Neha Venkatesh, Rebecca S Tidwell, Yao Yu, Ana Aparicio, Amado J Zurita, Sumit K Subudhi, Bilal A Siddiqui, Sagar S Mukhida, Justin R Gregg, Paul G Corn, Efstratios Koutroumpakis, Jennifer L McQuade, Daniel E Frigo, Patrick G Pilie, Chad Huff, Christopher J Logothetis, Andrew W Hahn
{"title":"Body composition in recurrent prostate cancer and the role of steroidogenic genotype.","authors":"Neha Venkatesh, Rebecca S Tidwell, Yao Yu, Ana Aparicio, Amado J Zurita, Sumit K Subudhi, Bilal A Siddiqui, Sagar S Mukhida, Justin R Gregg, Paul G Corn, Efstratios Koutroumpakis, Jennifer L McQuade, Daniel E Frigo, Patrick G Pilie, Chad Huff, Christopher J Logothetis, Andrew W Hahn","doi":"10.1530/ERC-24-0195","DOIUrl":"10.1530/ERC-24-0195","url":null,"abstract":"<p><p>Hormone therapy (HT) to treat prostate cancer is reported to cause adverse changes in body composition. Clinically, interpatient body composition changes are heterogeneous, but the biological and clinical determinants of body composition toxicity are unknown. Herein, we test the hypothesis that inherited polymorphisms in steroidogenic genes are associated with differential changes in body composition after HT. Men with biochemically recurrent prostate cancer (BCR) who received 8 months of LHRH analog (LHRHa) +/- abiraterone acetate (AAP) were eligible if they had: i) CT imaging of L3 prior to and after treatment; and ii) nucleated cells collected. Cardiometabolic co-morbidities were retrospectively extracted. Body composition was measured using an AI-based segmentation tool. Germline DNA whole exome or genome sequencing was performed. In 162 men treated with 8 months of HT, median skeletal muscle mass (SMMi) loss was 6.6% and subcutaneous adipose gain was 12.3%. Men with type 2 diabetes had higher losses of SMMi after treatment (-11.1% vs -6.3%, P = 0.003). For the 150 men with germline NGS, SRD5A2 rs523349 genotype was associated with differential loss in skeletal muscle density after HT, (-1.3% vs -7.1%, P = 0.04). In addition, the HSD3B1 rs104703 genotype was associated with decreased baseline visceral adipose tissue (63.0 cm2/m2 vs 77.9, P = 0.05). In men with BCR, HT induced notable loss of skeletal muscle and increased subcutaneous adipose tissue. An inherited polymorphism in SRD5A2 and T2DM was associated with differential skeletal muscle toxicity. These findings suggest that inherited polymorphisms may contribute to the body composition toxicity observed with HT.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12136150/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335080","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":"Nutrition, GH/IGF-1 signaling, and cancer.","authors":"Maura Fanti, Valter D Longo","doi":"10.1530/ERC-23-0048","DOIUrl":"10.1530/ERC-23-0048","url":null,"abstract":"<p><p>Cancer is the second leading cause of death in the United States and among the most prevalent diseases globally, with an incidence expected to grow because of smoking, pollution, poor dietary habits, obesity, and the rise in the older population. Given their ability to reduce risk factors, albeit with varying efficacy, nutrition and fasting could help prevent cancer and other age-related disorders. Calorie restriction (CR), various forms of intermittent fasting (IF) or periodic fasting (PF), and fasting-mimicking diets (FMDs) have been shown to improve health span, increase lifespan, and prevent or postpone cancer in rodents. The effects of specific diets and fasting regimens on aging and cancer appear to be mediated in part by the reduction in the activity of the growth hormone (GH)/insulin-like-growth-factor-I (IGF-1) axis. Nevertheless, recent data indicate that the alternation of low and normal levels of these hormones and factors may be ideal for optimizing longevity and function. Here, we review the role of nutrition, CR, and fasting/FMD on cancer, focusing on the hypothesis that the modulation of GH, IGF-1, and insulin signaling partly mediates the effect of these dietary interventions on cancer prevention.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11771996/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142019949","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}
A Mohamed, M Trybula, S L Asa, T R Halfdanarson, M B Sonbol
