Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases.

Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such cases presenting clinically and radiologically as congenital pulmonary airway malformation. On histology, a final diagnosis of type I pleuropulmonay blastoma was made. Thereafter, chemotherapy was administered following complete surgical excision.

Utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration in children with mediastinal pathology.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration (EUS-B-FNA) are useful modalities in the evaluation of mediastinal pathologies in adults. However, sparse data are available in children. OBJECTIVE: To describe the utility and safety of EBUS-TBNA and EUS-B-FNA in children with mediastinal pathologies of unknown etiology. DESIGN: Chart review. SETTING: Pediatric Chest and Tuberculosis Clinics, Department of Pediatrics, AIIMS, New Delhi from May 2015 to March 2018. PATIENTS: Children <18 years of age with mediastinal pathologies of undefined etiology. METHODS: Case records of children who underwent EBUS-TBNA and EUS-B-FNA were reviewed. Data on demographic profile, clinical features, laboratory investigations, the technique of EBUS-TBNA/ EUS -B- FNA, complications, and findings were collected. RESULTS: Thirty children (19 males) with mean (SD) age of 9.6 (±3.5) years underwent endobronchial procedures. Median (IQR) weight(kg) and height(cm) were 29 (19.5, 35) and 134 (125, 150) respectively. Tuberculosis was the most common preprocedure clinical diagnosis (73.3%), followed by lymphoma (13.3%). Presenting features were fever (80%), cough (53.3%), hepatomegaly (13%), peripheral lymphadenopathy (21.7%), and positive tuberculin skin test (63.3%). Approximately one fourth were on antitubercular therapy without definite evidence of TB. Conscious sedation was used for the procedures: midazolam and fentanyl (n = 22), propofol (n = 8). Transesophageal, transtracheal, and both routes were used in 20 (66.6%), 7 (23.3%), and 3 (10.1%), respectively. Lymph-nodes were sampled in 24 children (subcarinal in 16, right paratracheal in 4 and both in 4). Mean (SD) size of lymph node (in cm) on EBUS was 1.93(±0.5) and median (IQR) number of FNAC needle passes per node were 2 (2, 4). The diagnosis was confirmed in 11 (36.6%, tuberculosis in 10 by GeneXpert/ MGIT/ cytopath and lymphoma in one) patients. Only 3.3% had a minor complication. CONCLUSION: EBUS-TBNA and EUS-B-FNA are helpful in children with undiagnosed mediastinal pathology with fair diagnostic yield and excellent patient safety profile.