Primary rhabdomyosarcoma of the diaphragm: case report and literature review

Author:

Medeiros Cleverson Winston de Liz1,Kondo William1,Baptista Júnior Ivo1,Vizzotto Júnior Alvo Orlando1,Noronha Lúcia de1,Hakim Neto Calixto Antonio1

Affiliation:

1. Hospital Nossa Senhora das Graças

Abstract

The authors report a case of primary rhabdomyosarcoma of the diaphragm, an extremely rare presentation with only 14 cases reported in the literature. An 18-year-old male presented 2 spontaneous occurrences of pneumothorax. Computed tomography and magnetic resonance showed a tumoral mass on the right diaphragmatic surface, and after biopsy, the diagnosis was compatible with spindle cell rhabdomyosarcoma. Because the visceral pleura was invaded by the tumoral mass, a right pleuropneumonectomy was performed. The patient received adjuvant chemotherapy, and there was no evidence of disease 15 months after the operation. Based on the Intergroup Rhabdomyosarcoma Study Group (IRSG) criteria, which consider the extent of the disease and its surgical resectability, rhabdomyosarcomas can be classified into 4 groups. In clinical group I, which was the classification of our patient, the tumor is localized and completely resectable, which implies a good prognosis. Rhabdomyosarcoma is a rare tumor, and a good outcome may result if it is completely resected.

Publisher

FapUNIFESP (SciELO)

Subject

General Medicine

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1. Diagnosis and treatment of rhabdomyosarcomas;Oncology in Clinical Practice;2023-08-23

2. Primary alveolar rhabdomyosarcoma of the diaphragm requiring proximal gastrectomy;Journal of Pediatric Surgery Case Reports;2022-03

3. Etiopathogenesis of oncocytomas;Seminars in Cancer Biology;2017-12

4. Diaphragm reconstruction combined with thoraco-abdominal wall reconstruction after tumor resection;Journal of Plastic Surgery and Hand Surgery;2017-08-31

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