Lung tumor - Pleuropulmonary blastoma

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Lung tumor

Other malignancies

Pleuropulmonary blastoma

Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 23 January 2013, last major update February 2010
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Definition
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● Pleuropulmonary blastoma (PPB) is a childhood tumor of the lung or pleura that usually occurs in children age 4 years or younger
● First described in 1988 (Cancer 1988;62:1516)

Epidemiology
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● Rare overall, but one of the most common primary lung malignancies in children (Arch Pathol Lab Med 2008;132:1079)

Clinical features
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● International registry is at http://www.ppbregistry.org
● Children usually present with difficulty breathing or other respiratory problems
● MRI or CT can help diagnose, but biopsy is suggested (Pediatr Radiol 2007;37:337)
● Gross total resection is necessary for cure, and selected patients do not require radiation therapy (J Pediatr Hematol Oncol 2012;34:e182)
● Classified into three types:
- Type I is multicystic, and may progress into types II or III
- Type II shows thickened areas within the cysts
- Type III shows solid masses

Case reports
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● 8 month old infant with shortness of breath (Case of the Week #166)

Treatment
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● Radical surgery to excise the cyst or mass, with extensive follow up to detect relapses
● Chemotherapy is usually provided for types II and III; 11% of type II and 54% of type III patients develop cerebral metastases (Pediatr Blood Cancer 2007;49:266)

Gross images
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Case of the Week #166

Micro description (Histopathology)
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● Mixed blastematous and sarcomatous appearance and primitive cells (Singapore Med J 2007;48:e190)
● Primitive, undifferentiated blastema cells and sarcomatous elements of different types of differentiation, including lipoblastic, chondroblastic, rhabdomyoblastic and giant cell undifferentiated sarcomas
● Cystic and slit-like spaces lined by the primitive or sarcomatous cells
● Multiple foci of necrosis
● Type I is multicystic, and may progress into types II or III
● Type II shows thickened areas within the cysts
● Type III shows solid masses

Micro images
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Case of the Week #166

Molecular / cytogenetics description
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● Frequently gains in chromosome 8q (Mod Pathol 2007;20:1191)

Differential diagnosis
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● Many type III tumors resemble embryonal rhabdomyosarcoma, but this tumor is rare in lungs/pleura, and PPB also has cystic architectural, blastema tissue and other mesenchymal components such as cartilage
● Other tumors to consider include monophasic synovial sarcoma, malignant peripheral nerve sheath tumor, mesothelioma, malignant teratoma and undifferentiated sarcoma
● Immunostains are typically not helpful in differentiating these entities

End of Lung tumor > Other malignancies > Pleuropulmonary blastoma

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