Endocrine oncology (Bristol, England)最新文献

{"title":"Hyponatraemia and the syndrome of inappropriate antidiuresis (SIAD) in cancer.","authors":"D Mc Donald,&nbsp;M Sherlock,&nbsp;C J Thompson","doi":"10.1530/EO-22-0056","DOIUrl":"https://doi.org/10.1530/EO-22-0056","url":null,"abstract":"<p><p>Hyponatraemia is a common electrolyte abnormality seen in a wide range of oncological and haematological malignancies and confers poor performance status, prolonged hospital admission and reduced overall survival, in patients with cancer. Syndrome of inappropriate antidiuresis (SIAD) is the commonest cause of hyponatraemia in malignancy and is characterised by clinical euvolaemia, low plasma osmolality and concentrated urine, with normal renal, adrenal and thyroid function. Causes of SIAD include ectopic production of vasopressin (AVP) from an underlying tumour, cancer treatments, nausea and pain. Cortisol deficiency is an important differential in the assessment of hyponatraemia, as it has an identical biochemical pattern to SIAD and is easily treatable. This is particularly relevant with the increasing use of immune checkpoint inhibitors, which can cause hypophysitis and adrenalitis, leading to cortisol deficiency. Guidelines on the management of acute, symptomatic hyponatraemia recommend 100 mL bolus of 3% saline with careful monitoring of the serum sodium to prevent overcorrection. In cases of chronic hyponatraemia, fluid restriction is recommended as first-line treatment; however, this is frequently not feasible in patients with cancer and has been shown to have limited efficacy. Vasopressin-2 receptor antagonists (vaptans) may be preferable, as they effectively increase sodium levels in SIAD and do not require fluid restriction. Active management of hyponatraemia is increasingly recognised as an important component of oncological management; correction of hyponatraemia is associated with shorter hospital stay and prolonged survival. The awareness of the impact of hyponatraemia and the positive benefits of active restoration of normonatraemia remain challenging in oncology.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"R78-R89"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6a/98/EO-22-0056.PMC10259335.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10173150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypoparathyroidism: an uncommon adverse effect of treatment with durvalumab.","authors":"Alexander Kreze,&nbsp;Matěj Homer,&nbsp;Tereza Barešová,&nbsp;Kristina Klemperová","doi":"10.1530/EO-22-0047","DOIUrl":"https://doi.org/10.1530/EO-22-0047","url":null,"abstract":"<p><strong>Summary: </strong>Immune checkpoint inhibitors (ICIs) are monoclonal antibodies approved for the treatment of numerous cancer types. Toxicities induced by ICIs may affect any organ system and manifest as endocrinopathy. The main side effects related to treatment are immune-related adverse events (irAEs), especially thyroid dysfunction and hypophysitis. Rare endocrine irAEs are diabetes insipidus, hypoparathyroidism, thyrotoxic crisis and hypogonadism. We report a case of hypoparathyroidism induced by ICI treatment with durvalumab, which has not previously been described.</p><p><strong>Learning points: </strong>Treatment with immune checkpoint inhibitors (ICIs) is associated with many endocrine side effects.It is recommended that patients treated with ICIs are observed by an endocrinologist.If side effects are treated accordingly, ICI therapy can continue.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"K21-K24"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259288/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10173151","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pancreatic neuroendocrine neoplasms: survival trend analysis of a comprehensive center.","authors":"Sara Coelho,&nbsp;Cláudia Costa,&nbsp;Ana Paula Santos,&nbsp;Pedro Souteiro,&nbsp;Joana Oliveira,&nbsp;Júlio Oliveira,&nbsp;Isabel Azevedo,&nbsp;Isabel Torres,&nbsp;Maria José Bento","doi":"10.1530/EO-22-0043","DOIUrl":"https://doi.org/10.1530/EO-22-0043","url":null,"abstract":"<p><strong>Objectives: </strong>Therapeutic options for pancreatic neuroendocrine neoplasia (Pan-NEN) have increased over the last decade. We aim to understand the evolution of the prognosis of patients with diagnosis of Pan-NEN within a 12-year period, considering the implementation of new treatments.</p><p><strong>Methods: </strong>This study is a retrospective cohort study of patients diagnosed with Pan-NENs between 2006 and 2017. Survival outcome estimates were calculated by Kaplan-Meier method. The impact of baseline clinicopathological characteristics on survival was explored with the use of Cox proportional hazard model.</p><p><strong>Results: </strong>Of the 97 patients, 77 (79.9%) had well-differentiated neuroendocrine tumor (NET) according to WHO 2010 classification, and 52 (53.6%) had localized or locoregional disease. There were no differences between clinicopathological characteristics and survival outcomes when comparing patients diagnosed between 2006-2011 and 2012-2017. Neuroendocrine carcinoma - HR 2.76, 95% CI 1.17-6.55 - and stages III and IV at diagnosis were independent poor prognostic factors - HR 6.02, 95% CI 2.22-16.33 and HR 6.93, 95% CI 2.94-16.32, respectively.