Neoplasms of the Endocrine Pancreas Follow-up

Updated: Feb 01, 2018
  • Author: Evan S Ong, MD, MS; Chief Editor: Neetu Radhakrishnan, MD  more...
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Follow-up

Further Outpatient Care

Patients who have undergone surgical resection of neoplasms of the endocrine pancreas require close follow-up with an endocrinologist or medical oncologist, and surveillance serum hormone levels should be tested regularly to ensure that recurrence is diagnosed early and treated appropriately.

Adjuvant radiotherapy after surgery might aid local control according to one retrospective study. Patients who received radiotherapy had high long-term survival rates and similarly low rates of recurrence compared to patients who did not undergo radiotherapy. However, patients who received radiotherapy were more likely to have larger tumors and node positivity. [112]

Proper outpatient treatment of patients with unresectable pancreatic endocrine tumors requires regular follow-up with endocrinologists and medical oncologists.

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Further Inpatient Care

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  • Patients who have complications from chemotherapy (eg, myelosuppression, renal failure, hepatic failure, severe gastrointestinal toxicity) may require inpatient treatment.

  • Patients with tumor recurrences that lead to the recurrence of symptomatic hormone-excess states may again require inpatient care to correct fluid, electrolyte, and nutritional disturbances.

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Inpatient & Outpatient Medications

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  • Interferon and cytotoxic chemotherapeutic agents are generally administered in a well-controlled outpatient or inpatient setting.

  • Patients can also consider enrollment in a clinical trial, such as those listed on the National Institutes of Health ClinicalTrials.gov Web site.

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Transfer

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  • Neoplasms of the endocrine pancreas are rare, their diagnosis and localization often require advanced technologies, and their successful treatment requires a careful multidisciplinary approach.

  • For these reasons, the patient with a possible or confirmed pancreatic endocrine tumor is best treated at a large referral center where endocrinologists, gastroenterologists, pancreatic surgeons, and medical oncologists are available.

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Complications

Late in the course of pancreatic endocrine tumors, patients can have morbidity related to the mass effects of the disease.

  • Patients with tumors in the pancreatic head occasionally have biliary obstruction, pancreatic obstruction, or both.

  • Chronic abdominal pain can occur because of the compressive effects of a large intra-abdominal mass or obstructive pancreatitis.

Morbidity resulting from the effects of excess hormonal production by functional neoplasms of the endocrine pancreas occurs sooner than the mass effects in the course of the disease.

  • If untreated, patients with insulinoma can have hypoglycemic seizures and even frank coma. [113]

  • Prior to the development of effective antisecretory medications (eg, H2 blockers, proton pump inhibitors), patients with gastrinoma often had life-threatening gastrointestinal bleeding from peptic ulcers.

  • Untreated patients with VIPoma can become severely dehydrated from diarrhea, and fatal cardiac arrhythmias can develop secondary to associated hypokalemia.

  • The morbidity associated with the hormonal effects of glucagonoma can be striking; patients can have diabetes, wasting, stomatitis, and other features similar to severe nutritional deficiency.

A study of 125 patients who underwent surgical resection of pancreatic neuroendocrine tumors between 1949 and 1996 had complications that included pancreatic fistula (16%), wound infection (15%), delayed gastric emptying (8%), and death (2.8%) [25] :

Permanent diabetes mellitus can mandate extended pancreatic tumor resection.

Complications of medical therapies are discussed in Medication.

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Prognosis

Endocrine tumors of the pancreas typically have indolent growth. Nevertheless, the rate of tumor growth is still a major determinant of survival in patients with these lesions. [17]

Because these tumors usually grow slowly and have a relatively low metastatic potential and because no specific criteria have been defined to predict their behavior, the distinction between benign and malignant neoplasms is based on the presence of metastatic disease; therefore, long-term clinical follow-up is often required. [18, 28]

Neoplasms of the endocrine pancreas have patterns of initial tumor spread that are similar to those of other gastrointestinal carcinomas, as follows:

  • The neoplasms spread first to regional lymph nodes and then to the liver

  • Bone metastases may occur late in the course of the disease and indicate a poor prognosis [15]

  • In rare cases, pancreatic endocrine tumors metastasize to the lungs or brain

  • Currently, the most important prognostic factor that determines the survival of patients with neoplasms of the endocrine pancreas is the presence or absence of metastatic disease.

Because even metastatic pancreatic endocrine neoplasms typically grow slowly, the prognosis in patients with these tumors is relatively good compared with that in patients with nonendocrine pancreatic cancers.

More than 90% of patients with insulinomas have benign neoplasms without evidence of metastases, and as many as 97% of these patients can be cured with surgical resection. [114]

Patients with gastrinomas have a poorer prognosis; 60% of these tumors are malignant. [115] However, survival rates are strikingly different between patients in whom gastrinoma metastasis is limited to the lymph nodes and those with hepatic metastases, as follows:

  • Patients in whom gastrinoma metastasis involves only the lymph nodes can live a quarter decade or longer, and their life expectancy is indistinguishable from that of patients with gastrinoma in whom no tumor is identified at surgery. [116, 117, 118]

  • Patients with hepatic gastrinoma metastases have a 5-year survival rate of 20-30%, compared with approximately 90% in patients without liver metastasis. [119]

Among pancreatic endocrine tumors, glucagonomas tend to be relatively large (5-10 cm) at diagnosis. Also, as many as 80% are invasive or metastatic; this frequency rate is higher than that of the other tumors. [46, 45, 120] Because glucagonoma syndrome is recognized relatively late in most patients, surgical cure is likely in fewer than 20% of all patients. [23]

Approximately half of all VIPomas are metastatic at the time of diagnosis or surgery, [23] and approximately one third of patients are cured with surgery. [121, 122]

Most somatostatinomas (84%) are metastatic at the time of presentation, but a number of patients survive 5 years after combination surgery and chemotherapy. [50, 48]

The malignancy rate of nonfunctioning pancreatic endocrine tumors varies from 64-92% in different series. [23] One series of 25 patients with nonfunctioning neoplasms of the endocrine pancreas had 3- and 5-year survival rates of 60% and 44%, respectively, although most patients (92%) had metastatic disease. [123]

Finally, radical surgery continues to have a central role in the therapy of endocrine tumors of the pancreas. Tumor resection, the absence of liver and lymph node metastases, and the presence of MEN 1 syndrome are related to a better survival rate. [124]

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