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Bilateral Pleuropulmonary Blastoma (PPB) in a 2- Year-Old Girl: a Case Report with Review of Literature Pradosh Kumar Sarangi, H

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Bilateral Pleuropulmonary Blastoma (PPB) in a 2- Year-Old Girl: a Case Report with Review of Literature Pradosh Kumar Sarangi, H J M e d A l l i e d S c i 2 0 1 7 ; 7 ( 1 ) : 67- 71 Journal of www.jmas.in M edical & Print ISSN: 2 2 3 1 1 6 9 6 Online ISSN: 2 2 3 1 1 7 0 X Allied Sciences Case report Bilateral pleuropulmonary blastoma (PPB) in a 2- year-old girl: A case report with review of literature Pradosh Kumar Sarangi, H. S. Sagar, Sanjay Kumar Nahak, Jayashree Mohanty, Sasmita Parida Department of Radiodiagnosis, S. C. B. Medical College & Hospital, Manglabag, Cuttack-753007, Odisha, India. Article history: Abstract Received 12 December 2016 Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of Revised 18 January 2017 infancy and early childhood accounting for 0.25-0.5% of primary lung tu- Accepted 24 January 2017 Early online 28 January 2017 mors. PPB is a dysontogenic tumor composed of immature malignant epi- Print 31 January 2017 thelial and/or mesenchymal tissues whose features may resemble early embryological lung tissues. It is the pulmonary analog of other tumors of Corresponding author childhood including Wilms' tumor, neuroblastoma, hepatoblastoma, Pradosh Kumar Sarangi pancreatoblastoma and retinoblastoma. Morphologically, PPB has three Junior Resident, types (I, II, and III). A fourth type (Ir) was added in 2006 by PPB registry. Department of Radiodiagnosis, Type II and III are very aggressive malignancies with metastatic potential. S. C. B. Medical College & Hospital, Manglabag, Cuttack-753007, Bilateral PPB is very rare. To the best of our knowledge, only two cases of Odisha, India. bilateral PPB have been reported. No cases of bilateral PPB have been Phone: +91-7735860824 reported from India. Herein, we are reporting a case of bilateral PPB (type Email: [email protected] II) in a 2-year-old girl who presented with cough, dyspnea and mild fever for past 4 months. She died few days after admission. Key words: Bilateral pleuropulmonary blastoma, Bronchogenic cyst, Congenital pulmonary airway malformations, Dysontogenic tumor, PPB registry, Primitive neuroectodermal tumors DOI: 10.5455/jmas.252500 © 2017 Deccan College of Medical Sciences. All rights reserved. leuropulmonary blastoma (PPB) is a rare tal muscle, cartilage, fibrous tissue, sometimes fat intrathoracic tumor accounting for 15% of all and most often includes epithelium. The P primary pediatric pulmonary tumors1. Other mesenchymal elements are regarded as malignant3. primary pediatric pulmonary tumors include bron- They usually occur exclusively in children younger chogenic carcinoma and bronchial adenoma. About than 5 years and there is no gender predilection and 25% of PPB cases occur in familial settings showing the tumor occurs more commonly on the right side1. association with other malignant This neoplasm constitutes a distinct entity different (rhabdomyosarcoma, synovial sarcoma, thyroid car- from pulmonary blastoma in adults which is charac- cinoma, ovarian sertoli leydig cell tumors, gonadal terized by immature blastema, stroma and epitheli- germ cell tumors) and benign (thyroid nodule, cystic um; all of neoplastic nature5.There are few reports of nephroma, colonic polyps, neurofibromatosis) tu- bilateral PPB6,7. No cases of bilateral PPB have mors in first and second degree relatives1-4. PPB is been reported from India to the best of our a dysontogenetic intrathoracic neoplasm and ac- knowledge. cording to World Health Organization (WHO) classi- Case report fication of lung tumors they are classified as mesenchymal neoplasms. It is composed of imma- A 2-year-old girl presented with the complaint of ture mesenchyme, often differentiating toward skele- right sided chest pain, mild fever and shortness of 67 Sarangi PK et al Bilateral pleuropulmonary blastoma (PPB) breath for 4 months. She was diagnosed as pneu- monia based on chest X ray findings and started on antibiotics in an outside hospital. But, there was no improvement in patient’s condition. At admission to our hospital, she was dyspneic with respiratory rate of 60/min, BP: 120/70mm Hg, pulse rate: 120/min and oxygen saturation of 91% on room air. Subcos- tal, intercostal retractions and nasal flaring were present. There was no cyanosis or clubbing. She had no significant medical or surgical history. She had no significant family history. Chest examination revealed bilateral dullness on percussion, more on right side. Examination of other systems was unre- markable. Routine blood examination was normal. Chest radiograph showed opacity of the right hemithorax (Fig 1). Thoracic ultra sonography (USG) showed a large homogenous mass in right hemithorax without calcification (Fig 2). Computed tomography (CT) of thorax revealed a