</p><p><strong>Conclusions: </strong>The new therapeutic approaches did not induce better survival outcomes on Pan-NEN in recent years. This is possibly due to the indolent nature of NET grades 1 and 2, even metastatic, allowing patients to be submitted to new target therapies along their disease course.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"32-41"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/90/e8/EO-22-0043.PMC10259287.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10191703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of filamin A in the pathogenesis of neuroendocrine tumors and adrenal cancer.","authors":"Donatella Treppiedi,&nbsp;Rosa Catalano,&nbsp;Federica Mangili,&nbsp;Giovanna Mantovani,&nbsp;Erika Peverelli","doi":"10.1530/EO-22-0055","DOIUrl":"https://doi.org/10.1530/EO-22-0055","url":null,"abstract":"<p><p>Cell cytoskeleton proteins are involved in tumor pathogenesis, progression and pharmacological resistance. Filamin A (FLNA) is a large actin-binding protein with both structural and scaffold functions implicated in a variety of cellular processes, including migration, cell adhesion, differentiation, proliferation and transcription. The role of FLNA in cancers has been studied in multiple types of tumors. FLNA plays a dual role in tumors, depending on its subcellular localization, post-translational modification (as phosphorylation at Ser2125) and interaction with binding partners. This review summarizes the experimental evidence showing the critical involvement of FLNA in the complex biology of endocrine tumors. Particularly, the role of FLNA in regulating expression and signaling of the main pharmacological targets in pituitary neuroendocrine tumors, pancreatic neuroendocrine tumors, pulmonary neuroendocrine tumors and adrenocortical carcinomas, with implications on responsiveness to currently used drugs in the treatment of these tumors, will be discussed.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"R143-R152"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259351/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9815505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paragangliomas of the head and neck: a contemporary review.","authors":"Nathan J Graham,&nbsp;Joshua D Smith,&nbsp;Tobias Else,&nbsp;Gregory J Basura","doi":"10.1530/EO-22-0080","DOIUrl":"https://doi.org/10.1530/EO-22-0080","url":null,"abstract":"<p><p>Head and neck paragangliomas (HNPGLs) are slow-growing, vascular, typically benign tumors whose growth may induce significant lower cranial nerve deficits. While most tumors arise sporadically, a significant portion is associated with defined genetic syndromes. While surgical resection has historically been the gold standard, management strategies have evolved with acknowledgement of high surgical morbidity, slow tumor growth rates, and technological advances. Conservative management approaches via observation and newer radiation therapy techniques have become more common. This review seeks to provide an update on contemporary management strategies for HNPGLs and future directions.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"R153-R162"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259325/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10173152","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Low but not undetectable early postoperative nadir serum cortisol predicts sustained remission in Cushing's disease.","authors":"Anna Stroud,&nbsp;Pearl Dhaliwal,&nbsp;Richard J Harvey,&nbsp;Raquel Alvarado,&nbsp;Benjamin P Jonker,&nbsp;Mark J Winder,&nbsp;Jessica W Grayson,&nbsp;Ann McCormack","doi":"10.1530/EO-21-0026","DOIUrl":"https://doi.org/10.1530/EO-21-0026","url":null,"abstract":"<p><strong>Objective: </strong>Transsphenoidal surgery (TSS) is the first-line treatment for Cushing's disease. The objectives of the study were to determine remission and recurrence rates after TSS for Cushing's disease, identify factors that predict these outcomes, and define the threshold for postoperative morning serum cortisol (MSeC) that most accurately predicts sustained remission.</p><p><strong>Methods: </strong>Records were retrospectively reviewed for consecutive adults undergoing TSS for Cushing's disease at a tertiary centre (1990-2019). Remission was defined as MSeC <138 nmol/L by 6 weeks postoperatively. Recurrence was defined as elevated 24-h urine free cortisol, lack of suppression after dexamethasone or elevated midnight salivary cortisol.