{"title":"Management of advanced high grade gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs): comprehensive review of the current literature.","authors":"A Mohamed, M Trybula, S L Asa, T R Halfdanarson, M B Sonbol","doi":"10.1530/ERC-24-0025","DOIUrl":"10.1530/ERC-24-0025","url":null,"abstract":"<p><p>The classification and management of neuroendocrine neoplasms (NENs) arising in the tubular gastrointestinal (GI) tract and pancreas have significantly evolved over the last decades. In the latest WHO classification published in 2022, NENs are separated regardless of their primary origin into two main groups: well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The substantial changes in the grading system changed the definition of grade 3 to include high-grade well-differentiated NETs (G3-NETs), and poorly differentiated NECs (-NECs). Although these two subgroups are considered high grades with Ki-67 >20%, they have different genomic profiles, prognosis, and clinical behavior, which critically influence their treatment strategies. The available clinical trial data to guide therapy of these high-grade subgroups are extremely limited, which impacts their management. In this review, we will summarize the current advances in the multidisciplinary approach for the management of high-grade gastroenteropancreatic NENs (GEP-NENs) including G3-NETs and NECs.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141918391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Adam I Kaplan, Trisha Dwight, Catherine Luxford, Diana E Benn, Roderick J Clifton-Bligh
{"title":"SDHA-related phaeochromocytoma and paraganglioma: review and clinical management.","authors":"Adam I Kaplan, Trisha Dwight, Catherine Luxford, Diana E Benn, Roderick J Clifton-Bligh","doi":"10.1530/ERC-24-0111","DOIUrl":"10.1530/ERC-24-0111","url":null,"abstract":"<p><p>Phaeochromocytomas and paragangliomas (collectively termed PPGL) are rare yet highly heritable neuroendocrine tumours, with over one-third of cases associated with germline pathogenic variants (PVs) in numerous genes. PVs in the succinate dehydrogenase subunit-A gene (SDHA) were initially implicated in hereditary PPGL in 2010, and SDHA has since become an important susceptibility gene accounting for up to 2.8% of cases. However, it remains poorly understood, particularly regarding the clinical nature of SDHA PPGL, rates of recurrence and metastasis, and the nature of metastatic disease. We present a narrative review of SDHA-related PPGL, covering pathophysiology, relevance to current clinical practice, and considerations for clinical genetics. We analyse a pool of 107 previously reported cases of SDHA-associated PPGL to highlight the spectrum of SDHA-related PPGL. Our analysis demonstrates that SDHA PPGL occurs across a wide age range (11-81 years) and affects men and women equally. SDHA PPGL typically presents as single tumours (91%), usually occurring in the head and neck (46%) or abdomen (43%, including 15% with phaeochromocytomas). Metastatic disease was reported in 25.5% of cases, with bone (82%) and lymph nodes (71%) being the most common sites of metastasis, often identified many years after the initial diagnosis. A family history of SDHA-related neoplasia was rare, reported in only 4% of cases. Understanding the clinical nature and risks associated with SDHA PVs is essential for facilitating the optimal management of patients and their families.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11466202/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141918392","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}
Marta Araujo-Castro, Betina Biagetti, Edelmiro Menéndez Torre, Iría Novoa-Testa, Fernando Cordido, Eider Pascual-Corrales, Víctor Rodríguez Berrocal, Fernando Guerrero-Pérez, Almudena Vicente, Juan Carlos Percovich Hualpa, Rogelio García-Centeno, Laura González-Fernández, María Dolores Ollero García, Ana Irigaray Echarri, María Dolores Moure Rodríguez, Cristina Novo-Rodríguez, María Calatayud, Rocío Villar-Taibo, Ignacio Bernabéu, Cristina Alvarez-Escola, Pamela Benítez Valderrama, Carmen Tenorio-Jiménez, Pablo Abellán Galiana, Eva Venegas, Inmaculada González-Molero, Pedro Iglesias, Concepción Blanco-Carrera, Fernando Vidal-Ostos De Lara, Paz de Miguel Novoa, Elena López Mezquita, Felicia Alexandra Hanzu, Iban Aldecoa, Silvia Aznar, Cristina Lamas, Anna Aulinas, Queralt Asla, Paola Gracia Gimeno, José María Recio-Córdova, María Dolores Avilés-Pérez, Diego Asensio-Wandosell, Miguel Sampedro-Núñez, Rosa Cámara, Miguel Paja Fano, Ignacio Ruz-Caracuel, Carmen Fajardo, Mónica Marazuela, Manel Puig-Domingo
