pleuropulmonary blastoma


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pleuropulmonary blastoma

A rare solid and biphasic primitive paediatric neoplasm that develops in the lung parenchyma or pleura.

Clinical findings
Cough, recurrent upper respiratory tract infections, dyspnea, chest pain.

Prognosis
Generally poor with CNS metastasis; some cases may respond to excision and chemotherapy.

Pleuropulmonary blastoma types
• Type I—Cystic
Affects infants (average, 10 months); multicystic; difficult to diagnose early. It responds better to treatment than other types, and may not progress to higher type.
• Type Ir—(Regressing)
Similar to Type I, but lacks malignant cells.
• Type II—Solid and cystic
Affects older children. 
• Type III—Solid
Affects older children; very poor prognosis; requires intensive chemotherapy.
References in periodicals archive ?
The differential diagnosis includes pleuropulmonary blastoma (PPB), pulmonary sequestration, congenital lobar emphysema, diaphragmatic hernia, bronchogenic cyst and cystic bronchiectasis.
Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation: a case report.
Computerized Topographic scan of the chest showed a mixed solid and cystic mass that was proved histopathologically as Pleuropulmonary blastoma (PPB) after excision by thoracotomy which is a rare primitive aggressive malignant neoplasm of the thorax occurring in childhood.
round cell tumor (desmin+, EMA+, NSE+), one germ cell tumor (anaplastic dysgerminoma; vimentin+, PLAP+, CD117+), one carcinosarcoma (CK+, vimentin+), one pleuropulmonary blastoma (vimentin+, S-100+, CD34-,CD99-, SMA-, desmin-), one Wilm's tumor (EMA+, NSE+, WT1-, desmin-), one pheochromocytoma (S-100+, CGA+, EMA-, calretinin-, CD56-, inhibin-, CK7-), one glioblastoma multiforme (GFAP+, EMA-) and one interdigitating dendritic cell tumor (CD3+, CD5+, CD23+, CD68+, Bcl2+, CD10-, CD79a-, CD20-, CD56-, CD21-, EMA-, HMB45- and CGA-).
Not only was Lauren Cherry diagnosed with a brain tumor on July 12, 2013, but she was also found to have an extremely rare pediatric lung tumor called Pleuropulmonary Blastoma in mid-August.
He has now been diagnosed with Pleuropulmonary Blastoma, a rare childhood cancer of the lungs, making him one of only four children in the world to suffer from the disease.
Two-year-old Lucas Longthorpe is only one of 24 children in the world battling pleuropulmonary blastoma, a rare childhood chest tumour.
But Lucas's condition began to deteriorate and tests revealed he had pleuropulmonary blastoma.
20) Pulmonary sarcomas can be generally grouped into 4 categories: (1) spindle cell sarcomas, including SS, malignant peripheral nerve sheath tumor, leiomyosarcoma, inflammatory myofibroblastic tumor, solitary fibrous tumor, and undifferentiated pleomorphic sarcoma; (2) vascular tumors, including angiosarcoma, Kaposi sarcoma, epithelioid hemangioendothelioma, and pulmonary artery intima sarcoma; (3) primitive/embryonal tumors, including primary pleuropulmonary blastoma, primary NET, and rhabdomyosarcoma; and (4) others, including primary pulmonary myxoid sarcoma, angiomatoid fibrous histiocytoma, and alveolar soft part sarcoma.
Pleuropulmonary blastoma as characteristic cause of pneumothorax.
Gastroblastoma has been compared to other blastomas, such as pleuropulmonary blastoma, hepatoblastoma, and nephroblastoma.
Other than pleuropulmonary blastoma family tumor and dysplasia syndrome, a rare syndrome related to germline mutation of DICER1 gene, most intraocular medulloepitheliomas occur sporadically and are not associated with congenital malformation or cytogenic abnormality.