Biphasic pulmonary blastoma in a child

Pulmonary blastoma (PB) is a rare malignant pulmonary tumor composed of immature mesenchyme and/or epithelium that resembles an embryonic lung at 10-16 weeks gestation. PBs constitute only 0.25 to 0.5 percent of all primary malignant lung tumors. Approximately 20 percent of the reported cases have occurred in pediatric patients. A seven-year-old girl presented with fever, cough, respiratory distress and chest pain on the left side. An x-ray, ultrasonography and a computed tomographic scan of the chest showed a large mass consisting of solid and cystic components almost completely occupying the left hemithorax associated with pleural effusion. The diagnosis of biphasic PB was established by histological examination of thoracotomy material. The patient was considered inoperable due to tumor involvement of the mediastinum, and she died two days after the initiation of chemotherapy. We report this case of PB to raise attention to the clinical, radiological and pathological features of PB in childhood because of its rarity.     

Idiopathic pulmonary hemosiderosis: case report

OBJECTIVE: To alert pediatricians about the possibillity of childhood Idiopathic Pulmonary Hemosiderosis, in cases of anemia associated with chronic lung disease. METHODS: This article documents a case of Idiopathic Pulmonary Hemosiderosis in a 6 year-old child, with histopathological documentation, and reviews it against published literature. RESULTS: A 6 year-old child with history of anemia and lung disease characterized by wheezing, recurrent pneumonia and digital clubbing was admitted to the hospital for investigation, where he suffered sudden respiratory failure and hemoptysis.He was submitted to a lung biopsy which showed a histopathological diagnosis compatible with pulmonary hemosiderosis. Therapy with high doses of corticosteroids was initiated with a good early response. After two and a half months of therapy he had a new bleeding episode, culminating in death. CONCLUSIONS: Idiopathic Pulmonary Hemosiderosis should be included as a possible diagnosis of children with anemia and chronic lung disease. This case is a good example.     

Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl

SUMMARY: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant tumor characterized by its epithelioid structure and vascular endothelium origin. The clinical course of HEHE is variable, ranging from long-term survival without treatment to a rapidly progressive course with a fatal outcome. As a consequence, no standard treatment has been determined. We present a case of HEHE occurring in a 13-year-old girl, in which a novel treatment approach using antiangiogenic therapy was tried and was successful in slowing the progression of the disease.     

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.     

Recent advances in diagnosis and management of pulmonary hypertension in chronic lung disease

Pulmonary hypertension with elevated pulmonary vascular resistance is a common cardiovascular complication associated with increased morbidity and mortality in preterm infants with chronic lung disease. Injury to the developing pulmonary circulation results in structural and functional abnormalities of the pulmonary vasculature. Animal studies have demonstrated that disruption of angiogenesis may contribute to the failure of normal alveolarisation in chronic lung disease. Levels of vascular endothelial growth factor in bronchoalveolar lavage fluid are lower in infants with chronic lung disease compared to preterm controls. Supplemental oxygen is commonly used to prevent and treat pulmonary hypertension, although optimal arterial oxygen saturation levels remain uncertain. Other vasodilators such as inhaled nitric oxide appear promising, but as yet have not been evaluated in the form of randomised controlled trials. Further studies are required to investigate the long-term effectiveness of pulmonary vasodilator therapy.     

Recent advances in diagnosis and management of pulmonary hypertension in chronic lung disease

Pulmonary hypertension with elevated pulmonary vascular resistance is a common cardiovascular complication associated with increased morbidity and mortality in preterm infants with chronic lung disease. Injury to the developing pulmonary circulation results in structural and functional abnormalities of the pulmonary vasculature. Animal studies have demonstrated that disruption of angiogenesis may contribute to the failure of normal alveolarisation in chronic lung disease. Levels of vascular endothelial growth factor in bronchoalveolar lavage fluid are lower in infants with chronic lung disease compared to preterm controls. Supplemental oxygen is commonly used to prevent and treat pulmonary hypertension, although optimal arterial oxygen saturation levels remain uncertain. Other vasodilators such as inhaled nitric oxide appear promising, but as yet have not been evaluated in the form of randomised controlled trials. Further studies are required to investigate the long-term effectiveness of pulmonary vasodilator therapy.     

Hemangioendothelioma: A Rare Vascular Tumor in Childhood and Adolescence

Most vascular tumors occurring in children are benign. They are recognized by their ability to form angiomatous structures. In some instances, there is no clear-cut line between a benign vascular tumor (or angioma) and a malignant vascular tumor (or angiosarcoma). The hemangioendothelioma is a rare tumor of vascular origin, involving bone or soft tissue, and represents 1% of all vascular neoplasms. Accurate diagnosis is critical in recommending the most appropriate therapy for each patient. The aim of this paper is to give a brief review of the literature of this rare entity, particularly in childhood and adolescence.     

