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A Clinical Study of Childhood Soft Tissue Sarcoma

Journal of the Korean Pediatric Society 1993;36(9):1258-1270.
Published online September 15, 1993.
A Clinical Study of Childhood Soft Tissue Sarcoma
Hye Lim Jung1, Hong Heo Koo2, Hee Young Shin3, Hyo Seop Ahn3
1Department of Pediatrics, Koryo General Hospital, Seoul, Korea
2Department of Pediatrics, Ulsan University College of Medicine, Seoul, Korea
3Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
소아 연부보직육종에 관한 연구
정혜림1, 구홍회2, 신희영3, 안효섭3
1고려병원 소아과
2울산대학교 의과대학 소아과학교실
3서울대학교 의과대학 소아과학교실
Abstract
Medicine, Seoul, Korea Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea To study the clinical characteristics and treatment results of childhood soft tissue sarcoma, the retrospective study was performed on 67 patients with soft tissue sarcoma, experienced at the Department of Pediatrics, Seoul National University Hospital from January, 1982 to July, 1990. The median age of 67 soft tissue sarcoma patients was 4 years 5 months and age distribution showed that 0-4year age group was most common(55.2%). The sex ratio of male to female was 1.2:1. There were 3 cancers among relatives of soft tissue sarcoma patients, including one cancer among relatives of soft tissue sarcoma patients,including one cancer among first-degree relatives. As for pathological classification, rhabdomyosarcoma(67.1%) was the most common childhood soft tissue sarcoma, followed by malignant Schwannoma(8.9%), extraskeletal Ewing's sarcoma(6.0%), infantile fibrosarcoma (4.5%), malignant fibrous histiocytoma(3.0%), malignant hemangiopericytome (3.0%), and there were 1 case each of angiosarcoma, leiomyosarcoma, synovial sarcoma, malignant mesenchymoma and mesenchymal chondrosarcoma. The median ageof 45 rhabdomyosarcoma patients was 3 years 8 months and age distribution showed that 0-4year age group was most common(64.5%). Twenty thre patients were male and 22 were female. The histolgic subtype of rhabdomyosarcoma was embryonal type in 38 patients(84.5%), alveolar type in 5 patients(11.1%) and unclassified type in 2 patients(4.4%). As for primary site of soft tissue sarcomas, the most frequent site was the head and neck region (32.8%) including parameningeal region(13.4%) and orbit(6.0%), followed by extremities(20.9%), trunk (19.4%), retroperitoneum and pelvis(11.9%), urogenital region(7.5%), perineum and perianal region(4.5%) and other region(3.0%). As for primary site of 45 rhabdomyosarcoma cases, the most frequent site was also the head and neck region(37.8%). The most common initial symptom of soft tisue sarcoma patients was mass(68.7%). As for Intergroup Rhabdomyosarcoma Study clinical grouping system of 67 soft tissue asrcoma patients, clinical groupⅢ(58.2%) was most common, followed by clnical groupⅡ(20.9%), Ⅳ(14.9%) and Ⅰ(6.0%). Of 10 cases of clinical groupⅣ with distant metastasis, lung(8 cases) was the most common metastaic region and other metastatic regions were bone, kidney, liver and bone marrow. As for IRS clinical grouping system of 45 rhabdomyosarcoma patients, clinical groupⅢ was most common(68.9%). Of 6 cases of clinical groupⅣ, lung(5 cases) was also the most common metastatic region, followed by kidney and liver. From 1982 to 1985, chemotherapy was done with pulse VAC or pulse VAdrC-VAC regimen based on IRS-Ⅰ and IRS-Ⅱ. From 1986, patients in clinical group Ⅰ and Ⅱ received vincristine and actinomycin-D for 1 year and patients in clinical groupⅢ, Ⅳ and Ⅱwith alveolar histologic subtype (unfavorable histologic group) received vincristine, actinomycin-d, adriamycin, cyclophosphamide and cisplatinum based on IRS-Ⅲ. Radiation therapy was administered to patients in clinical groupⅡ, Ⅲ and Ⅳ. Of 67 cases of soft tissue sarcoma, 54 case were eligible for treatment analysis. The 3 year disease free survival(DFS)of an 54 cases was 54.1%, 3year DFS of clinical groupⅠ and Ⅱ was 83.9%, 3year DFS of clinical groupⅢ and Ⅳ before 1986 was 35.7% and after 1986 was 48.2%. Of 45cases of rhabdomyosarcoma, 41cases were eligible for treatment analysis. The 3year DFS of all 41cases was 49.1%, 3year DFS of clinical groupⅠ and Ⅱ was 87.5%, 3year DFS of clinical groupⅢ and Ⅳ befor 1986 was 27.2% and after 1986 was 45.0%. Patients in clinical groupⅠ and Ⅱ who had no gross residual tumor after primary sugical excision had best prognosis with 3year DFS approximating 90% with only 2drugs regimen, significantly better than patients in clinical groupⅢ andⅣ with 3year DFS below 50% even after intensifying chemotherapy since year 1986. This analysis suggests that total surgical removal is very important for improving prognosis and should be undertaken where possible in all patients without distant metastasis. Treatment results also showed that after year 1986 with intensification of chemotherapy, 3year DFS of clinical groupⅢ and Ⅳ as well as early toxic deaths increased, and after lowering doses of chemotherapeutic agents of regimen 35 of IRS-Ⅲ, treatment results improved much. Therfore to improve prognosis of patients with gross residual tumor after surgical excision or biopsy and patients with distant metastasis at diagnosis, intensified multiagent chemc herapeutic regimen with adequate dose modification should be done to lower early toxic deaths.
Key Words: Soft tissue sarcoma, Rhabdomyosarcoma, Childhood, Chemotherapy, Survival rate


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