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      First-generation somatostatin ligand receptor treatment in a pregnant patient with a neuroendocrine tumor with liver metastases

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          Abstract

          Summary

          This rare case describes the course of a pregnancy in a patient with a disseminated small intestinal neuroendocrine tumor. The patient received treatment with first-generation somatostatin ligand receptor (SLR) every 4 weeks and had stable disease for several years before her pregnancy. First-generation SLR treatment was initially paused after detection of the pregnancy. During pregnancy, the patient experienced moderate gastro-intestinal discomfort and fatigue, which was considered predominantly pregnancy related. However, since symptoms could be linked to the patient’s cancer, treatment was resumed after the first trimester. Chromogranin-A measurements remained stable throughout pregnancy and was paralleled by the absence of diarrhea and only minor flushing. She gave birth by elective caesarean section in week 37 to a healthy baby. Subsequent follow up imaging immediately after and 10 months postpartum showed no disease progression. The safety profile of SLR treatment during pregnancy in the context of disseminated neuroendocrine tumors (NET) is discussed.

          Learning points
          • Neuroendocrine neoplasms (NEN) are rare cancers often occurring in the gastro-intestinal tract or lungs.

          • Many patients with NEN live for several years with disseminated disease.

          • SLR treatment has been given to pregnant patients before; often patients with acromegaly. Pregnancies are reported uneventful.

          • This patient completed an uneventful pregnancy while receiving SLR treatment for disseminated neuroendocrine disease and gave birth to a healthy baby.

          • More research regarding long term effects and safety signals of SLR treatment during pregnancy are much needed.

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          Most cited references10

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          Nothing But NET: A Review of Neuroendocrine Tumors and Carcinomas

          This review covers the diverse topic of neuroendocrine neoplasms (NENs), a relatively rare and heterogeneous tumor type, comprising ~2% of all malignancies, with a prevalence of <200,000 in the United States, which makes it an orphan disease (Basu et al., 2010). 1 For functional purposes, NENs are divided into two groups on the basis of clinical behavior, histology, and proliferation rate: well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinoma (Bosman et al., 2010) 2 ; this histological categorization/dichotomization is highly clinically relevant with respect to impact on treatment and prognosis even though it is not absolute since a subset of tumors with a low-grade appearance behaves similarly to high-grade lesions. Given the relative dearth of evidenced-based literature about this orphan disease as a whole (Modlin et al., 2008),3 since the focus of most articles is on particular anatomic subtypes of NENs (i.e., gastroenteropancreatic or pulmonary), the purpose of this review is to summarize the presentation, pathophysiology, staging, current standard of care treatments, and active areas of current research.
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            ENETS 2016 Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumors: An Update

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              Pregnancy in acromegaly: experience from two referral centers and systematic review of the literature.

              Acromegaly results from increased growth hormone and its target insulin-like growth factor-1, most commonly due to a pituitary tumour. As it is frequently accompanied by infertility, little is known about the course of this disease in pregnancy. We describe 13 new pregnancies in acromegalic women and compare their outcomes in a systematic review of the literature. We collected clinical, biochemical, imaging, and outcomes data during and following pregnancy and performed a systematic review for a total of 47 pregnancies. An extended analysis of 106 pregnancies was also performed. In 13 newly described cases, pregnancy was un-complicated without need for additional surgical intervention. In these pregnancies, adjunctive medical therapy was required in three patients. This was in the form of somatostatin analogs (SA) (3/13) as well as pegvisomant in 1/13 to control symptomatic and biochemical progression. One 37-year-old female succeeded in having two separate pregnancies 2 years apart both without need for any form of medical therapy. Review of an additional 34 published reports allowed for an analysis of outcomes in 47 pregnancies. Adjunctive medical therapy during pregnancy was required in 15 of these cases where 12 received SA and an additional three received dopamine agonists. None of these patients developed endocrine or neurologic complications during pregnancy. In an extended analysis of 106 pregnancies, treatment during pregnancy appears to be associated with good disease control but increased risk of microsomic or macrosomic newborns depending on the medical agent used. In 13 newly described pregnancies along with systematic review of an additional 34 cases indicate that pregnancy in treated acromegalic women can proceed without significant complications or teratogenicity. Medical treatment during pregnancy with DA or SA appears to be associated with altered neonatal weight. Nevertheless, gestation may have a beneficial impact on acromegaly control both during and following pregnancy. © 2011 Blackwell Publishing Ltd.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                15 September 2021
                2021
                : 2021
                : 21-0126
                Affiliations
                [1 ]Department of Hepatology and Gastroenterology , Aarhus University, Aarhus, Denmark
                [2 ]Department of Obstetrics and Gynecology , Aarhus University, Aarhus, Denmark
                [3 ]Department of Endocrinology and Internal Medicine , Aarhus University, Aarhus, Denmark
                [4 ]Department of Clinical Pharmacology , Aarhus University, Aarhus, Denmark
                [5 ]Department of Clinical Medicine , Aarhus University, Aarhus, Denmark
                [6 ]Department of Biomedicine , Aarhus University, Aarhus, Denmark
                Author notes
                Correspondence should be addressed to G E Villadsen; Email: Gerda.Elisabeth.Villadsen@ 123456aarhus.rm.dk
                Author information
                http://orcid.org/0000-0002-2757-929X
                Article
                EDM210126
                10.1530/EDM-21-0126
                8558896
                34612206
                19d90896-f817-44a2-9aa7-86812b2ce178
                © The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

                History
                : 14 September 2021
                : 15 September 2021
                Categories
                Pregnant Adult
                Female
                White
                Denmark
                Liver
                Endocrine-Related Cancer
                Gynaecology
                Unique/Unexpected Symptoms or Presentations of a Disease
                Unique/Unexpected Symptoms or Presentations of a Disease

                pregnant adult,female,white,denmark,liver,endocrine-related cancer,gynaecology,unique/unexpected symptoms or presentations of a disease,october,2021

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