Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
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DOI：
10.1080/14656566.2020.1845651
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发表时间：
2021-04
影响因子：
3.2
通讯作者：
Jensen RT
中科院分区：
文献类型：
--
作者：
Ito T;Jensen RT
In the past the inability to control the hormone-excess-state was the main determinant of survival in patients with Functional Neuroendocrine Neoplasm syndromes (F-NENs). This could prove especially difficult because the pharmacological armamentarium available until relatively recently was limited. In the last few years there have been a marked increase in the therapeutic strategies available which have opened new pharmacotherapeutic approaches, but have also generated some controversies, uncertainties and confusion. In this perspective, the authors briefly review the different F-NENs that are established as well as those proposed; the rationale for approaching their treatment and why both an approach to controlling their hormone-excess-state and their malignant nature is required in most cases; the current recommended initial pharmacotheraputic approach to controlling the hormone-excess-state of the different F-NENs; the secondary approaches to controlling the hormone-excess-state if the initial approach fails or resistance develops; and the approach to deal with the malignant nature of the NEN per se. Also discussed are the controversies the new treatments have generated particularly related to the timing of the diagnosis of the F-NEN, as well as the sequences of secondary treatments, and the exact role of PRRT. Unfortunately, except for patients with insulinomas(>90-95%), gastrinomas(<20-40%), a minority with the other F-panNEN syndromes and 0-<1% with Carcinoid syndrome is curative surgery possible allowing control of the hormone-excess-state and the malignant nature of most NENs(except insulinomas-being-95%). Except for insulinomas, gastrinomas and ACTHomas, long-acting somatostatin analogues are the initial pharmacological treatments for hormone-excess-state. For insulinomas prior to surgery/malignant insulinomas, diazoxide/frequent,small feedings are the initial treatments; for gastrinomas, oral PPIs need to be started as soon as possible and for ACTHomas Steroidogenesis inhibitors are generally initially used. There are now a number of secondary pharmacotherapeutic treatments for the different syndromes, including older drugs which are effective in some patients as well as newer therapies. These include telotristast for Carcinoid syndrome[primarily for the diarrhea, but also helps flushing in some patients]; mTor inhibitors(everolimus); pasireotide; and a number of newer agents effective in patients with ectopic Cushing syndrome. Particularly promising, is recent results with PRRT for the hormone-excess-state, independent of its anti-growth effect. The sequence to use various agents and the approach to syndrome diagnosis while taking various agents remains unclear/controversial in many cases. In the past controlling the hormone-excess-state was the main determinant of survival in Functional-Neuroendocrine-Neoplasm-syndromes(F-NENs). This was difficult because the pharmacological-armamentarium available was limited. More recently therapeutic strategies available have increased, opening new pharmacotherapeutic-approaches,but also generated controversies/uncertainties. In this perspective, the authors briefly review current established/proposed F-NENs; the rationale for treatments; the current recommended initial-pharmacotheraputic-approach to controlling F-NENs hormone-excess-state; the secondary-approaches if the initial approach fails or resistance develops; and the approach to deal with the malignant nature of the NEN per se. Also discussed are controversies/uncertainties related to the new treatments. Unfortunately,except for patients with insulinomas(>90-95%), gastrinomas(<20-40%),a minority with the other F-panNENs and 0-<1% with Carcinoid-syndrome is curative-surgery possible. Except for insulinomas,gastrinomas and ACTHomas,long-acting somatostatin-analogues are the initial-pharmacological-treatments for hormone-excess-state. For insulinomas prior to surgery/malignant,diazoxide is initial drug-treatment; for gastrinomas,oral PPIs; and for ACTHomas Steroidogenesis inhibitors. There are now a number of secondary pharmacotherapeutic treatments for the different F-NEN-syndromes.These include telotristast(Carcinoid-syndrome; mTor- inhibitors(everolimus); pasireotide; and some newer agents in ectopic Cushing-syndrome. Particularly promising,is recent results with PRRT for the hormone-excess-state,independent of its anti-growth effect. The sequence to use various agents and the approach to syndrome diagnosis while taking various agents remains unclear/controversial in many cases.
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影响因子：
28.4
作者：
Dasari, Arvind;Shen, Chan;Yao, James C.
通讯作者：
Yao, James C.
影响因子：
5.9
作者：
BENYA, RV;METZ, DC;JENSEN, RT
通讯作者：
JENSEN, RT
影响因子：
4.1
作者：
Falconi M;Eriksson B;Kaltsas G;Bartsch DK;Capdevila J;Caplin M;Kos-Kudla B;Kwekkeboom D;Rindi G;Klöppel G;Reed N;Kianmanesh R;Jensen RT;Vienna Consensus Conference participants
通讯作者：
Vienna Consensus Conference participants
影响因子：
39.2
作者：
FISHBEYN, VA;NORTON, JA;JENSEN, RT
通讯作者：
JENSEN, RT
影响因子：
39.2
作者：
COLLEN, MJ;HOWARD, JM;JENSEN, RT
通讯作者：
JENSEN, RT