Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature

Abstract

Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor originates from either lung or pleura. It was recently linked to the DICER I mutation as a part of predisposition syndrome for different type of tumor. It is characterized histologically by a primitive, variably mixed blastomatous and sarcomatous tissue. PPB is classified into four subtypes: cystic (type I and type Ir); cystic and solid (type II); solid (type III). PPB has no characteristic imaging findings. Integrated imaging can help to make a differential diagnosis and to recognize the subtypes in order to set up therapy. An early recognition and differentiation from congenital airway malformations and other benign cysts are very important. The treatment consists in a multimodal therapy including surgery and chemoterapy. We report a case of 3 years old female admitted at our hospital with fever, non productive cough and dyspnea, who was diagnosed with type II PPB.

A rare case of type III pleuropulmonary blastoma infiltrating the left heart in an 11-year-old girl

“…( 2 , 3 ) Histopathology must be done because the PPB type was the strongest outcome predictor. ( 1 , 2 , 9 ) The gross lesion of our patient is a well-circumscribed white tan solid mass with necrosis and hemorrhage in the friable areas. The microscopic elements of our patient's lesion consist of extensive areas of necrosis with hyperplastic round oval to spindle-shaped cells that condense and group together and give a blastemal appearance, some form an adenomatoid image, and some form a fasciculus image.…”

“…It may not be helpful in a significant tumor mistaken for massive pleural effusion. ( 9 ) Computed Tomography (CT) of the chest, abdomen, and head are useful in excluding other diseases, estimating tumor size, and locating metastases. Echocardiography should be done to assess the heart function and intracardiac anatomy if the tumor extends to the heart chamber.…”

“…Echocardiography should be done to assess the heart function and intracardiac anatomy if the tumor extends to the heart chamber. ( 2 ) Seeking germline mutation in the DICER1 gene might be helpful as it happens in 66 % -80 % of children with PPB ( 1 , 2 , 9 ), but it was unrelated to the outcome. ( 2 , 3 ) Histopathology must be done because the PPB type was the strongest outcome predictor.…”

“…The regiments used in therapy are combinations of drugs (vincristine, actinomycin-D, ifosfamide, doxorubicin, carboplatin, etoposide, epirubicin, and cyclophosphamide) like CEVAIE, VAIA, IVA, VAC, VACA, VA, and IVAD. ( 1 , 5 , 9 , 12 ). Complete resection of the tumor with radical surgery should be applied for surgical treatment.…”

Cytologic diagnostic approach of pleuropulmonary blastoma: a case report

“…9 Clinically, PPB patients may present with chest or upper abdominal pain, dry cough, fever, dyspnea, tachypnea, fatigue, respiratory distress with or without an associated pneumothorax, hemoptysis, anorexia, malaise, or neurological symptoms resulting from brain metastases. 10 PPB is often difficult to diagnose due to non-specific imaging findings. It may appear as a cystic lung mass and therefore it should be considered in the differential diagnosis of other benign cystic lung lesions on imaging findings.…”