PMID- 35794780
OWN - NLM
STAT- MEDLINE
DCOM- 20220729
LR  - 20231213
IS  - 1365-2826 (Electronic)
IS  - 0953-8194 (Linking)
VI  - 34
IP  - 7
DP  - 2022 Jul
TI  - Efficacy, safety and unmet needs of evolving medical treatments for carcinoid 
      syndrome.
PG  - e13174
LID - 10.1111/jne.13174 [doi]
AB  - This review reports on the currently available medical treatment options for the 
      control of symptoms due to carcinoid syndrome in patients with neuroendocrine 
      tumors. The efficacy and adverse events (AEs) of approved drugs such as 
      somatostatin analogues (SSA), telotristat ethyl (TE) and interferon-alpha, are 
      reviewed. Somatostatin analogues remain the standard treatment of carcinoid 
      syndrome based on the high expression of somatostatin receptors and the resulting 
      inhibition of secretion of bioactive compounds; their use is associated with 
      relatively mild AEs, involving mainly the gastrointestinal system, and being 
      usually transient. Although dose escalation of SSA remains an unapproved option, 
      it is clinically implemented to alleviate symptoms in refractory carcinoid 
      syndrome and supported by the most recent guidelines. The side effects associated 
      with the increased dose are in general mild and consistent with standard dose of 
      SSA. Telotristat ethyl, an oral inhibitor of tryptophan hydroxylase, the 
      rate-limiting enzyme in serotonin biosynthesis, represents a rather novel 
      innovative treatment option in patients with carcinoid syndrome suffering from 
      diarrhea and complements the standard therapy of SSA. Given the low toxicity 
      profile, TE may be considered an early add-on treatment to SSA in patients with 
      uncontrolled carcinoid syndrome. However, further prolonged follow-up of patients 
      treated with TE may be needed to exclude potential AEs, such as liver toxicity or 
      depressed mood, in patients with long-term treatment. Interferon alpha is a 
      cytokine with direct inhibitory effect on hormone secretion and tumor cell 
      proliferation and an approved therapy in carcinoid syndrome but is associated 
      with significant AEs in the majority of the patients requiring frequently dose 
      reduction. The finding of a more favorable tolerability of pegylated interferon 
      needs to be confirmed in a prospective study.
CI  - (c) 2022 British Society for Neuroendocrinology.
FAU - Koumarianou, Anna
AU  - Koumarianou A
AUID- ORCID: 0000-0002-4159-2511
AD  - Hematology-Oncology Unit, Fourth Department of Internal Medicine, Attikon 
      Hospital, Medical School, National and Kapodistrian University of Athens, Athens, 
      Greece.
FAU - Daskalakis, Kosmas
AU  - Daskalakis K
AUID- ORCID: 0000-0003-4224-8912
AD  - Department of Surgery, Faculty of Medicine and Health, Orebro University, Orebro, 
      Sweden.
AD  - 2nd Department of Surgery, "Korgialenio-Benakio", Red Cross General Hospital, 
      Athens, Greece.
FAU - Tsoli, Marina
AU  - Tsoli M
AD  - 1st Propaedeutic Department of Internal Medicine, National and Kapodistrian 
      University of Athens, Athens, Greece.
FAU - Kaltsas, Gregory
AU  - Kaltsas G
AD  - 1st Propaedeutic Department of Internal Medicine, National and Kapodistrian 
      University of Athens, Athens, Greece.
FAU - Pavel, Marianne
AU  - Pavel M
AD  - Department of Endocrinology, Universitatsklinikum Erlangen, Erlangen, Germany.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20220706
PL  - United States
TA  - J Neuroendocrinol
JT  - Journal of neuroendocrinology
JID - 8913461
RN  - 51110-01-1 (Somatostatin)
RN  - EC 1.14.16.4 (Tryptophan Hydroxylase)
SB  - IM
EIN - J Neuroendocrinol. 2024 Jan;36(1):e13361. PMID: 38088820
MH  - Diarrhea/complications/drug therapy/pathology
MH  - Humans
MH  - *Malignant Carcinoid Syndrome/complications/drug therapy/pathology
MH  - Somatostatin
MH  - Tryptophan Hydroxylase
OTO - NOTNLM
OT  - alpha-interferon
OT  - carcinoid syndrome
OT  - medical therapy
OT  - neuroendocrine tumors
OT  - somatostatin analogues
EDAT- 2022/07/08 06:00
MHDA- 2022/07/30 06:00
CRDT- 2022/07/07 00:57
PHST- 2022/04/06 00:00 [revised]
PHST- 2022/02/18 00:00 [received]
PHST- 2022/05/31 00:00 [accepted]
PHST- 2022/07/08 06:00 [pubmed]
PHST- 2022/07/30 06:00 [medline]
PHST- 2022/07/07 00:57 [entrez]
AID - 10.1111/jne.13174 [doi]
PST - ppublish
SO  - J Neuroendocrinol. 2022 Jul;34(7):e13174. doi: 10.1111/jne.13174. Epub 2022 Jul 
      6.