Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Primary Biphasic Hepatic Sarcoma in DICER1 Syndrome.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society - 1 Jan 2000

See Sharlene C, Wadhwani Nitin R, Yap Kai Lee, Arva Nicoleta C

Abstract excerpt

DICER1 tumor predisposition syndrome is a rare genetic disorder that predisposes individuals to multiple benign and malignant neoplasms. The phenotype is vast and includes pleuropulmonary blastoma (PPB), thyroid nodules, cystic nephroma, Wilms tumor, ovarian Sertoli-Leydig cell tumor, and medulloepithelioma, among others. Herein, we describe a patient with a DICER1 germline pathogenic variant presenting with two...

Topics

Join the communities discussing this publication.

Biomarkers, Tumor
Child
DEAD-box RNA Helicases
Liver Neoplasms
Neoplastic Syndromes, Hereditary

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Ribonuclease III

Source

Identifiers

PMID: 33872107
DOI: 10.1177/10935266211008443

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

Community interpretation

Discuss the evidence

Ask a question, add context or challenge an interpretation. Community activity is separate from scientific evidence strength.

0

discussions

0

replies

Citations encouraged

Claims remain attributable.

No discussion yet

Be the first to add a careful, cited interpretation.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Primary Biphasic Hepatic Sarcoma in DICER1 Syndrome.PMID 33872107Read ↗

The Histological Spectrum of DICER1-Associated Neoplasms.Semantic proximity Read ↗

Pleuropulmonary blastoma-like peritoneal sarcoma: a newly described malignancy associated with biallelic DICER1 pathogenic variation.Semantic proximity Read ↗

DICER1-Pleuropulmonary Blastoma Familial Tumor Predisposition SyndromeSemantic proximity Read ↗

DICER1 Syndrome and Cancer Predisposition: From a Rare Pediatric Tumor to Lifetime RiskSemantic proximity Read ↗

Pediatric imaging in DICER1 syndrome.Semantic proximity Read ↗

Expanding Our Knowledge of DICER1 Gene Alterations and Their Role in Thyroid DiseasesSemantic proximity Read ↗

DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome.Semantic proximity Read ↗

Pleuropulmonary Blastoma: Evolution of an Entity as an Entry into a Familial Tumor Predisposition Syndrome.Semantic proximity Read ↗

DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.Semantic proximity Read ↗

Identification of somatic and germ-line DICER1 mutations in pleuropulmonary blastoma, cystic nephroma and rhabdomyosarcoma tumors within a DICER1 syndrome pedigreeSemantic proximity Read ↗

Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations.Semantic proximity Read ↗

DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.