Lung tumor
Other malignancies
Pleuropulmonary blastoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 4 January 2017, last major update February 2010

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: pleuropulmonary blastoma
Cite this page: Pleuropulmonary blastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lungtumorPPB.html. Accessed January 22nd, 2017.
Definition / General
  • 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
Clinical Features
  • International registry is at Pleuropulmonary Blastoma Registry
  • 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
Treatment
  • 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

Images hosted on PathOut server:

Case of the Week #166

Micro Description
  • 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

Images hosted on PathOut server:

Case of the Week #166



Images hosted on other servers:

Contributed by Dr. Roopa Arora:

Case Report

SMA

Molecular / Cytogenetics Description
Differential Diagnosis