All issues > Volume 34(4); 1991

Original Article

J Korean Pediatr Soc. 1991;34(4):525-531. Published online April 30, 1991.

Malignant Schwannomas in children.

Joon Jai Kim¹, Dong Joo Shin¹, Dong Won Son¹, Hong Hoe Koo¹, In Sang Jeon¹, Hee Young Shin¹, Hyo Seop Ahn¹

¹Department of Pediatrics, Seoul National University Colllege of Medicine, Seoul, Korea

Received: July 10, 1990;  Accepted: November 6, 1990.

Abstract

Six pediatric patients who were diagnosed as malignant Schwannoma and treated at Seoul National University Children’s Hospital from Aug. 1981 to Jun. 1990 were reviewed. Mean age was 11 years and 1 month and all cases except one were over 10 years old. Of six cases, three (50%) were associated with von Recklinghausen’s disease. Overall male to female ratio was 1 : 2. In case of primary malignant Schwannomas, two cases were male and one case was female, but the other three cases who were associated with von Recklinghausen’s disease were all female. The main presenting symptom was mass (67%) with or without pain. Primary sites of tumors were head and neck (2 cases), retroperitoneum (2 cases), pelvic cavity (1 case) and left thigh (1 case). At the time of diagnosis, pulmonary metastasis was found in one case and another case had regional lymph node metastasis. All cases received tumorectomy first and, in four cases (67%), it was followed by chemotherapy or radiotherapy or both. Relapse occurred in four cases; three cases had local recur- rence and one case had both local recurrence and bone metastasis. The mean duration from the first operation to relapse was 11.5 months. Two cases were expired because of the progression of the disease and their survival time after diagnosis was 4 years and 2 months, and 1 year and 2 months, respectively. The number of recurred cases in the following situations was as follows: incomplete tumor excision or regional lymph node metastasis at the time of diagnosis; all of three cases, complete tumor excision without regional lymph node metastasis (excluding case 3); one of two cases, postoper- ative combined chemotherapy and radiotherapy; one of two cases, operation alone or operation and chemotherapy or operation and chemotherapy followed by the second operation and radiotherapy (excluding case 3); all of three cases. These results suggest radical tumor excision and postoperative combined chemotherapy and radiotherapy seem to improve survival. But, considering small number of cases and short follow-up period in this study, the more extended study should be performed before definite conclusion.

Keywords :Maligmant Schwamoma;Von Recklinghausen's disease;Childhood