Thymic carcinoma, systemic lupus erythematosus, and hypertrophic pulmonary osteoarthropathy in an 11-year-old boy: a novel association

Thymic carcinoma is exceptionally rare in children and it has never previously been associated with autoimmune disorders. The authors report the case of an 11-year-old boy with thymic carcinoma, hypertrophic pulmonary osteoarthropathy, and an autoimmune disease that resembled systemic lupus erythematosus. To their knowledge, this is the first case of such complex clinical findings. The tumor was of high grade histologically and the boy died after 1 year, in spite of chemotherapy and radiotherapy. A review is presented of the available medical literature on thymic malignancy in childhood.     

Changes in pulmonary arterial pressure in preterm infants with chronic lung disease

BACKGROUND: Pulmonary arterial pressure (PAP) is raised in preterm infants with respiratory distress syndrome who subsequently develop chronic lung disease. The natural history of pulmonary hypertension in infants with chronic lung disease is unknown. OBJECTIVES: To investigate changes in PAP, assessed non-invasively using Doppler echocardiography, in infants with chronic lung disease during the 1st year of life. METHODS: Serial examinations were performed in infants with chronic lung disease and healthy preterm infants. The Doppler derived acceleration time to right ventricular ejection time ratio (AT/RVET) was calculated from measurements made from the pulmonary artery velocity waveform. RESULTS: A total of 248 examinations were performed in 54 infants with chronic lung disease and 44 healthy preterm infants. The median AT/RVET was significantly lower in infants with chronic lung disease than in healthy preterm infants (0.31 v 0.37). AT/RVET significantly correlated with age corrected for prematurity in both infants with chronic lung disease (r = 0.67) and healthy infants (r = 0.55). There was no significant difference between the rate of change in AT/RVET between the two groups. In infants with chronic lung disease, multivariate analysis showed that AT/RVET was significantly independently associated with age and inversely with duration of supplemental oxygen treatment. Median AT/RVET was significantly lower in infants with chronic lung disease until 40-52 weeks of age corrected for prematurity. CONCLUSIONS: Although PAP falls with increasing age in both infants with chronic lung disease and healthy preterm infants, it remains persistently raised in infants with chronic lung disease until the end of the 1st year of life.     

Pulmonary infiltrates with eosinophilia syndromes in children

Pulmonary infiltrates with eosinophilia (PIE) are a group of heterogeneous disorders having the common findings of lung disease and eosinophilia in the peripheral blood, bronchoalveolar lavage fluid, or pulmonary interstitium. Eleven cases of PIE syndromes were identified through a retrospective and prospective chart review: drug-induced (2), acute eosinophilic pneumonia (3), infant pulmonary eosinophilia (2), parasite-induced (2), Churg-Strauss syndrome (1), and atypical chronic PIE (1). Patient demographics, clinical presentation, and disease severity varied considerably among groups. Therapeutic interventions included bronchodilators (10), oxygen (7), corticosteroids (9), and mechanical ventilation (3). A single patient with acute eosinophilic pneumonia died. Our experience suggests that PIE syndromes are rare in childhood and that clinical presentation can vary widely. Because of the potential for significant morbidity and mortality, aggressive diagnostic evaluations are warranted, particularly in children with respiratory failure of unknown etiology.     

Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.

OBJECTIVE: We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients). DESIGN: Case series. SETTING: Tertiary academic center. PATIENTS: Infants and children ages 0-11 yrs with CDH (n = 3) or CCAM (n = 1) with evidence of chronic pulmonary hypertension by echocardiogram and cor pulmonale (n = 3). INTERVENTIONS: Catheterization and pulmonary vasodilator therapy. MEASUREMENTS AND MAIN RESULTS: Pulmonary vascular resistance, pulmonary arterial pressure, and changes in these measures were assessed. A 20% change in pulmonary vascular resistance was considered a clinically significant response. Ten catheterizations were performed in four patients. All patients had elevated pulmonary vascular resistance and pulmonary arterial pressures at initial catheterizations and significant vasodilation during inhaled nitric oxide. CONCLUSIONS: Chronic lung disease following pulmonary hypoplasia from CDH and CCAM is associated with abnormal pulmonary vascular tone in infants and children with evidence of chronic pulmonary hypertension. Chronic pulmonary vasodilator therapy may improve pulmonary vascular function and enhance lung growth in infants and children who are treated during their period of potential for rapid lung growth.     

Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children. A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. Prednisone++ improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin. CONCLUSIONS: PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.     

Clinical practice: pulmonary hypertension in children

Introduction  

Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases.     

Intralobar pulmonary sequestration associated with a congenital pulmonary airway malformation type II

Intralobar pulmonary sequestration (ILPS) is a rare congenital malformation of the lung. It is described as a segment of lung parenchyma with normal pleura and systemic blood supply and it has poor communication with the tracheobronchial tree. Patients usually present in later childhood or adulthood with a history of recurrent pneumonias. The malformation is rarely associated with other congenital anomalies such as a congenital pulmonary airway malformation (CPAM). A CPAM is a congenital cystic lesion of the lung that presents usually in the newborn period as respiratory distress. We describe the case of a 2-month-old female who presented to the local emergency room with the initial diagnosis of pneumonia, surgical diagnosis of ILPS, and a final pathological diagnosis of ILPS with an associated CPAM.     

