pNETs are divided into functional versus non-functional tumors with about 90% being classified as non-functional. Commonly, tumors are defined as non-functional if the patient does not suffer from symptoms due to hormone hyper-secretion, even if hormone levels are elevated on laboratory evaluation. Most non-functional pNETs present with symptoms due to mass effect, such as jaundice, weight loss, abdominal pain, palpable mass, nausea/emesis, pancreatitis, or back pain, and mimic the presentation of pancreatic adenocarcinoma. As routine diagnostic imaging becomes more prevalent, some patients present asymptomatically with an incidental finding on cross sectional imaging. Most patients present with metastatic (60%) or locally advanced disease (21%) (3).
As mentioned before, pNETs are functional 10% of the time. The presenting symptoms of functional tumors depend on the particular hormone that is being overproduced. The most common …show more content…
The most common presenting symptom of glucagonomas is a dermatitis called migratory necrolytic erythema, consisting of erythematous lesions that become necrotic and develop pigmented scarring (34). Other common symptoms from glucagonomas include glucose intolerance, weight loss, diarrhea, and deep vein thrombosis (DVT). Together these symptoms are sometimes referred to as the 4D syndrome (dermatitis, diabetes, diarrhea, DVT) (8,11). VIPomas secrete vasoactive intestinal polypeptide and result in symptoms of large volume watery diarrhea and hypokalemia. Somatostatinomas comprise less than 5% of pNETs. They secrete somatostatin and can cause diabetes mellitus, gallbladder disease, diarrhea/steatorrhea, anemia, and weight loss. Somatostatinomas have the subtlest syndrome of any of the functional pNETs and rarely is the syndrome present in its entirety in a single patient (8,11,35). The various syndromes associated with functional pNETs are summarized in Table
As mentioned before, pNETs are functional 10% of the time. The presenting symptoms of functional tumors depend on the particular hormone that is being overproduced. The most common …show more content…
The most common presenting symptom of glucagonomas is a dermatitis called migratory necrolytic erythema, consisting of erythematous lesions that become necrotic and develop pigmented scarring (34). Other common symptoms from glucagonomas include glucose intolerance, weight loss, diarrhea, and deep vein thrombosis (DVT). Together these symptoms are sometimes referred to as the 4D syndrome (dermatitis, diabetes, diarrhea, DVT) (8,11). VIPomas secrete vasoactive intestinal polypeptide and result in symptoms of large volume watery diarrhea and hypokalemia. Somatostatinomas comprise less than 5% of pNETs. They secrete somatostatin and can cause diabetes mellitus, gallbladder disease, diarrhea/steatorrhea, anemia, and weight loss. Somatostatinomas have the subtlest syndrome of any of the functional pNETs and rarely is the syndrome present in its entirety in a single patient (8,11,35). The various syndromes associated with functional pNETs are summarized in Table