Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes

Identifying necrolytic migratory erythema in glucagonoma syndrome

“…3 Other clinical manifestations of glucagonoma syndrome include venous thrombosis (up to 50%), neuropsychiatric manifestations (20%), chronic diarrhea (14% to 18%), and glossitis, stomatitis, or cheilitis (41%). [9][10][11][12] Of note, a normocytic normochromic anemia is present in up to 90% of patients. 9 The most common site for metastasis is the liver.…”

Diagnóstico y tratamiento del VIPoma o cólera pancreático.

“…La alta concentración de PIV produce una activación de la cAMP a nivel intracelular, produciendo en los enterocitos una secreción aumentada de electrólitos, seguida de su liberación, junto con agua, a la luz intestinal, lo que origina una diarrea profusa con hipovolemia, y en algunos casos, incluso una insuficiencia renal, que suele ser la causa más frecuente de muerte en tumores secretores de PIV (Farina et al, 2019;Ito & Jensen, 2021). Las alteraciones séricas de electrólitos son típicas del síndrome de WDHA.…”

Predictive Factors for Resistant Disease with Medical/Radiologic/Liver-Directed Anti-Tumor Treatments in Patients with Advanced Pancreatic Neuroendocrine Neoplasms: Recent Advances and Controversies

“…Recent studies report that PRRT, in addition to its anti-growth effects on NEN growth, is also highly effective for refractory functional panNEN syndromes, especially VIPoma’s, malignant insulinomas, and carcinoid syndrome [ 39 , 160 ]. Such an approach is rarely needed in the most common, malignant F-NEN syndrome, Zollinger-Ellison syndrome [ 1 , 158 ], because, at present, in contrast to the past [ 157 , 271 ], the acid hypersecretion [ 272 ] due to the ectopic secretion of gastrin by the gastrinoma [ 154 ], can be controlled by medical therapy (Proton pump inhibitors, histamine H 2 - receptor antagonists) in almost every patient, even those with multiple endocrine neoplasia-type 1 [ 157 , 271 , 273 , 274 , 275 , 276 ]. This effect of PRRT on functional NEN activity is independent of the antigrowth effect of PRRT [ 39 , 160 ].…”

“…These results suggest an increasingly limited role of liver-directed therapies options in NEN patients presenting with extrahepatic lesions. In the past, one of the main indications for liver-directed therapies in such patients was the presence of unresectable tumors causing a functioning tumor syndrome which was difficult to control [ 39 , 274 ]. However, with the increasing effectiveness of other less invasive therapies, such as molecular targeted therapies, SSA and PRRT, this is less of an indication for liver-directed therapies [ 39 , 274 ].…”

“…In the past, one of the main indications for liver-directed therapies in such patients was the presence of unresectable tumors causing a functioning tumor syndrome which was difficult to control [ 39 , 274 ]. However, with the increasing effectiveness of other less invasive therapies, such as molecular targeted therapies, SSA and PRRT, this is less of an indication for liver-directed therapies [ 39 , 274 ]. Because of the lack of well-established indication criteria for TACE, TAE and radio-embolization in panNEN patients, these histological factors may provide important clinical information in deciding on therapeutic strategies, specifically for patients whose tumor location is limited to the liver.…”