The Combined Therapy of Peripheral Primitive Neuroectodermal Tumor in Childhood / 대한소아혈액종양학회지

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.

Fine Needle Aspiration Cytology of Peripheral Neuroepithelioma of Soft Tissue: Report of A Case / 대한세포병리학회지

Peripheral neuroepithelioma (PNE) of soft tissue is a malignant neuroectodermal tumor arising from peripheral(nonautonomic) nerve. It may occur in both children and adults, and are highly aggressive neoplasms that rapidly give rise to metastatic disease and death. We exprienced a case of peripheral neuroepithelioma of soft tissue in the upper arm in a 18-year-old female. Cytologic features revealed small round cells with scanty cytoplasm occurring both singly and in clusters. The clusters frequently tended to form Homer-Wright rosettes. The cells had a round to oval nucleus with fine chromatin and inconspicuous nucleoli in a hemorrhagic background.