Stenosis of pulmonary veins in Down syndrome

Two patients with Down syndrome, intracardiac communications and elevated pulmonary arteriolar resistance presented early in life. Both patients had significant stenosis of pulmonary veins. The progressive nature of the stenosis is illustrated in one patient. Pulmonary venous stenosis in Down syndrome has been recorded only twice before in the literature, and may play a part in the early onset of pulmonary vascular occlusive disease in some patients.     

Chemotherapy of pulmonary blastoma: A case report and review of the literature

Pulmonary blastoma is a rare lung tumor of uncertain histogenesis. Although surgery may be curative therapy for localized lesions, recurrent tumor has been resistant to various treatment modalities. We report a case of metastatic pulmonary blastoma that had a partial remission with combination chemotherapy. Suggestions are made for the treatment of localized and metastatic pulmonary blastoma.     

Unusual Clinical Presentation of Rare Adult Survivors With a Missing Pulmonary Valve Associated With a Missing Left Pulmonary Artery

Tetralogy of Fallot with absent pulmonary valve syndrome is associated with absence of the left pulmonary artery in one-seventh of cases. Infants with this disorder commonly present very early in life with respiratory distress and heart failure. Because the pulmonary blood flow is primarily to the right lung only, severe respiratory distress occurs. Survival to late childhood without surgery is very rare due to clinical worsening in infancy. This report describes two adult natural survivors with this rare association who presented with extremely unusual unreported clinical features. The aneurysmally dilated main pulmonary artery was compressed on the left coronary ostium, causing angina in the first patient. The second patient had recurrent ventricular tachycardia originating from the dilated thinned out right ventricular outflow tract. Both patients had successful surgical correction with valved xenograft conduits.     

Long-term pulmonary function in survivors of childhood Hodgkin disease and non-Hodgkin lymphoma

BACKGROUND: The aim of our study was to evaluate the long-term effects of chemotherapy and/or radiotherapy on lung function in 75 childhood Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL) survivors several years after treatment. PATIENTS AND METHODS: We studied 37 HD and 38 NHL survivors. These patients were divided into two groups according to the treatment protocols applied. Group I consisted of 23 patients who were treated with both chemotherapy and thoracic irradiation and Group II consisted of 52 patients who were treated with chemotherapy and no thoracic irradiation. A detailed history of smoking habits, respiratory symptoms, and diseases was recorded. Complete physical examinations and pulmonary function tests [PFT, including spirometry, lung volume, and diffusion capacity for carbon monoxide (DLCO)] were performed on all subjects. RESULTS: No patients reported acute or chronic respiratory symptoms or diseases. Pulmonary function abnormality (reduced lung volume and diffusion capacity) was found in 13% of patients at a median 5 years after diagnosis. The percentage of predicted normal value of forced expiratory volume in the 1st sec (FEV(1)), residual volume (RV), and DLCO were significantly lower in Group I than these values for Group II. There were no significant differences in PFT parameters between patients with HD and NHL (P > 0.05). It appears that the risk of reduced lung function was greater the younger the patient in therapy. CONCLUSION: Chemotherapy or chemo-radiotherapy-induced pulmonary sequalae in childhood may remain asymptomatic for many years.     

Reversible pulmonary arterial hypertension in a 6-year-old girl with extrinsic allergic alveolitis

A case is reported of a 6-year-old girl who developed extrinsic allergic alveolitis due to hypersensitivity to her pet budgerigars. Pulmonary artery pressure measurements obtained by a cardiac catheterization demonstrated a significant increase in the pulmonary vascular resistance during the acute stage of the disease. Six months after exclusion of exposure to the budgerigars the hemodynamic of the pulmonary circulation was normalized. The present case thus shows that a reversible increase in the pulmonary vascular resistance in extrinsic allergic alveolitis of childhood may occur without development of an irreversible pulmonary fibrosis.     

Severe acute White-Spirit poisoning with pulmonary hypertension]

The Authors report a case of acute White-Spirit poisoning with pulmonary hypertension associated to respiratory distress syndrome. CASE REPORT: A 14-month-old infant drank an unknown quantity of White-Spirit while his parents were painting. After he spontaneously vomited, he presented a seizure at the emergency department. After a 36 h stay in Pediatric Intensive Care Unit (PICU), acute lung injury required mechanical ventilation and vasoactive support. Cardiac ultrasounds showed pulmonary hypertension, which rapidly resolved with inhaled nitric oxide. The child was discharged of PICU after five days. Respiratory follow-up two months after poisoning was normal. CONCLUSION: Pulmonary hypertension should be checked for in case of White-Spirit ingestion complicated with severe acute lung injury.