large, hypodense homogeneous round tumor with cystic Fig 2. USG Thorax showing a hypoechoic mass lesion. components occupying almost whole of the right hemithorax. Solid components showed moderate Discussion enhancement. Similar lesion of smaller size was PPB present as parenchymal and/or pleural based noted in left hemithorax. The mass lesion abuts mass lesion and divided into three types (I, II, and chest wall and pericardium. Mild right pleural effu- III) based on morphological characteristics by sion noted. There was no evidence of chest wall Dehner et al8. A fourth type (Ir) was added in 2006 invasion, rib erosion or calcification within mass (Fig by PPB registry9. Type Ir (type I-regressed) tumors 3). She underwent USG-guided fine-needle aspira- are cystic containing few spindle shaped cells in the tion cytology (FNAC) which was suggestive of PPB cyst wall with few foci of dystrophic calcification but (Fig 4). She was shifted to pediatric intensive care without subepithelial malignant cell condensation. It unit. Unfortunately, she died few days after admis- might represent a regressed or a genetically des- sion possibly due to respiratory compromise. Based tined but abortive type I tumor. on radiological and cytological findings, diagnosis of bilateral PPB type II was made. Certain genetic mutations are associated with pleuropulmonary blastomas including germ line DICER1 mutation (loss of function) in familial cases, gains of chromosome 8 (most consistent chromo- somal abnormality), trisomy 2, unbalanced translo- cation between chromosomes 1 and X, and p53 mu- tations or deletions2. Type I (14%) tumors are less aggressive ‘Purely Cystic’ tumors which presents at earlier age with median age of diagnosis 10 months8 and 5-year survival rate of 83%. They account for 15 to 20% of all PPB1. The cyst wall is lined by cuboidal or co- lumnar ciliated respiratory epithelium. Under the epi- thelium there is cambium-like areas with proliferation of primitive mesenchymal cells with foci of blastematous cells3,9-11. Type II (48%) tumors are ‘Solid and Cystic’ tumors where cystic lesion shows features of type I PPB and solid lesions shows feature of type III PPB and Fig 1. Chest radiograph PA view showing a pleural-based opacity median age of diagnosis 34 months. Type III (38%) occupying almost the whole of right hemithorax. There is loss of tumors are ‘Purely solid’ with the median age of di- right hemidiaphragmatic silhouette. Subtle opacity is also noted in 10 left middle zone. agnosis of 44 months . Both type II and type III tu- J Med Allied Sci 2017; 7(1) 68 Sarangi PK et al Bilateral pleuropulmonary blastoma (PPB) mors are more aggressive with 5-year survival rate ing from brain metastasis. Metastasis to other areas of 42% despite chemotherapy and radiotherapy. The may lead to signs and symptoms associated with solid component seen in type II and III tumors are abnormalities in the affected organs and systems of similar and show blastematous and sarcomatous the body. Detail family history is needed to rule out characteristics8.The pathogenetic linkage among the familial association. Radiological features show var- cystic, cystic and solid, and solid PPB is supported ied appearance depending upon tumor type. Chest by the recurrence of a type I PPB as type II PPB, X ray shows partial or complete opacification of with both epithelial-lined cysts and complex hemithorax with mediastinal shift to contralateral blastematous and sarcomatous features in the solid side. Transectional imaging shows unilocular cysts, component. These three tumor types form a contin- a multicystic structure, a cyst containing a polypoid uum with progression over time from type I to type III mass, and solid-cystic or entirely solid masses of tumor1. At times type II and III tumors show hemor- variable sizes located peripherally in the lung with or rhagic and necrotic areas may be seen. The histo- without involvement of the pleura or chest wall and logic components of type III PPB generally include may fill the entire hemithorax1,10. blastematous islands, cartilaginous nodules, 1 The diagnosis of PPB is often missed at first, be- rhabdomyoblasts, and anaplastic cells . cause the clinical and radiographic findings are Clinically, the patient presents with chest or upper thought to indicate other respiratory disorders such abdominal pain, fever, dyspnea, cough, hemoptysis, as pneumonia or a benign congenital cyst, particu- anorexia, malaise, or neurological symptoms result- larly a congenital cystic adenomatoid malformation. Fig 3. (a) Axial Non-contrast computed tomography (CT) mediastinal and lung window showing bilateral homogenous hypodense mass in both hemithoraces, almost occupying whole of the right hemithorax. No evidence of calcification, chest wall invasion, rib erosion was noted. (b) Axial contrast enhanced CT showing few cystic areas within the mass. there is moderate enhancement of the
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