Case Reports
Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: A case report and review of the literature*

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Abstract

The current report describes a 13-month-old boy with primary pulmonary rhabdomyosarcoma (RMS) that originated within a congenital cystic adenomatoid malformation (CCAM). To the best of our knowledge, he is the youngest patient of all primary pulmonary RMS cases reported in the English-language literature. The tumor, localized in left upper lobe, was removed completely, and histologic examination showed embryonal subtype. Postoperative systemic chemotherapy was carried out. Recent evaluation 15 months after resection has not identified any residual or recurrent disease. Primary pulmonary RMS, although very rare in the pediatric age group, should be considered in young patients with solitary pulmonary masses and associated cystic lesions. J Pediatr Surg 36:1062-1065. Copyright © 2001 by W.B. Saunders Company.

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Case report

A 13-month old boy with a 3-month history of persistent cough, fever, and dyspnea was admitted to a pediatric surgical center for a left pleural effusion (Fig 1).

. Initial chest radiograph shows a left pleural effusion.

There he had been treated with antibiotherapy and tube thoracostomy with a misdiagnosis of empyma. Chest radiograph after completion of treatment showed an air cyst together with a large mass in the left hemithorax (Fig 2), and he was referred to our department for further

Discussion

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and occurs commonly in the head, neck, and urogenital organs. Primary pulmonary neoplasms are unusual in the pediatric age group, and RMS of the lung is one of the rarest. It accounts only 0.5% of childhood RMS2, and for 4.4% of childhood pulmonary neoplasms.1 Only 3 of 2,747 children included in the Intergroup Rhabdomyosarcoma Study had a primary pulmonary lesion.2, 3, 4

In review of the English-language literature, we found

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    *

    Address reprint requests to Coşkun Özcan, Ege University, Faculty of Medicine, Department of Pediatric Surgery, 35100 Bornova-İzmir, Turkey.

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