다량의 늑막삼출을 동반한 늑막횡문근육종 1예

A Case of Rhabdomyosarcoma Arising at the Pleura

원발성 늑막횡문근육종은 세계적으로 그 예가 드문 희귀한 질환으로, 저자등은 호흡곤란을 주소로 내원한 15세 남자 환아의 늑막조직검사에서 진단되고, 항암화학요법에 좋은 반응을 보인 원발성 늑막횡문근육종 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Although uncommon, rhabdomyosacomas are one of the most frequent forms of cancer of soft parts, particularly in children under the age of 15. There has been only one case of primary rhabdomyosarcoma arising at the pleura, reprted by Hamada, Japan, 1989, in the world. A case of primary rhabdomyosacoma arising at the pleura is reported. This 15 year-old male patient was admitted to the hospital due to a one-month history of dyspnea on exertion and massive right pleural effusion. Pleural biopsy revealed embryonal rhabdomyosarcoma histologically. Immunohistochemical study shows positive reactivity to desmin, vimentin, and cytokeratin. Ultrastructural demonstration of thin and thick myofilaments was most helpful for confirming the histopathological diagnosis. The patient was received 6 cycles of chemotherapy with adriamycin, cyclophosphamide, vincristine and dacarbazine. The chemotherapy response was fairly good that the patient's symptom was absent and pleural effusion and mass size was improved 6 months after chemotherapy. This paper reports the second case of primary rhabdomyosarcoma of the pleura in the world with the review of literature.