{"title":"ERRATUM: Pegvisomant and pasireotide in PRL and GH co-secreting vs GH-secreting Pit-NETs.","authors":"Marta Araujo-Castro, Betina Biagetti, Edelmiro Menéndez Torre, Iría Novoa-Testa, Fernando Cordido, Eider Pascual-Corrales, Víctor Rodríguez Berrocal, Fernando Guerrero-Pérez, Almudena Vicente, Juan Carlos Percovich Hualpa, Rogelio García-Centeno, Laura González-Fernández, María Dolores Ollero García, Ana Irigaray Echarri, María Dolores Moure Rodríguez, Cristina Novo-Rodríguez, María Calatayud, Rocío Villar-Taibo, Ignacio Bernabéu, Cristina Alvarez-Escola, Pamela Benítez Valderrama, Carmen Tenorio-Jiménez, Pablo Abellán Galiana, Eva Venegas, Inmaculada González-Molero, Pedro Iglesias, Concepción Blanco-Carrera, Fernando Vidal-Ostos De Lara, Paz de Miguel Novoa, Elena López Mezquita, Felicia Alexandra Hanzu, Iban Aldecoa, Silvia Aznar, Cristina Lamas, Anna Aulinas, Queralt Asla, Paola Gracia Gimeno, José María Recio-Córdova, María Dolores Avilés-Pérez, Diego Asensio-Wandosell, Miguel Sampedro-Núñez, Rosa Cámara, Miguel Paja Fano, Ignacio Ruz-Caracuel, Carmen Fajardo, Mónica Marazuela, Manel Puig-Domingo","doi":"10.1530/ERC-24-0043e","DOIUrl":"10.1530/ERC-24-0043e","url":null,"abstract":"","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":"31 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141768316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E Baudin, J Capdevila, D Hörsch, S Singh, M E Caplin, E M Wolin, W Buikhuisen, M Raderer, E Dansin, C Grohe, D Ferone, A Houchard, X-M Truong-Thanh, D Reidy-Lagunes
{"title":"Treatment of advanced BP-NETS with lanreotide autogel/depot vs placebo: the phase III SPINET study.","authors":"E Baudin, J Capdevila, D Hörsch, S Singh, M E Caplin, E M Wolin, W Buikhuisen, M Raderer, E Dansin, C Grohe, D Ferone, A Houchard, X-M Truong-Thanh, D Reidy-Lagunes","doi":"10.1530/ERC-23-0337","DOIUrl":"10.1530/ERC-23-0337","url":null,"abstract":"<p><p>Prospective data are lacking on early somatostatin analog (SSA) therapy in bronchopulmonary neuroendocrine tumors (BP-NETs; typical carcinoids and atypical carcinoids (TCs and ACs)). SPINET (EudraCT: 2015-004992-62; NCT02683941) was a phase III, double-blind study of lanreotide autogel/depot (LAN; 120 mg every 28 days) plus best supportive care (BSC) vs placebo plus BSC, with an optional open-label treatment phase (LAN plus BSC). Patients had metastatic/unresectable, somatostatin receptor (SSTR)-positive TCs or ACs. Recruitment was stopped early owing to slow accrual; eligible patients from the double-blind phase transitioned to open-label LAN. The adapted primary endpoint was progression-free survival (PFS) during either phase for patients receiving LAN. Seventy-seven patients were randomized (LAN, n = 51 (TCs, n = 29; ACs, n = 22); placebo, n = 26 (TCs, n = 16; ACs, n = 10)). Median (95% CI) PFS during double-blind and open-label phases in patients receiving LAN was 16.6 (11.3; 21.9) months overall (primary endpoint), 21.9 (12.8, not calculable (NC)) months in TCs, and 13.8 (5.4; 16.6) months in ACs. During double-blind treatment, median (95% CI) PFS was 16.6 (11.3; 21.9) months for LAN vs 13.6 (8.3; NC) months for placebo (not significant); corresponding values were 21.9 (13.8; NC) and 13.9 (13.4; NC) months, respectively, in TCs and 13.8 (5.4; 16.6) and 11.0 (2.8; 16.9) months, respectively, in ACs. Patients' quality of life did not deteriorate and LAN was well tolerated. Although recruitment stopped early and the predefined sample size was not met, SPINET is the largest prospective study to date of SSA therapy in SSTR-positive TCs and ACs and suggests clinical benefit in TCs.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11301421/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141447868","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}
Marcia S Brose, Jaume Capdevila, Rossella Elisei, Lars Bastholt, Dagmar Führer-Sakel, Sophie Leboulleux, Iwao Sugitani, Matthew H Taylor, Zhuoying Wang, Lori J Wirth, Francis P Worden, John Bernard, Paolo Caferra, Raffaella M Colzani, Shiguang Liu, Martin Schlumberger
