A case of Primary Malignant Fibrous Histiocytoma of the Diaphragm

A malignant fibrous histiocytoma (MFH) is a major subset of soft tissue sarcomas, which occurs principally on the extremities or in the retroperitoneum, as well as on the head and neck of elderly patient. However, it is an extremely rare event when a MFH occurs primarily in the diaphragm of a young people. A 25-year-old woman visited our hospital complaining of right chest pain. The chest X-ray showed a diaphragmatic mass. An exploratory thoracotomic biopsy revealed a primary MFH of the diaphragm. The patient was treated with combined chemotherapy consisting of ifosfamide and doxorubicin. A partial response was seen after 6 cycles of chemotherapy. However, she died of brain metastasis 12 months after the diagnosis.

횡경막에 발생한 악성 섬유종 조직구종 1예

저자들은 우측 흉부 불편감을 주소로 내원한 25세 여자 환자를 개흉 조직 검사를 시행하여 원발성 횡격막 기원 악성 섬유성 조직구종을 진단하였고 ifosfamide, doxorubicin으로 항암 화학 요법 시행 후 부분 관해가 있었으나 진단 후 12개월째 외래 추적 검사 중 뇌전이로 사망한 젊은 여성 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.