KISEP Clinical Article J Korean Neurosurg Soc , 2003 후각신경아세포종 : 임상양상및치료결과 황승균 1 성원진 2 전윤경 3 지제근 3 이철희 2 정희원 1 Olfactory Neuroblastoma:Clinic

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1 KISEP Clinical Article J Korean Neurosurg Soc , 2003 후각신경아세포종 : 임상양상및치료결과 황승균 1 성원진 2 전윤경 3 지제근 3 이철희 2 정희원 1 Olfactory Neuroblastoma:Clinical Features and Treatment Outcome Sung Kyun Hwang, M.D., 1 Weon-Jin Seong, M.D., 2 Yoon-Kyung Jeon, M.D., 3 Je G. Chi, M.D., 3 Chull Hee Lee, M.D., 2 Hee-Won Jung, M.D. 1 Departments of Neurosurgery, 1 Otorhinolaryngology-Head and Neck Surgery, 2 Pathology, 3 Seoul National University College of Medicine, Seoul, Korea Objective:The authors analyzed clinical features, long-term treatment outcome, and prognostic factors of the olfactory neuroblastoma. Methods:Twenty-one cases of olfactory neuroblastomas, treated from 1979 to 2000, were retrospectively reviewed with medical records and radiological findings. Mean follow-up periods are 28.7 months(range 4-178). Extent of tumor was classified by UCLA staging system. Statistical analysis for survival was done using Kaplan Meier method and log-lank test. Results:Mean age was 27 years(13-62), and most common group are second decades(8/21, 38%). Male to female ratio was 13:8. Common symptoms are nasal obstruction, epistaxis, exopthalmos and headache. There were three cases of T1, five T2, six T3, and seven T4 according to UCLA staging system. The 5-year survival rate was 21.3% and average time was 28.9 months in surgical resection group(n=14) as primary modality have higher survival rate than radiation and chemotherapy group(n=7)[2-year survival rate;39.2% vs 14.3%, 5-year survival rate;19.6% vs 14.3%(p=0.0274)]. Early stage(t1, T2) groups showed better survival rate than advanced(t3, T4) groups(38.1% vs 9.1% p=0.0336). The local and regional recurrences were observed in 6(27%) and 2(9%) cases. Mean recurrence free time was 7.8 months(range 1-25). Conclusion:Early detection and extent of resection are the important prognostic factors. Regular follow up is mandatary for the detection of recurrence or metastasis. KEY WORDS:Olfactory neuroblastoma UCLA staging system Radiation therapy Metastasis Chemotherapy Recurrence. 서 론 ReceivedAugust 29, 2001 AcceptedFebruary 22, 2003 Address for reprintshee-won Jung, M.D., Department of Neurosurgery, Seoul National University College of Medicine, 28 Yeongeondong, Jongno-gu, Seoul , Korea Tel , 2358, Fax hwnjung@snu.ac.kr J Korean Neurosurg SocVolume 33May, 2003

2 Table 1. Patients characteristicsn=21, No. Age Sex Grade Stage Treatment Surgical procedure Recurrence Time to recurrence* Treatment of recurrence Final outcome F/U duration* 01 F 17 4 T3 SRTCTx CFR Distant site 19 RT DD M 25 3 T4 CTxRT Neck 01 RT DD F 19 3 T2 SRT CFR NED F 19 Unknown T1 SRT TM NED M 42 1 T1 S MM, EE NED M 19 4 T3 SRT CFR, OE, ND Local 01 DD M 36 Unknown T4 RT DD M 36 2 T4 SRT CFR Local 02 S DD M 42 3 T3 SRT CFR Neck 02 CTx, RT DD F 62 3 T2 S CFR DOC M 13 Unknown T2 SRTCTx CFT, ND NED M 52 4 T3 S TM, OE, ND Local 01 CTx DD M 54 Unknown T4 RTCTx Distant site DD F 14 4 T4 RT DD M 62 1 T4 RT DD F 17 Unknown T4 CTxRT Distant site 04 CTx DD M 23 Unknown T3 RTCTx Local 22 RT, CTx DD M 27 Unknown T1 S Excision Local 25 S DD M 14 1 T2 S MM Local 01 RT DD F 42 2 T2 S CFR NED F 25 Unknown T3 SRT CFR NED 005 SSurgery, RTRadiotherapy, CTxChemotherapy, CFRCraniofacial resection, TMTotal maxillectomy, EEExternal ethmoidectomy, OEOribital excenteration, NDNeck dissection, MMMedial maxillectomy, NEDNo evidence of disease, DDDied of disease, DOCDied of other cause, months 대상및방법 - Table 2. UCLA staging system T1 Tumor involving the nasal cavity and/or paranasal sinusesexcluding the sphenoid, sparing the most superior ethmoidal cells T2 Tumor involving the nasal cavity and/or paranasal sinusesincluding the sphenoid with extension to or erosion of the cribriform plate T3 Tumor extending into the orbit or protruding into the anterior cranial fossa T4 Tumor involving the brain J Korean Neurosurg SocVolume 33May,

