横紋筋肉腫の治療と妊娠・出産

Abstract

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, is highly malignant tumor arising in striated muscle or in embryonal mesenchymal cells. They represent the most frequent soft-tissue sarcoma in children and young adults, accounting for 4% of malingnant tumors in children under 15 years of age. Embryonal rhabdomyosarcoma is the predopminate form (46%) in children and young adults, arising primarily in the head and neck, retroperitoneum, and genito-urinary tract. Metastases frequently occur to lymph nodes, lung, and bone. Prognosis is grave. Even with aggressive surgery, radiotherapy, and chemotherapy, only 10% to 15% of these children survive for 5 years. Treatment approaches to RMS incorporate surgery, radiation therapy, and chemotherapy. Surgery and radiation therapy is used to control local microscopic or gross residual disease, whereas systemic chemotherapy plays a role in primary cytoreduction as well as eradication of gross and micrometastatic disease. VAC regimen (vincristine, Actinomycin D, cyclophosphamide) has been the gold standard for combination chemotherapy in the treatment of most cases of RMS. Sixty percent to 70% of newly diagnosed patients with nonmetastatic disease can be cured with combined modality therapy. Complications of therapy are becoming more apparent. Because of the increasing frequency of long-term survivors, attention has recently focused on the effects of chemotherapy on ovarian function and reproductive potential. Early reports on the effects of chemotherapy on ovarian function seemed to emphasize the potentially harmful consequences, including amenorrhea and infertility. More recent studies of the effects of chemotherapy on female patients with Hodgkin's disease and leukemias have included several patients with subsequent normal ovarian function and successful pregnancies.