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pleuropulmonary blastoma


Thursday 13 November 2003

pediatric pneumoblastoma, pulmonary blastoma of childhhod, PPB

Definition: Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal tumor of lung parenchyma and/or pleura in young children.

Pleuropulmonary blastoma is an aggressive tumor characterized by mesenchymal elements (including undifferentiated blastema and often cartilaginous, rhabdomyoblastic, or fibroblastic differentiation) and epithelium-lined spaces.


- Pleuropulmonary blastoma (PPB)

Digital cases

- HPC:47 : PPB type 1 and 2.
- HPC:66 : PPB NOS.
- HPC:11 : PPB type 1.
- HPC:163 : PPB type 2.
- HPC:164 : PPB type 2.
- HPC:195 : Fetal PPB type 1.
- JRC:6115 : Pleuropulmonary blastoma (PPB).


- type 1 pleuropulmonary blastoma (purely cystic PPB)
- type 2 Pleuropulmonary blastoma (solid and cystic PPB)


- sporadic pleuropulmonary blastoma
- familial pleuropulmonary blastoma (8636815)


- cystic or solid (10870072)
- unilateral or bilateral (10839886)


- epithelial pulmonary blastoma: low-grade adenocarcinoma of the fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA)


Approximately 25% of children with PPB have other neoplasms or dysplasias in themselves or their close family members.

- congenital pulmonary cystic lesion

- renal cystic lesions

- tumors

- other conditions

Familial associations (8636815)

- pulmonary cysts
- cystic nephroma in CNPPB syndrome (10881016, 8382107)
- intestinal hamartomatous polyp in CNPPB syndrome (10442587)
- sarcomas
- medulloblastomas
- thyroid dysplasias and neoplasias
- malignant germ cell tumors
- Hodgkin disease
- leukemia
- Langerhans cell histiocytosis


- trisomy 2 (12660036, 9236843), 2q anomalies (8063284)
- trisomy 8 (12660036, 11910518, 11779045, 9050063, 10890933, 17163790), confined to mesenchymal elements (11779045)

- dup(7), der(10) (12660036)
- t(8; 10)(q13; q22) (12660036)
- add(17) (12660036)
- double minutes (dmin) (12660036)
- loss of 17p

CGH (15104284)

Gains 1q12-13 2q 3q23-qter 7 8pter-q24.1 9p13-q21 19q11-q13.3 17p12-p11 17q11-q22 17q23-q25 19pter-p11
Losses 5 6q13-qter 10pter-p13 10q22-qter 20p13

Molecular biology

- p53 mutations (2/3) (11881786, 9236836, 9224760, 8912818)
- beta-catenin gene (CTNNB1) missense mutations in epithelial pulmonary blastoma (15223963, 12754743)
- DICER1 mutations in familial forms

Target regions


Differential diagnosis

- congenital pumonary airways malformations (pulmonary cystic adenomatoid malformations - PCAMs)
- blastomatoid variant of carcinosarcoma (BCS)


- The International Pleuropulmonary Blastoma Registry
- Pleuropulmonary blastoma website at Washington University in St.Louis, MI


- Boman F, Hill DA, Williams GM, Chauvenet A, Fournet JC, Soglio DB, Messinger Y, Priest JR. Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma Registry. J Pediatr. 2006 Dec;149(6):850-854. PMID: 17137906

- MacSweeney F, Papagiannopoulos K, Goldstraw P, Sheppard MN, Corrin B, Nicholson AG. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol. 2003 Aug;27(8):1139-46. PMID: 12883247

- Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H, Lapierre JG, Vischoff D, Tucci M, Brochu P. Pleuropulmonary blastoma: a rare pathology with an even rarer presentation. J Pediatr Surg. 1999 Jul;34(7):1057-9. PMID: 10442587

- Yang P, Hasegawa T, Hirose T, Fukumoto T, Uyama T, Monden Y, Sano T. Pleuropulmonary blastoma: fluorescence in situ hybridization analysis indicating trisomy 2. Am J Surg Pathol. 1997 Jul;21(7):854-9. PMID: 9236843

- Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP. Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer. 1997 Jul 1;80(1):147-61. PMID: 9210721

- Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohut G, Freyer DR, Bostrom B, Richardson MS, Barredo J, Dehner LP. Pleuropulmonary blastoma: a marker for familial disease. J Pediatr. 1996 Feb;128(2):220-4. PMID: 8636815

- Hachitanda Y, Aoyama C, Sato JK, Shimada H. Pleuropulmonary blastoma in childhood. A tumor of divergent differentiation. Am J Surg Pathol. 1993 Apr;17(4):382-91. PMID: 7684203

- Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP. Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. Cancer. 1988 Oct 15;62(8):1516-26. PMID: 3048630