[Primary rhabdomyosarcoma of the pleura presenting as recurrent pneumothorax]. / Pneumothorax récidivant révélateur d'un rhabdomyosarcome primitif de la plèvre.
Rev Mal Respir
; 26(3): 333-7, 2009 Mar.
Article
em Fr
| MEDLINE
| ID: mdl-19367209
ABSTRACT
INTRODUCTION:
Rhabdomyosarcoma is the most common soft tissue sarcoma in the first two decades of life. Its most common location is the head and neck. It rarely arises in the thorax including the pleura. There are only 7 previously reported cases in the literature. We report a case of primary pleural rhabdomyosarcoma with an unusual clinical presentation and location. Our aim is to discuss the clinical presentation, treatment and prognosis of this uncommon location of rhabdomyosarcoma. CASE REPORT We report a case of primary embryonal rhabdomyosarcoma of the pleura in a boy of 21 months. The presentation was characterized by recurrent spontaneous pneumothorax. A computed tomography scan showed only pleural detachment with no evidence of any pleural disease. The malignant cells incidentally found in routine pleural biopsy were the diagnostic clue. The rhabdomyoblastic nature of these cells was confirmed by positive immunostains for myoD1 and desmin. Investigation for metastases was negative. Despite chemotherapy, the tumour quickly increased in size and the infant died from acute respiratory failure.CONCLUSION:
Thoracic rhabdomyosarcoma is rare and remains clinically silent for a long time. Its management is still controversial. Prognosis is generally poor when compared with other locations.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Pleurais
/
Pneumotórax
/
Rabdomiossarcoma
Tipo de estudo:
Prognostic_studies
Limite:
Humans
/
Infant
/
Male
Idioma:
Fr
Revista:
Rev Mal Respir
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Tunísia