</p><p><strong>Results: </strong>In this study, 42 patients (age 47 ± 13 years, 83% female) were assessed with 55 ± 56 months of follow-up. Remission occurred after 77% of primary (<i>n</i> = 30) and 42% of revision operations (<i>n</i> = 12). After primary surgery, remission was associated with lower MSeC nadir (26 ± 36 nmol/L vs 347 ± 220 nmol/L, <i>P</i>  < 0.01) and lower adrenocorticotropin nadir (2 ± 3 pmol/L vs 6 ± 3 pmol/L, <i>P</i> = 0.01). Sustained remission 5 years after surgery was predicted by MSeC <92 nmol/L within 2 weeks postoperatively (sensitivity 100% and specificity 100%). After revision surgery, remission was predicted by lower MSeC nadir (70 ± 45 nmol/L vs 408 ± 305 nmol/L, <i>P</i> = 0.03), smaller tumour diameter (3 ± 2 mm vs 15 ± 13 mm, <i>P</i> = 0.05) and absence of cavernous sinus invasion (0% vs 71%, <i>P</i> = 0.03). Recurrence after primary and revision surgery occurred in 17% and 20% of patients respectively.</p><p><strong>Conclusions: </strong>Lower postoperative MSeC nadir strongly predicted remission after both primary and revision surgery. Following primary surgery, an MSeC <92 nmol/L within 2 weeks predicted sustained remission at 5 years. MSeC nadir was the most important prognostic marker following TSS for Cushing's disease.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"19-31"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9805302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diverse role of androgen action in human breast cancer.","authors":"Kiyoshi Takagi,&nbsp;Mio Yamaguchi,&nbsp;Minoru Miyashita,&nbsp;Hironobu Sasano,&nbsp;Takashi Suzuki","doi":"10.1530/EO-22-0048","DOIUrl":"https://doi.org/10.1530/EO-22-0048","url":null,"abstract":"<p><p>Breast cancer is a hormone-dependent cancer, and sex steroids play a pivotal role in breast cancer progression. Estrogens are strongly associated with breast cancers, and the estrogen receptor (estrogen receptor α; ERα) is expressed in 70-80% of human breast carcinoma tissues. Although antiestrogen therapies (endocrine therapies) have significantly improved clinical outcomes in ERα-positive breast cancer patients, some patients experience recurrence after treatment. In addition, patients with breast carcinoma lacking ERα expression do not benefit from endocrine therapy. The androgen receptor (AR) is also expressed in >70% of breast carcinoma tissues. Growing evidence supports this novel therapeutic target for the treatment of triple-negative breast cancers that lack ERα, progesterone receptor, and human EGF receptor 2, and ERα-positive breast cancers, which are resistant to conventional endocrine therapy. However, the clinical significance of AR expression is still controversial and the biological function of androgens in breast cancers is unclear. In this review, we focus on the recent findings concerning androgen action in breast cancers and the contributions of androgens to improved breast cancer therapy.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"R102-R111"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9812413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unusual presentation of lung carcinoma with pituitary metastasis: a challenging diagnosis and sodium management dilemmas.","authors":"Poh Shean Wong,&nbsp;Subashini Rajoo,&nbsp;Hairuddin Achmad Sankala,&nbsp;Mohamed Badrulnizam Long Bidin","doi":"10.1530/EO-22-0064","DOIUrl":"https://doi.org/10.1530/EO-22-0064","url":null,"abstract":"<p><strong>Summary: </strong>Pituitary metastasis (PM) is a rare complication of an advanced malignancy. Albeit rare, PM can be more detected and achieve a longer survival rate through frequent neuroimaging and newer oncology therapies. Lung cancer is the most frequent primary site, followed by breast and kidney cancers. Patients with lung cancer generally present with respiratory symptoms and are commonly diagnosed at an advanced stage already. Nevertheless, physicians should be mindful of other systemic manifestations as well as signs and symptoms related to metastatic spread and paraneoplastic syndromes. Herein, we report the case of a 53-year-old woman who presented with PM as the first sign of an undiagnosed lung cancer. Initially, her condition was a challenging diagnosis and was even complicated with diabetes insipidus (DI), which can present as severe hyponatremia when coexisting with adrenal insufficiency. This case also highlights that treating DI with antidiuretic hormone (ADH) replacement was complicated by extreme difficulties in attaining satisfactory sodium and water balance during the clinical course, with the possibility of coexistent DI and syndrome of inappropriate ADH secretion because of the underlying lung cancer.</p><p><strong>Learning points: </strong>When patients present with pituitary mass and diabetes insipidus (DI), pituitary metastasis should be considered as an initial differential diagnosis. DI caused by pituitary adenoma is rare and is typically a late finding.DI can present as severe hyponatremia when coexisting with adrenal insufficiency.Cortisol can directly inhibit endogenous antidiuretic hormone (ADH) secretion. Patients with adrenocorticotropic hormone deficiency will have increased tonic ADH activity and subsequently reduced capacity for free-water excretion. However, when on steroid therapy, patients should be monitored for possible DI because steroids can restore free-water excretion.A substantial change in serum sodium after desmopressin treatment should eliminate the possibility of desmopressin overdose or coexistence of DI and syndrome of inappropriate ADH secretion in patients with lung cancer. Therefore, frequent monitoring of serum sodium concentrations is crucial.