Pleuropulmonary Blastoma

Department of Nursing and Patient Care Services, Riley Hospital For Children, 1960, Indianapolis, IN 46202, USA.
Journal of Pediatric Oncology Nursing (Impact Factor: 0.9). 10/2008; 25(6):295-302. DOI: 10.1177/1043454208323292
Source: PubMed


Pleuropulmonary blastoma (PPB) is a dysontogenetic neoplasm of childhood that involves lung and/or pleura. There is an increased incidence of neoplasias and dysplasias among young relatives of children with PPB. Pathophysiologically, PPB evolves from a cystic to solid state over time. It is subclassified as type I (purely cystic), type II (both cystic and solid elements), and type III (completely solid). Type II and type III may be associated with metastasis, with the brain being the most common metastatic site. The absence of epithelial malignancy in PPB is a feature that distinguishes it from the adult-type pulmonary blastoma. The clinical presentation includes signs and symptoms associated with various respiratory disorders. To make a definitive diagnosis of PPB, an examination of the cystic fluid or solid tumor is required. Treatment for PPB consists primarily of surgery and chemotherapy. Nursing care is directed toward maintaining normal respiratory and neurological function, maintaining normal fluid and electrolyte balance, minimizing side effects associated with treatment, and providing education for the family.

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    ABSTRACT: Pleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma. Five cases of PPB were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed. Five cases of patients suffered from PPB were aged from 21 to 47 months (mean 32.8 months). Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sarcomatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Desmin and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99. Pleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation (CCAM) and embryonal rhabdomyosarcoma.
    Zhongguo fei ai za zhi = Chinese journal of lung cancer 05/2010; 13(5):550-3. DOI:10.3779/j.issn.1009-3419.2010.05.31
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    ABSTRACT: F-FDG PET/CT scanning plays an important role in the management of thoracic malignancy. The authors would like to present FDG PET/CT images of a rare thoracic malignancy, pulmonary blastoma in adulthood. The patient had recurrent metastatic disease of previously resected primary pulmonary blastoma. The foci of recurrent metastases in lung, mediastinum, and subcutaneous tissue are intensely FDG-avid.
    Clinical nuclear medicine 06/2013; 38(9). DOI:10.1097/RLU.0b013e3182995e2d · 3.93 Impact Factor
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    ABSTRACT: Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting younger children, even in newborns with an unfavorable outcome. PPB is histologically composed of a primitive, variably mixed blastematous and sarcomatous components, and exclusively subclassified as type I (purely cystic), type II (both cystic and solid elements) and type III (completely solid) by increasing histological evidence of malignancy. At present, well-documented cases or cases of truly precise presentation of either pathological or immunohistochemical findings in PPB are rare. The authors report one case of PPB in a 44-month-old child presenting as a solid and cystic mass with special emphasis on its radiological, histopathological and immunohistochemical aspects. The histological diagnosis was PPB, which would belong to the type II category.
    Fetal and pediatric pathology 09/2013; 33(1). DOI:10.3109/15513815.2013.839011 · 0.48 Impact Factor
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