Pleuropulmonary blastoma type I and congenital pulmonary airway malformation type 4: distinct entities or sides of the same coin?

Pleuropulmonary blastoma is an extremely rare lung neoplasm exclusive to children under 5 years of age. It presents a diagnostic challenge both prenatally and in early childhood due to its similarity to benign lung cysts, which are managed differently. We present the first case, to our knowledge, of a neonate with pleuropulmonary blastoma and myelomeningocele, though prenatally diagnosed as a congenital pulmonary airway malformation. We detail the prenatal imaging that facilitated counseling and delivery management in addition to the correlating postnatal imaging.

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A Rare Case of Congenital Pulmonary Airway Malformation Associated with Intralobar Pulmonary Sequestration in an Adult - Case Report

ARS Medica Tomitana ◽

10.2478/arsm-2018-0029 ◽

2018 ◽

Vol 24 (3) ◽

pp. 161-163

Author(s):

Suciu Bogdan Andrei ◽

Hălmaciu Ioana ◽

Bud Vasile ◽

Mezei Tibor ◽

Molnar Călin ◽

...

Keyword(s):

Respiratory Symptoms ◽

Pulmonary Sequestration ◽

Type I ◽

Type Ii ◽

Adult Case ◽

Congenital Pulmonary Airway Malformation ◽

Intralobar Pulmonary Sequestration ◽

Angio Ct ◽

Pulmonary Airway ◽

Multiple Cyst

Abstract The congenital pulmonary airway malformations (CPAM) are extremely rare malformations which are most frequently diagnosed before birth or during childhood and are extremely rarely diagnosed in adults. Sometimes, type II CPAM can be associated with pulmonary sequestrations (PS), especially those extralobar. We present the case of a 39 years old female without any known previous respiratory symptoms until this age, who has having for approximatively 2 months repeated episodes of haemoptysis. The angio-CT exam showed the existence of multiple cyst-like formations in the left inferior pulmonary lobe (LLL) and 2 arteries starting from thoracic aorta that participated at the vascularization of the LLL. We performed a left inferior lobectomy and the postoperative evolution was favorable. The histopathological exam confirmed the diagnosis of type I CPAM associated with intralobar PS. The particularity of the presented case is given by the fact that this patient of 39 years old had no previous respiratory symptoms and no cases of type I CPAM associated with intralobar PS were cited before.

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It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation

The Thoracic and Cardiovascular Surgeon Reports ◽

10.1055/s-0037-1598625 ◽

2017 ◽

Vol 06 (01) ◽

pp. e10-e14 ◽

Cited By ~ 3

Author(s):

Fayza Haider ◽

Khulood Al Saad ◽

Fatima Al-Hashimi ◽

Hakima Al-Hashimi

Keyword(s):

Early Childhood ◽

Poor Prognosis ◽

Lung Neoplasm ◽

Good Prognosis ◽

Pleuropulmonary Blastoma ◽

Type I ◽

Congenital Pulmonary Airway Malformation ◽

Cystic Lung ◽

Malignant Changes ◽

Infancy And Early Childhood

Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis.We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early.

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