Pleuropulmonary blastoma

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Pleuropulmonary blastoma
Other names: Pulmonary blastoma
Usual onsetVery young children[1]
TypesI, II and III[1]

Pleuropulmonary blastoma (PPB) is a cancer originating in the lung or its lining.[1] It occurs most often in children younger than 6-years old.[1]

Around 70% of cases of pleuropulmonary blastoma are a type of DICER1 syndrome; associated with a germline mutation of the tumor suppressor gene, DICER1.[2][3] 92% are in very young children and comprise of types I, II and III.[1] Type I does not spread, but spread may be present at diagnosis of types II and III.[1]

Pleuropulmonary blastoma is rare.[1] It accounts for less tham 0.5% of lung cancer.[2] It was first described in 1988.[4]

Signs and symptoms

Symptoms may include coughing, an upper respiratory tract infection, shortness of breath, and chest pain. These symptoms are very non-specific, and can be caused by other types of tumor in the lung or mediastinum more generally, and by other conditions. Imaging (X-ray, CT, MRI) may be used to determine the presence and precise location of a tumor, but not a specific diagnosis of PPB or other tumor.[1] Doctors are unable to tell if a child has PPB right away, and not upper respiratory tract infection, until more test are taken and they show that there is no infection. Another symptom is pneumothorax.[citation needed]


A number of PPBs have shown trisomy 8 (17 out of 23 cases studied per the PPB registry). Trisomy 2 and p53 mutations/deletions have also been described.[citation needed]

Around 70% of cases of pleuropulmonary blastoma are a type of DICER1 syndrome; associated with a germline mutation of the tumor suppressor gene, DICER1.[2]


Diagnosis is by biopsy. Other tests include x-rays, CAT scans, and MRI's.[citation needed]


Pleuropulmonary blastoma is classified into 3 types:

  • Type I is multicystic
  • Type II shows thickening areas (nodules) within this cystic lesion
  • Type III shows solid masses.

Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts, and there is some evidence that not all type I PPB will progress to types II and III.[5] Types II and III are aggressive, and cerebral metastasis is more frequent in PPB than in other childhood sarcomas.[6]


Treating PPB depends on the size and location of the tumor, whether the cancer has spread, and the child's overall health. Surgery is the main treatment for PPB. The main goal of surgery is to remove the tumor. If the tumor is too large to be completely removed, or if it's not possible to completely remove the tumor, surgery may be performed after chemotherapy. Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis.[citation needed]


In a retrospective review of 204 children with lung tumors, pleuropulmonary blastoma and carcinoid tumor were the most common primary tumors (83% of the 204 children had secondary tumors spread from cancers elsewhere in the body).[7]


Pleuropulmonary blastoma was first described in 1988.[1]

An international registry has been established.[5]

See also


  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 Schultz, Kris Ann P.; Messinger, Yoav (2022). "18. Pleuropulmonary Blastoma". In Schneider, Dominik T.; Brecht, Ines B.; Olson, Thomas A.; Ferrari, Andrea (eds.). Rare Tumors in Children and Adolescents. Springer. pp. 159–168. ISBN 978-3-030-92071-5. Archived from the original on 2022-06-30. Retrieved 2022-06-27.
  2. 2.0 2.1 2.2 Watanabe, Takafumi; Soeda, Shu; Endo, Yuta; Okabe, Chikako; Sato, Tetsu; Kamo, Norihito; Ueda, Makiko; Kojima, Manabu; Furukawa, Shigenori; Nishigori, Hidekazu; Takahashi, Toshifumi; Fujimori, Keiya (29 January 2022). "Rare Hereditary Gynecological Cancer Syndromes". International Journal of Molecular Sciences. 23 (3): 1563. doi:10.3390/ijms23031563. ISSN 1422-0067. PMID 35163487. Archived from the original on 30 June 2022. Retrieved 27 June 2022.
  3. WHO Classification of Tumours Editorial Board, ed. (2020). "16. Genetic tumour syndromes of the female genital tract: DICER1 syndrome". Female genital tumours: WHO Classification of Tumours. Vol. 4 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 556–557. ISBN 978-92-832-4504-9. Archived from the original on 2022-06-17. Retrieved 2022-06-25.
  4. Manivel JC, Priest JR, Watterson J, et al. (October 1988). "Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood". Cancer. 62 (8): 1516–26. doi:10.1002/1097-0142(19881015)62:8<1516::AID-CNCR2820620812>3.0.CO;2-3. PMID 3048630.
  5. 5.0 5.1 Hill DA, Jarzembowski JA, Priest JR, Williams G, Schoettler P, Dehner LP (February 2008). "Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry". Am. J. Surg. Pathol. 32 (2): 282–95. doi:10.1097/PAS.0b013e3181484165. PMID 18223332. S2CID 3193037.
  6. Priest JR, Magnuson J, Williams GM, Abromowitch M, Byrd R, Sprinz P, Finkelstein M, Moertel CL, Hill DA (September 2007). "Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma". Pediatr Blood Cancer. 49 (3): 266–73. doi:10.1002/pbc.20937. PMID 16807914. S2CID 20486633.
  7. Dishop MK, Kuruvilla S (July 2008). "Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital". Arch. Pathol. Lab. Med. 132 (7): 1079–103. doi:10.5858/2008-132-1079-PAMLTI. PMID 18605764.

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