Observational studyThe Journal of clinical endocrinology and metabolism2021
Clinical Outcomes and Complications of Pituitary Blastoma.
Observational study in The Journal of clinical endocrinology and metabolism, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
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Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it, 54 citations in OpenAlex.
- Consensus guideline for the diagnosis and management of pituitary adenomas in childhood and adolescence: Part 1, general recommendations.Nature reviews. Endocrinology · 2024Guideline
- Rare solid tumours as indicators of hereditary cancer syndromes.European journal of human genetics : EJHG · 2026Review
- Pituitary blastoma in a dog: comparative clinical, imaging, and pathologic features of a newly recognized and rare human entity.Acta neuropathologica communications · 2026Article
- A contemporary overview of multiple endocrine neoplasia syndromes: MEN syndromes 1-5 and beyond.Endocrine oncology (Bristol, England) · 2026Review
- Clinical integration and application of the 2022 WHO pituitary tumor classification.Neuro-oncology advances · 2025Article
- Article
- Review
- [DICER1 syndrome: clinical variety endocrine manifestations and features of diagnostics].Problemy endokrinologii · 2023Review
- Newly Recognized CNS Tumors in the 2021 World Health Organization Classification: Imaging Overview with Histopathologic and Genetic Correlation.AJNR. American journal of neuroradiology · 2023Review
- Pediatric diencephalic tumors: a constellation of entities and management modalities.Frontiers in oncology · 2023Review
- Case report: Identification of potential prognosis-related LAG3 overexpression and DICER1 mutation in pituitary carcinoma: two cases.Frontiers in neuroscience · 2023Article
- Endoscopic Endonasal Surgery for Uncommon Pathologies of the Sellar and Parasellar Regions.Advances and technical standards in neurosurgery · 2023Review
- Neuroimaging of pediatric tumors of the sellar region-A review in light of the 2021 WHO classification of tumors of the central nervous system.Frontiers in pediatrics · 2023Review
- Review
- Overview of the 2022 WHO Classification of Pituitary Tumors.Endocrine pathology · 2022Review
- DICER1 syndrome in a young adult with pituitary blastoma.Acta neuropathologica · 2021Article
- Genetics of Acromegaly and Gigantism.Journal of clinical medicine · 2021Review
- The clinical aspects of pituitary tumour genetics.Endocrine · 2021Review
Corrections and comments
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Authors and funding
21 authors at 18 institutions in 9 countries.
Funding
Abstract
contextPituitary blastoma is a rare, dysontogenetic hypophyseal tumor of infancy first described in 2008, strongly suggestive of DICER1 syndrome.
objectiveThis work aims to describe genetic alterations, clinical courses, outcomes, and complications in all known pituitary blastoma cases. DESIGN AND
settingA multi-institutional case series is presented from tertiary pediatric oncology centers. PATIENTS: Patients included children with pituitary blastoma.
interventionsGenetic testing, surgery, oncologic therapy, endocrine support are reported. OUTCOME MEASURES: Outcome measures included survival, long-term morbidities, and germline and tumor DICER1 genotypes.
resultsSeventeen 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.
conclusionsPituitary 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.
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