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"K15-K20"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259289/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9815506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"SAKK 21/12: a phase II trial of transdermal CR1447 in breast cancer patients.","authors":"Marcus Vetter,&nbsp;Karin M Rothgiesser,&nbsp;Qiyu Li,&nbsp;Hanne Hawle,&nbsp;Wolfgang Schönfeld,&nbsp;Karin Ribi,&nbsp;Salome Riniker,&nbsp;Roger von Moos,&nbsp;Andreas Trojan,&nbsp;Elena Kralidis,&nbsp;Mathias Fehr,&nbsp;Andreas Müller,&nbsp;Beat Thürlimann","doi":"10.1530/EO-21-0009","DOIUrl":"https://doi.org/10.1530/EO-21-0009","url":null,"abstract":"<p><strong>Objective: </strong>CR1447, a novel transdermal formulation of 4-hydroxytestosterone, has aromatase-inhibiting and androgen receptor (AR)-modulating properties (IC₅₀4.4 nM) with antitumor effects against AR-positive tumor cells <i>in vitro.</i> This trial investigated the efficacy and safety of CR1447 for patients with metastatic estrogen receptor-positive (A) and AR-positive triple-negative breast cancers (B).</p><p><strong>Design and methods: </strong>(A) included patients with at most one prior endocrine therapy line without progression ≥6 months, whereas (B) included patients with ≤2 prior chemotherapy lines, all displaying advanced signs of disease. The primary endpoint was disease control at week 24 (DC24). The null hypothesis was DC24 ≤30% (A) and ≤15% (B). Thirty-seven patients were recruited (29 in (A) and 8 in (B)); accrual was stopped following an interim analysis demonstrating futility in (A) and slow accrual in (B).</p><p><strong>Results: </strong>DC24 was attained in 5/21 (95% CI: 8.2-47.2) patients in (A) and none in (B). The median progression-free survival was 5.1 months (95% CI: 2.5-5.6) in (A) and 2.5 months (95% CI: 0.7-2.6) in (B). The median overall survival was 24.6 months (95% CI: 22.9-not applicable) in (A) and 10.8 months (95% CI: 3.3-10.9) in (B). CR1447 had a favorable safety profile without treatment-related grade 3-5 toxicities in (A). Especially no side effects linked to androgenic effects were observed.</p><p><strong>Conclusions: </strong>Despite this trial being negative, the 24% DC24 rate in a second-line setting, and the prolonged partial response experienced by a patient, indicate activity. Further evaluation of CR1447 in endocrine-sensitive patients or combination trials appears warranted.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"9-18"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259314/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9870423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Synchronous AML and pancreatic neuroendocrine neoplasm, both successfully treated with somatostatin analogs and decitabine.","authors":"Syed Ehsanullah,&nbsp;Nikolaos A Trikalinos","doi":"10.1530/EO-22-0052","DOIUrl":"https://doi.org/10.1530/EO-22-0052","url":null,"abstract":"<p><strong>Summary: </strong>Downregulation of tumor suppression genes by DNA hypermethylation has been proposed as a potential cause of neuroendocrine neoplasm (NEN) formation. In this report, we present a patient simultaneously diagnosed with acute myeloid leukemia (AML) and a metastatic nonfunctioning pancreatic NEN. Because of the two competing diagnoses, he was treated with lanreotide, venetoclax and a long course of the hypomethylating agent decitabine. The AML responded to venetoclax and decitabine treatment while the PanNEN stabilized on lanreotide. Over multiple months of treatment, the PanNEN showed gradual tumor response, consistent with decitabine treatment effect, and the patient remained without disease progression for both malignancies. We believe that some PanNENs can benefit from treatment with hypomethylating agents such as decitabine. To support this, we review the relevant literature and suggest a mechanism for the efficacy of decitabine in our case.</p><p><strong>Learning points: </strong>Neuroendocrine neoplasms are associated with an increased risk of second primary cancers.Epigenetic changes such as hypermethylation and inhibition of tumor suppressor genes might explain the development and behavior of certain NENs.The use of hypomethylating agents such as decitabine might have a role in the treatment of PanNENs. Future studies are needed to confirm that.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"2 1","pages":"K1-K4"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0e/ee/EO-22-0052.PMC10259290.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10191704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}