The Combined Therapy of Peripheral Primitive Neuroectodermal Tumor in Childhood.
- Author:
Pil Sang JANG
1
;
Jun Ah LEE
;
Yeong CHOI
;
Hyo Jeong HAN
;
Hyoung Soo CHOI
;
Hee Young SHIN
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Peripheral primitive neuroectodermal tumor;
Peripheral neuroepithelioma;
Combination chemotherapy;
Survival rate;
Childhood
- MeSH:
Diagnosis;
Drug Therapy;
Drug Therapy, Combination;
Extremities;
Head;
Humans;
Neck;
Neuroectodermal Tumors, Primitive*;
Retrospective Studies;
Seoul;
Survival Rate;
Thorax
- From:Korean Journal of Pediatric Hematology-Oncology
1998;5(2):304-313
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: A review of the literature for patients with peripheral primitive neuroectodermal tumor (PPNET; peripheral neuroepithelioma) suggests that the outcome is usually poor. We performed a retrospective study to emphasize the need for triple combined therapy of PPNET. METHODS: The clinical data of 10 patients (M:F=1:1, aged 1 8/12~14 3/12 years) with PPNET seen at Seoul National University Children's Hospital from Jan. 1991 to Jun. 1998 were reviewed. RESULTS: The primary sites were head and neck, chest, limbs, and retroperitoneal area in order of frequency. A palpable mass was the major symptom. The median duration of symptoms before diagnosis was 3 months. Metastatic disease at diagnosis was found in 3 patients, 2 of them were multiple. All of the patients had combined modality treatment with surgery, chemotherapy and radiation therapy. The 3-year relapse-free survival rate was 64%, and the overall 5-year survival rate was 66%. CONCLUSION: Best results were obtained with combined therapy with combination chemotherapy, extensive surgery and local radiation therapy.
