Clinical Outcomes and Complications of Pituitary Blastoma.

Autor:	Liu APY; Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA., Kelsey MM; Department of Pediatrics, Section of Pediatric Endocrinology, Children's Hospital Colorado and University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA., Sabbaghian N; Department of Medical Genetics, The Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montreal, Quebec, Canada., Park SH; Department of Pathology, Seoul National University, College of Medicine, Seoul, Republic of Korea., Deal CL; Endocrinology and Diabetes Service, CHU-Sainte Justine and Department of Pediatrics, University of Montreal, Montreal, Quebec, Canada., Esbenshade AJ; Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Nashville, Tennessee, USA., Ploner O; Diakonie-Klinikum, Stuttgart, Germany., Peet A; Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK.; Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK., Traunecker H; Noah's Ark Children's Hospital for Wales, Cardiff, UK., Ahmed YHE; Royal College of Surgeons in Ireland-Bahrain, Bahrain., Zacharin M; Department of Endocrinology and Diabetes, Royal Children's Hospital, Parkville, Melbourne, Victoria, Australia., Tiulpakov A; Department and Laboratory of Inherited Endocrine Disorders, Endocrinology Research Centre, Moscow, Russia., Lapshina AM; Department of Fundamental Pathomorphology, Endocrinology Research Centre, Moscow, Russia., Walter AW; duPont Hospital for Children, Wilmington, Delaware, USA., Dutta P; Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India., Rai A; Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India., Korbonits M; Department of Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, London, UK., de Kock L; Harry Perkins Institute of Medical Research, QEII Medical Centre and UWA Centre for Medical Research, the University of Western Australia, Perth, Australia., Nichols KE; Division of Cancer Predisposition, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA., Foulkes WD; Department of Medical Genetics, The Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montreal, Quebec, Canada.; Department of Medical Genetics and Cancer Research Program, Research Institute McGill University Health Centre, Montreal, Quebec, Canada., Priest JR; Minneapolis, Minnesota, USA.
Jazyk:	angličtina
Zdroj:	The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2021 Jan 23; Vol. 106 (2), pp. 351-363.
DOI:	10.1210/clinem/dgaa857
Abstrakt:	Context: Pituitary blastoma is a rare, dysontogenetic hypophyseal tumor of infancy first described in 2008, strongly suggestive of DICER1 syndrome. Objective: This work aims to describe genetic alterations, clinical courses, outcomes, and complications in all known pituitary blastoma cases. Design and Setting: A multi-institutional case series is presented from tertiary pediatric oncology centers. Patients: Patients included children with pituitary blastoma. Interventions: Genetic testing, surgery, oncologic therapy, endocrine support are reported. Outcome Measures: Outcome measures included survival, long-term morbidities, and germline and tumor DICER1 genotypes. Results: Seventeen pituitary blastoma cases were studied (10 girls and 7 boys); median age at diagnosis was 11 months (range, 2-24 months). Cushing syndrome was the most frequent presentation (n = 10). Cushingoid stigmata were absent in 7 children (2 with increased adrenocorticotropin [ACTH]; 5 with normal/unmeasured ACTH). Ophthalmoplegia and increased intracranial pressure were also observed. Surgical procedures included gross/near-total resection (n = 7), subtotal resection (n = 9), and biopsy (n = 1). Six children received adjuvant therapy. At a median follow-up of 6.7 years, 9 patients were alive; 8 patients died of the following causes: early medical/surgical complications (n = 3), sepsis (n = 1), catheter-related complication (n = 1), aneurysmal bleeding (n = 1), second brain tumor (n = 1), and progression (n = 1). Surgery was the only intervention for 5 of 9 survivors. Extent of resection, but neither Ki67 labeling index nor adjuvant therapy, was significantly associated with survival. Chronic complications included neuroendocrine (n = 8), visual (n = 4), and neurodevelopmental (n = 3) deficits. Sixteen pituitary blastomas were attributed to DICER1 abnormalities. Conclusions: Pituitary blastoma is a locally destructive tumor associated with high mortality. Surgical resection alone provides long-term disease control for some patients. Quality survival is possible with long-term neuroendocrine management. (© The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
Databáze:	MEDLINE
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