Pleuropulmonary blastoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2], Maria Fernanda Villarreal, M.D. [3]

Synonyms and Keywords: Pulmonary blastoma; PPB

Overview

Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignancy originating in the lung or pleural cavity. Pleuropulmonary blastoma occurs most often in infants and young children.[1] The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue. The p53 mutations/deletions have been associated with the development of pleuropulmonary blastoma. Pleuropulmonary blastoma may be classified into 3 groups: type I, II, III. Common causes of pleuropulmonary blastoma include trisomy 8, trisomy 2, and p53 mutations/deletions (e.g., Li-Fraumeni syndrome). On gross pathology, characteristic findings of pleuropulmonary blastoma include extra-pulmonary location, and attachment to the parietal pleura. Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old. Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.[2]

Historical Perspective

  • Pleuropulmonary blastoma was first discovered by Dr. Juan C. Manivel, MD in 1988.[3]

Classification

  • Pleuropulmonary blastoma may be classified into 3 groups:[2]
  • Type I: multicystic lesions
  • Type II: thickened areas (nodules) within cystic lesions
  • Type III: solid masses
  • Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts; not all type I PPB will progress to types II and III.

Pathophysiology

  • The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue.[2]
  • The p53 and DICER-1 gene mutations/deletions have been associated with the development of pleuropulmonary blastoma.[2]
  • On gross pathology, characteristic findings of pleuropulmonary blastoma include:[4]
  • On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
  • On inmunohistochemistry analysis, characteristic findings of pleuropulmonary blastoma include:[2]
  • Positive for TTF-1
  • Positive for vimentin
  • Positive for epithelial membrane antigen (EMA)

Causes

  • Common causes of pleuropulmonary blastoma include:[2]

Differentiating Pleuropulmonary Blastoma from Other Diseases

Epidemiology and Demographics

  • Pleuropulmonary blastoma is very uncommon.[2]

Age

  • Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old.
  • Pleuropulmonary blastoma is more commonly observed among infants and children.
  • Pleuropulmonary blastoma is less commonly observed among adults.[4]

Gender

  • Pleuropulmonary blastoma affects men and women equally.

Race

  • There is no racial predilection for pleuropulmonary blastoma

Risk Factors

  • There are no risk factors associated with the development of pleuropulmonary blastoma.[2]

Natural History, Complications and Prognosis

  • The majority of patients with pleuropulmonary blastoma are asymptomatic.
  • Pleuropulmonary blastoma is usually an incidental finding during routine examination.[4]
  • Early clinical features include persisting upper respiratory tract infection, coughing, and shortness of breath.
  • If left untreated, the majority of patients with may progress to develop acute respiratory distress syndrome.
  • Common complications of pleuropulmonary blastoma include respiratory failure, pneumonia, or mortality.
  • Prognosis is generally poor, and the 5-year survival rate of patients with pleuropulmonary blastoma is approximately 15%.
  • Pleuropulmonary blastomas larger than 5 cm have a worse prognosis.

Diagnosis

Symptoms

  • Pleuropulmonary blastoma is usually asymptomatic.
  • Symptoms of pleuropulmonary blastoma are often non-specific.
  • Symptoms of pleuropulmonary blastoma may include the following:[4]

Physical Examination

  • Patients with pleuropulmonary blastoma usually have dysmorphic facies.[4]
  • Physical examination may be remarkable for:

Laboratory Findings

  • There are no specific laboratory findings associated with pleuropulmonary blastoma.

Imaging Findings

  • CT is the imaging modality of choice for pleuropulmonary blastoma. On CT, findings of pleuropulmonary blastoma may include:
  • Large mass in the thorax with solid mixed cystic heterogeneous low attenuation
  • Pleural effusion
  • Contralateral mediastinal shift
  • Lack of chest wall invasion

Treatment

Medical Therapy

  • There is no treatment for pleuropulmonary blastoma; the mainstay of therapy is supportive care.[4]

Surgery

  • Surgery is the mainstay of therapy for pleuropulmonary blastoma.
  • Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.

Prevention

  • There are no primary preventive measures available for pleuropulmonary blastoma.[4]

References

  1. Indolfi P, Casale F, Carli M, et al. (September 2000). "Pleuropulmonary blastoma: management and prognosis of 11 cases". Cancer. 89 (6): 1396–401. PMID 11002236. doi:10.1002/1097-0142(20000915)89:6<1396::AID-CNCR25>3.0.CO;2-2. 
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016
  3. Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR; et al. (1988). "Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood.". Cancer. 62 (8): 1516–26. PMID 3048630. 
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Dehner LP (1994). "Pleuropulmonary blastoma is THE pulmonary blastoma of childhood". Semin Diagn Pathol. 11 (2): 144–51. PMID 7809508. 

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