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pleuropulmonary blastoma
MIM.601200
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.
Images
Pleuropulmonary blastoma (PPB)
- https://twitter.com/amy_treece13/status/875049771341418496
- https://twitter.com/AngelPathology/status/875140925978181632
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).
Types
type 1 pleuropulmonary blastoma (purely cystic PPB)
type 2 Pleuropulmonary blastoma (solid and cystic PPB)
Epidemiology
sporadic pleuropulmonary blastoma
familial pleuropulmonary blastoma (8636815)
Macroscopy
cystic or solid (10870072)
unilateral or bilateral (10839886)
Variants
epithelial pulmonary blastoma: low-grade adenocarcinoma of the fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA)
Associations
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
- medulloblastoma
- thyroid tumors
- sarcomas
- intestinal hamartomatous polyp (10442587)
- ovarian teratoma (10602867)
- carcinosarcoma (7982680)
- neurofibromatosis (8432176)
- hematological proliferations
- colonic hamartomatous polyp
- nasal chondroid hamartoma (nasal chondromesenchymal hamartoma) (17091268)
other conditions
- fatal hypercalcemia (9154681)
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
Cytogenetics
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
| 8p11-p12 |
| 2q |
Differential diagnosis
congenital pumonary airways malformations (pulmonary cystic adenomatoid malformations - PCAMs)
blastomatoid variant of carcinosarcoma (BCS)
Links
The International Pleuropulmonary Blastoma Registry
Pleuropulmonary blastoma website at Washington University in St.Louis, MI
References
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
