Korean J Pediatr Hematol Oncol.  1998 Oct;5(2):304-313.

The Combined Therapy of Peripheral Primitive Neuroectodermal Tumor in Childhood

• Jang PS
• Lee JA
• Choi Y
• Han HJ
• Choi HS
• Shin HY
• Ahn HS

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.

Keyword

Peripheral primitive neuroectodermal tumor; Peripheral neuroepithelioma; Combination chemotherapy; Survival rate; Childhood

MeSH Terms

Diagnosis
Drug Therapy
Drug Therapy, Combination
Extremities
Head
Humans
Neck
Neuroectodermal Tumors, Primitive*
Retrospective Studies
Seoul
Survival Rate
Thorax