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In most cases the outcome of a fetus with CPAM is very good. In rare cases, the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CPAM can be life-threatening for the fetus. CPAM can be separated into five types, based on clinical and pathologic features. [2]
It occurs most often in infants and young children [1] but also has been reported in adults. [2] 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). [ 1 ]
About 60% to 70% of children with this condition die from respiratory failure as babies or young children. Children with Jeune syndrome who survive often develop problems with their kidneys, and over time they may experience kidney failure. [2] As a result, few children with Jeune syndrome live into their teen years.
Lung cancer is the most diagnosed and deadliest cancer worldwide, with 2.2 million cases in 2020 resulting in 1.8 million deaths. [3] Lung cancer is rare in those younger than 40; the average age at diagnosis is 70 years, and the average age at death 72. [2] Incidence and outcomes vary widely across the world, depending on patterns of tobacco use.
Bronchopulmonary dysplasia (BPD; part of the spectrum of chronic lung disease of infancy) is a chronic lung disease which affects premature infants. Premature (preterm) infants who require treatment with supplemental oxygen or require long-term oxygen are at a higher risk. [ 1 ]
Alveolar capillary dysplasia (ACD) is a rare, congenital diffuse lung disease characterized by abnormal blood vessels in the lungs that cause highly elevated pulmonary blood pressure and an inability to effectively oxygenate and remove carbon dioxide from the blood.
Medical diagnosis of pulmonary hypoplasia in utero may use imaging, usually ultrasound or MRI. [12] [13] The extent of hypoplasia is a very important prognostic factor. [14]One study of 147 fetuses (49 normal, 98 with abnormalities) found that a simple measurement, the ratio of chest length to trunk length, was a useful predictor of postnatal respiratory distress. [15]
Unlike extensive-stage small cell lung cancer, limited-stage small cell lung cancer is potentially curable. [4] In limited small cell lung cancer, the median overall survival time is approximately 12–16 months, with five year survival rate of approximately 26% and long-term survival rate of approximately 4–5%. [19]