A clinical study of childhood soft tissue sarcoma

Jung, Hye Lim; Koo, Hong Heo; Shin, Hee Young; Ahn, Hyo Seop

Abstract

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 0-4 year 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 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 hemangiopericytoma (3.0%), and there were 1 case each of angiosarcoma, leiomyosarcoma, synovial sarcoma, malignant mesenchymoma and mesenchymal chondrosarcoma. The median age of 45 rhabdomyosarcoma patients was 3 years 8 months and age distribution showed that 0-4 year age group was most common (64.5%). Twenty three patients were male and 22 were female. The histologic 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 tissue sarcoma patients was mass (68.7%). As for Intergroup Rhabdomyosarcoma Study clinical grouping system of 67 soft tissue sarcoma patients, clinical group III (58.2%) was most common, followed by clinical group II(20.9%), IV (14.9%) and I (6.0%). Of 10 cases of clinical group IV 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 group III was most common (68.9%). Of 6 cases of clinical group IV, 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 group III, IV and II with 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 group II, 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 all 54 cases was 54.1%, 3 year DFS of clinical group I and II was 83.9%,3 year DFS of clinical group III and IV before 1986 was 35.7% and after 1986 was 48.2%. Of 45 cases of rhabdomyosarcoma, 41 cases were eligible for treatment analysis. The 3 year DFS of all 41 cases was 49.1%,3 year DFS of clinical group I and II was 87.5%,3 year DFS of clinical group III and IV before 1986 was 27.2% and after 1986 was 45.0%. Patients in clinical group I and II who had no gross residual tumor after primary surgical excision had best prognosis with 3 year DFS approximating 90% with only 2 drugs regimen, significantly better than patients in clinical group III and IV with 3 year 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, 3 year DFS of clinical group III 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 of biopsy and patients with distant metastasis at diagnosis, intensified multiagent chemcherapeutic regimen with adequate dose modification should be done to lower early toxic deaths.