pleuropulmonary blastoma
Pronunciation: ploo-roh-PUL-moh-nair-ee BLAS-toh-mah
A rare, aggressive intrathoracic malignancy of early childhood arising from pulmonary or pleural mesenchyme, classified into three histological types (I, II, and III).
Full Definition
Pleuropulmonary blastoma (PPB) is the most common primary malignant lung tumour in children under six years of age and is distinct from the adult pulmonary blastoma. It is categorised as Type I (purely cystic), Type Ir (regressed cystic), Type II (mixed cystic and solid), and Type III (purely solid), with prognosis worsening from Type I to Type III. PPB is strongly associated with germline DICER1 mutations, placing it within the DICER1 tumour predisposition syndrome. Editors must render the Roman numeral type correctly (I, II, III) and avoid confusion with other paediatric lung lesions. The abbreviation PPB is standard in oncology and pathology literature.
Usage
Usage note: Always spell out 'pleuropulmonary blastoma' on first mention before introducing the abbreviation PPB.
In Context
- "Genetic counselling was recommended after the child's Type II pleuropulmonary blastoma tested positive for a DICER1 pathogenic variant." — Oncology case summary
- "The manuscript incorrectly used Arabic numerals; PPB types should be rendered as Roman numerals (I, II, III) per convention." — Manuscript peer-review note