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Clinical and molecular features of pleuropulmonary blastoma in children in Hong Kong: case reports
Hong Kong Med J. 2022 Aug;28(4):328-330. doi: 10.12809/hkmj219503.NO ABSTRACTPMID:35989435 | DOI:10.12809/hkmj219503 (Source: Hong Kong Med J)
Source: Hong Kong Med J - August 22, 2022 Category: General Medicine Authors: A P Y Liu M K L Fung M Lee J L F Fung M H Y Tsang C W Luk B H Y Chung G C F Chan Source Type: research

Clinical and molecular features of pleuropulmonary blastoma in children in Hong Kong: case reports
Hong Kong Med J. 2022 Aug;28(4):328-330. doi: 10.12809/hkmj219503.NO ABSTRACTPMID:35989435 | DOI:10.12809/hkmj219503 (Source: Hong Kong Medical Journal)
Source: Hong Kong Medical Journal - August 22, 2022 Category: General Medicine Authors: A P Y Liu M K L Fung M Lee J L F Fung M H Y Tsang C W Luk B H Y Chung G C F Chan Source Type: research

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis
DICER1 syndrome is a tumor predisposition syndrome in which patients are at an increased risk of developing a wide variety of benign and malignant neoplasms with a hallmark constellation of pediatric pleuropulmonary blastoma, cystic nephroma, and thyroid lesions. DICER1 encodes an RNA endoribonuclease that is crucial to the processing of microRNA and may play a role in the maturation of Müllerian tissue. Within the gynecologic tract, germline mutations in DICER1 are associated with an array of rare tumors, including Sertoli-Leydig cell tumor, embryonal rhabdomyosarcoma of the cervix, gynandroblastoma, and juvenile granulo...
Source: Advances in Anatomic Pathology - August 21, 2022 Category: Pathology Tags: Review Articles Source Type: research

Likely foregut endoderm origin for a postzygotic mutation affecting the RNase IIIb domain of DICER1
In 2016, we reported a child with bilateral lung cysts and left lung type II pleuropulmonary blastoma (PPB), classic phenotypes of DICER1 syndrome; we identified a DICER1 hotspot mutation c.5425G>A, p.Gly1809Arg in both a lung cyst and PPB, but the variant was absent in other tissues studied. A somatic mutation (c.1966C>T, p.Arg656Ter) was identified in the PPB, and mosaicism was suspected.1 The child has since developed multinodular goitre (MNG) with cystic thyroid nodules. We have now detected p.Gly1809Arg in the thyroid lesions, while various other tissues were negative. The exquisite localisation of the variant c...
Source: Journal of Medical Genetics - June 27, 2022 Category: Genetics & Stem Cells Authors: Chong, A.-S., Apellaniz-Ruiz, M., de Kock, L., Bouron-Dal Soglio, D., Doyle, W. R., Priest, J. R., Rivera, B., Foulkes, W. D. Tags: Somatic mosaicism Source Type: research

Metastatic Sarcomatous Tumor to Mandible Identifies Pleuropulmonary Blastoma Tumor Predisposition Syndrome
Pleuropulmonary blastoma (PPB) affects infants and young children and may occur as a cystic (Type I), partially cystic-partially solid (Type 2), or solid (Type 3) tumor. PPB is an embryonal tumor derived from lung and pleural mesenchyme. This malignant tumor is composed of sarcomatous elements, most commonly a mixture of spindle cell sarcoma with focal rhabdomyosarcomatous elements. However, it may have a wide variety of sarcomatous components. The tumor is characterized by DICER1 mutation, which may be somatic (tumoral) or constitutional. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics)
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - April 15, 2022 Category: ENT & OMF Authors: Dr. John Hicks, Dr. Catherine Flaitz Source Type: research

Surgical resection for congenital lung malformation: Lessons learned from thoracotomy to biportal thoracoscopy under one-lung ventilation
CONCLUSIONS: VATS for CLMs is associated with better postoperative recovery and fewer complications. Biportal VATS is also a safe and feasible approach.PMID:35331621 | DOI:10.1016/j.jfma.2022.03.003 (Source: J Formos Med Assoc)
Source: J Formos Med Assoc - March 25, 2022 Category: General Medicine Authors: Wan-Ting Hung Yi-Chia Wang Hsing-Hao Huang Jui-Hung Tai En-Ting Wu Jin-Chung Shih Wen-Ming Hsu Source Type: research