{"title":"Vandetanib in locally advanced or metastatic differentiated thyroid cancer refractory to radioiodine therapy.","authors":"Marcia S Brose, Jaume Capdevila, Rossella Elisei, Lars Bastholt, Dagmar Führer-Sakel, Sophie Leboulleux, Iwao Sugitani, Matthew H Taylor, Zhuoying Wang, Lori J Wirth, Francis P Worden, John Bernard, Paolo Caferra, Raffaella M Colzani, Shiguang Liu, Martin Schlumberger","doi":"10.1530/ERC-23-0354","DOIUrl":"10.1530/ERC-23-0354","url":null,"abstract":"<p><p>The VERIFY study aimed to determine the efficacy of vandetanib in patients with differentiated thyroid cancer (DTC) that is either locally advanced or metastatic and refractory to radioiodine (RAI) therapy. Specifically, VERIFY is a randomized, double-blind, multicenter phase III trial aimed to determine the efficacy and safety of vandetanib in tyrosine kinase inhibitor-naive patients with locally advanced or metastatic RAI-refractory DTC with documented progression (NCT01876784). Patients were randomized 1:1 to vandetanib or placebo. The primary endpoint was progression-free survival (PFS). Secondary endpoints included best objective response rate, overall survival (OS), safety, and tolerability. Patients continued to receive randomized treatment until disease progression or for as long as they were receiving clinical benefit unless criteria for treatment discontinuation were met. Following randomization, 117 patients received vandetanib, and 118 patients received a placebo. Median PFS was 10.0 months in the vandetanib group and 5.7 months in the placebo group (hazard ratio: 0.75; 95% CI: 0.55-1.03; P = 0.080). OS was not significantly different between treatment arms. Common Terminology Criteria for Adverse Events (CTCAE) of grade ≥3 were reported in 55.6% of patients in the vandetanib arm and 25.4% in the placebo arm. Thirty-three deaths (28.2%; one related to study treatment) occurred in the vandetanib arm compared with 16 deaths (13.6%; two related to treatment) in the placebo arm. No statistically significant improvement was observed in PFS in treatment versus placebo in patients with locally advanced or metastatic, RAI-refractory DTC. Moreover, active treatment was associated with more adverse events and more deaths than placebo, though the difference in OS was not statistically significant.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11301419/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201610","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}
Andreas Machens, Kerstin Lorenz, Frank Weber, Tim Brandenburg, Dagmar Führer-Sakel, Henning Dralle
{"title":"Genotype-specific development of MEN 2 constituent components in 683 RET carriers.","authors":"Andreas Machens, Kerstin Lorenz, Frank Weber, Tim Brandenburg, Dagmar Führer-Sakel, Henning Dralle","doi":"10.1530/ERC-24-0038","DOIUrl":"10.1530/ERC-24-0038","url":null,"abstract":"<p><p>The age-specific development of the three constituent components of multiple endocrine neoplasia type 2 (MEN 2) is incompletely characterized for many of the >30 causative rearranged during transfection (RET) mutations, which this genetic association study aimed to specify. Included in the study were 683 carriers of heterogeneous RET germline mutations: 53 carriers with 1 highest-risk mutation (codon 918); 240 carriers with 8 different high-risk mutations (codon 634); 176 carriers with 16 different intermediate-risk mutations (codon 609, 611, 618, 620, or 630); and 214 carriers with 6 different low-risk mutations (codon 768, 790, 804, or 891).There was a strong genotype-specific development of MEN 2 constituent components, with distinct age gradients from C cell disease to node negative medullary thyroid cancer (MTC), from node negative to node positive MTC, from node positive MTC to pheochromocytoma, and from pheochromocytoma to primary hyperparathyroidism. Primary hyperparathyroidism was not observed among the 53 MEN 2B patients who carried highest-risk mutations (age range: 0.5-50 years), of whom no more than 12 (23%) and 3 (6%) carriers were older than age 30 years and 35 years, respectively. The age-specific development of MTC differed significantly between the four RET risk categories, whereas the age-specific development of pheochromocytoma differed significantly only between the two strongest RET risk categories. No significant differences were noted in the development of primary hyperparathyroidism. These findings delineate age-specific disease manifestation corridors for the three constituent components of MEN 2 by RET genotype. These corridors are useful for initial risk assessment and organ-specific surveillance of newly identified RET carriers going forward.