• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.92-95
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• 2016

Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid mass in the right nasal cavity was detected through sinuscopy. Biopsy of the right nasal polyp was performed. Based on the result, the patient was diagnosed with a high-grade esthesioneuroblastoma. Nuclear imaging revealed increased uptake in both the right posterior nasal cavity and the right cervical IB-II space, suggesting metastatic lymph nodes. Cytogenetic analysis revealed a 47, XYY karyotype. Twelve courses of concurrent chemotherapy were administered. Three years after the completion of chemotherapy, the patient had had no disease recurrence. He manifested behavioral violence and temper tantrums, so we started methylphenidate for correction of the behavior.

• 대한두경부종양학회지
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• 제16권2호
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• pp.228-234
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• 2000

Objectives: Esthesioneuroblastoma is a rare malignant neoplasm that originates from the olfactory sensory cells. This tumor grows from the upper nasal cavity and ethmoid sinus and invades surrounding structures through the cribriform plate into intracranium or orbit in advanced stage. Even though there has been some controversies in determining standard treatment due to rarity of this tumor, the combination treatment of surgery and adjuvant radiation has been recommended for the locally advanced esthesioneuroblastomas. However, the recent clinical experiences of advanced cases showed that combination chemotherapy is highly effective to reduce tumor mass and improve clinical outcomes. Materials and Methods: The authors conducted a retrospective analysis of 6 esthesioneuroblastoma patients who were treated in our hospital from 1986. Results: The age of these patients was between 19 and 86 year-old. Among the 6 cases, 2 were diagnosed at stage B and 4 at stage C, according to Kadish classification. Anti-tumor treatments were performed in 5 patients. One patient refused active treatment and was lost to follow-up. Better survival outcome were observed in 3 patients who were treated with combination chemotherapy alone or combined modality treatment including chemotherapy. Conclusion: Based on our retrospective study, the combined treatment consisting of surgery, radiotherapy, and combination chemotherapy should be used to improve treatment results. And furthermore, innovative clinical approaches such as neoadjuvant chemotherapy, high-dose chemotherapy and autologous peripheral stem cell transplantation, which have been reported to have good therapeutic results, should be considered and applied actively.

• Radiation Oncology Journal
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• 제27권4호
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• pp.189-193
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• 2009

목 적: 감각신경모세포종의 시대에 따른 치료방침과 생존율의 변화를 보고하고자 하였다. 대상 및 방법: 1989년 3월부터 2007년 6월 사이에 감각신경모세포종으로 진단을 받고 처음으로 치료를 받은 42명의 환자를 후향적으로 분석하였다. Kadish 병기에 따르면, 3명(7%)의 환자가 A병기, 6명(14%)의 환자가 B병기, 33명(79%)의 환자가 C병기였다. 33명의 C병기 환자 중, 19명은 1989년부터 2000년에, 14명은 2001년부터 2007년까지 치료 받았다. 치료는 수술, 방사선치료, 항앙화학요법 및 이들의 조합이었다. 1989년부터 2000년까지 치료를 받은 19명 중 8명(42%)이 항암화학요법을 받은 것에 비해, 2001년부터 2007년까지 치료를 받은 환자 14명은 모두 항암화학요법을 받았다(p<0.001). 삼차원입체조형방사선치료는 1989년부터 2000년에는 아무도 받지 않았으나 2001년부터 2007년도에 치료를 받은 14명 중 8명이 받았다(p<0.001). 생존환자에 대한 중앙 추적기간은 6.5년(범위, 2.2~15.8년)이었다. 결 과: 전체 환자에 대한 5년 생존율과 무진행생존율은 각각 53%와 39%였다. Kadish A혹은 B 환자의 5년 생존율은 100%이고 Kadish C병기의 5년 생존율은 39%였다(p=0.007). C병기 환자 중 1989년부터 2000년까지 치료받은 환자와 2001년부터 2007년까지 치료받은 환자의 5년 생존율은 각각 26%와 59%였다(p=0.029). 상응하는 5년 무진행생존율은 각각 16%와 46%였다(p=0.001). 다변량분석에서 안와내침범과 치료시대(1989~2000 vs. 2001~2007)가 생존율과 무진행생존율에 영향을 주는 독립인자로 확인되었다. 결 론: 본 연구의 결과 C병기 환자에서 삼차원입체조형방사선치료의 도입과 같은 치료방법과 기술의 진화를 반영하는 치료시기가 향상된 생존율과 무진행생존율의 원인이 될 수도 있음을 제시 하였다. C병기 환자에서 더 나은 결과를 얻기 위해서는 수술에 더하여 항암화학요법과 방사선치료 병용요법, 특히 삼차원입체조형방사선치료가 추천된다.

• Imaging Science in Dentistry
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• 제30권4호
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• pp.287-292
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• 2000

A case of dento-maxillofacial abnormality involving a 10-year-old male patient with a history of esthesioneuro-blastoma is presented. This patient had been treated with 54 Gy /sup 60/Co-gamma-radiation to the nasal cavity for 6 weeks and 6 cycles of combination chemotherapy of Cyclophosphamide, Cisplatin, Adriamycin, VM-26 (Tenipo-side), and DTIC (Dacarbazine) when he was 16 months of age. Five years after cessation of cancer therapy, he was disease free and transferred for extensive dental care to Kyung Hee University Dental Hospital. A clinical and radiologic follow-up over last 4 years showed root stunting, premature closure of the root apices, microdontia, developmental arrest, small crowns, and partial anodontia. Maxillofacial morphology evaluated by cephalometric analysis showed deficiency of maxillary development.