastrin. Separately in the body of the pancreas, he had a very infiltrative tumor that stained only weakly for gastrin but strongly for pancreatic polypeptide and was adjacent to four involved lymph nodes with similar staining characteristics. He is disease free 1 year after operation.
or without doxorubicin, is the current therapy of choice for metastatic enteropancreatic neuroendocrine tumors.64,65 Because of the toxicity and generally poor response rates to chemotherapy, most clinicians believe that routine chemotherapy should be reserved for symptomatic patients or those with clear evidence of tumor progression on imaging studies.
The use of interferon has been investigated for patients with various pancreatic tumors, many of whom have failed chemotherapy. In one study, the overall response rate, defined as a decrease of > 50% in tumor size, was 77%, and patients with VIPoma appeared to respond the best.66 Octreotide is probably not useful as an antitumor agent.
Regional therapies include hepatic artery embolization or ligation and selective administration of streptozocin via the hepatic artery.67 68 It is important to recognize that these therapies are invasive and only palliative, and the side effects may be severe. On the other hand, because these tumors grow slowly, regional palliation may provide significant long-lasting symptomatic relief in carefully selected patients. Regional therapy is considered only after other options have been exhausted and is of limited value in controlling metastatic spread. However, hepatic artery embolization with or without interferon or chemotherapy may be very helpful in decreasing hormone levels, making controlling symptoms easier in patients with severe symptoms who are no longer responsive to octreotide.
The role of debulking surgery, as briefly mentioned, is controversial. Debulking surgery has been recommended in patients with VIPomas, glucagono-mas, somatostatinomas, and intestinal carcinoid tumors and may lead to a marked improvement in symptoms. Recently, nonoperative debulking has been used by radiofrequency ablation of liver tumors under ultrasonographic guidance. In general, debulking laparoscopic and open surgery should be considered in patients with uncontrolled hormonal syndromes who have exhausted the other treatment modalities.
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