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140946665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marta Araujo-Castro, Betina Biagetti, Edelmiro Menéndez Torre, Iría Novoa-Testa, Fernando Cordido, Eider Pascual-Corrales, Víctor Rodríguez Berrocal, Fernando Guerrero-Pérez, Almudena Vicente, Juan Carlos Percovich Hualpa, Rogelio García-Centeno, Laura González-Fernández, María Dolores Ollero García, Ana Irigaray Echarri, María Dolores Moure Rodríguez, Cristina Novo-Rodríguez, María Calatayud, Rocío Villar-Taibo, Ignacio Bernabéu, Cristina Alvarez-Escola, Pamela Benítez Valderrama, Carmen Tenorio-Jiménez, Pablo Abellán Galiana, Eva Venegas, Inmaculada González-Molero, Pedro Iglesias, Concepción Blanco-Carrera, Fernando Vidal-Ostos De Lara, Paz de Miguel Novoa, Elena López Mezquita, Felicia Alexandra Hanzu, Iban Aldecoa, Silvia Aznar, Cristina Lamas, Anna Aulinas, Queralt Asla, Paola Gracia Gimeno, José María Recio-Córdova, María Dolores Avilés-Pérez, Diego Asensio-Wandosell, Miguel Sampedro-Núñez, Rosa Cámara, Miguel Paja Fano, Ignacio Ruz-Caracuel, Carmen Fajardo, Mónica Marazuela, Manel Puig-Domingo
{"title":"Pegvisomant and pasireotide in PRL and GH co-secreting vs GH-secreting Pit-NETs.","authors":"Marta Araujo-Castro, Betina Biagetti, Edelmiro Menéndez Torre, Iría Novoa-Testa, Fernando Cordido, Eider Pascual-Corrales, Víctor Rodríguez Berrocal, Fernando Guerrero-Pérez, Almudena Vicente, Juan Carlos Percovich Hualpa, Rogelio García-Centeno, Laura González-Fernández, María Dolores Ollero García, Ana Irigaray Echarri, María Dolores Moure Rodríguez, Cristina Novo-Rodríguez, María Calatayud, Rocío Villar-Taibo, Ignacio Bernabéu, Cristina Alvarez-Escola, Pamela Benítez Valderrama, Carmen Tenorio-Jiménez, Pablo Abellán Galiana, Eva Venegas, Inmaculada González-Molero, Pedro Iglesias, Concepción Blanco-Carrera, Fernando Vidal-Ostos De Lara, Paz de Miguel Novoa, Elena López Mezquita, Felicia Alexandra Hanzu, Iban Aldecoa, Silvia Aznar, Cristina Lamas, Anna Aulinas, Queralt Asla, Paola Gracia Gimeno, José María Recio-Córdova, María Dolores Avilés-Pérez, Diego Asensio-Wandosell, Miguel Sampedro-Núñez, Rosa Cámara, Miguel Paja Fano, Ignacio Ruz-Caracuel, Carmen Fajardo, Mónica Marazuela, Manel Puig-Domingo","doi":"10.1530/ERC-24-0043","DOIUrl":"10.1530/ERC-24-0043","url":null,"abstract":"<p><p>The objective of the study was to evaluate the efficacy of second-line therapies in patients with acromegaly caused by a growth hormone (GH) and prolactin (PRL) co-secreting pituitary neuroendocrine tumor (GH&PRL-Pit-NET) compared to their efficacy in patients with acromegaly caused by a GH-secreting pituitary neuroendocrine tumor (GH-Pit-NET). This is a multicenter retrospective study of patients with acromegaly on treatment with pasireotide and/or pegvisomant. Patients were classified in two groups: GH&PRL-Pit-NETs when evidence of hyperprolactinemia and immunohistochemistry (IHC) for GH and PRL was positive or if PRL were >200 ng/dL regardless of the PRL-IHC and GH-Pit-NETs when the previously mentioned criteria were not met. A total of 28 cases with GH&PRL-Pit-NETs and 122 with GH-Pit-NETs met the inclusion criteria. GH&PRL-Pit-NETs presented at a younger age, caused hypopituitarism, and were invasive more frequently than GH-Pit-NETs. There were 124 patients treated with pegvisomant and 49 with pasireotide at any time. The efficacy of pegvisomant for IGF-1 normalization was of 81.5% and of pasireotide of 71.4%. No differences in IGF-1 control with pasireotide and with pegvisomant were observed between GH&PRL-Pit-NETs and GH-Pit-NETs. All GH&PRL-Pit-NET cases treated with pasireotide (n = 6) and 82.6% (n = 19/23) of the cases treated with pegvisomant normalized PRL levels. No differences in the rate of IGF-1 control between pegvisomant and pasireotide were detected in patients with GH&PRL-Pit-NETs (84.9% vs 66.7%, P = 0.178). We conclude that despite the more aggressive behavior of GH&PRL-Pit-NETs than GH-Pit-NETs, no differences in the rate of IGF-1 control with pegvisomant and pasireotide were observed between both groups, and both drugs have shown to be effective treatments to control IGF-1 and PRL hypersecretion in these tumors.</p>","PeriodicalId":93989,"journal":{"name":"Endocrine-related cancer","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}