Olfactory Neuroblastoma 로 종괴절제술만을 시행하였다. 초기치료로서 수술을 시행한 경 우는 14예였고, 이중에서 6예는 수술만 시행하였고, 6예 에서는 수술 후 방사선치료를 병행하였고, 2예에서는 수술 후 방사선치료와 항화학요법을 병행하였다.

예였고, 재발하였 을 경우 치료받은 환자는 5예였다. 항화학요법을 초기치료 의 일부로써 시행 받은 환자는 6예였고, 재발하였을 경우 치 료받은 환자는 4예 있었다. 3예에서는 방사선치료만 시행하 였고, 2예에서는 방사선치료 후 화학요법을 병행하였고, 2예 Fig. 1.

Sagittal T1 weighted enhanced magnetic resonance image. The tumor eroded the floor of frontal sinus and anterior wall of sphenoid sinuses(right, T3 stage by UCLA staging system).

Hyams classification Lobular architecture Mitotic activity Grade 1 Grade 2 Grade 3 Grade 4 Present Present +/- +/- Absent Present Prominent Marked 결 과 임상 양상 환자들은 1979년 6월부터 2000년 10월까지 총 21예로 Nuclear

3 Olfactory Neuroblastoma 로 종괴절제술만을 시행하였다. 초기치료로서 수술을 시행한 경 우는 14예였고, 이중에서 6예는 수술만 시행하였고, 6예 에서는 수술 후 방사선치료를 병행하였고, 2예에서는 수술 후 방사선치료와 항화학요법을 병행하였다. 방사선치료를 초기 치료의 일부로써 시행 받은 환자는 15예였고, 재발하였 을 경우 치료받은 환자는 5예였다. 항화학요법을 초기치료 의 일부로써 시행 받은 환자는 6예였고, 재발하였을 경우 치 료받은 환자는 4예 있었다. 3예에서는 방사선치료만 시행하 였고, 2예에서는 방사선치료 후 화학요법을 병행하였고, 2예 Fig. 1. Coronal T1 weighted enhanced magnetic resonance image shows a enhancing mass in the left nasal cavity and ethmoid sinus that extends into the anterior cranial fossa(left). Sagittal T1 weighted enhanced magnetic resonance image. The tumor eroded the floor of frontal sinus and anterior wall of sphenoid sinuses(right, T3 stage by UCLA staging system). 에서는 화학요법 후 방사선치료를 시행하였다. 방사선치료의 평균 방사선 조사량은 55.8Gy이었고, 화학요법은 cisplatin based regimen을 사용하였다. 생존분석은 Kaplan-Meier 법과 log-rank 법을 사용하였다. Table 3. Hyams classification Lobular architecture Mitotic activity Grade 1 Grade 2 Grade 3 Grade 4 Present Present +/- +/- Absent Present Prominent Marked 결 과 임상 양상 환자들은 1979년 6월부터 2000년 10월까지 총 21예로 Nuclear Absent Moderate Prominent Marked pleomorphism Rosette H-W +/- H-W +/- Flexner +/- Absent 진단당시 평균연령은 27(13~62)세로 10대가 8예로 가장 Necrosis Absent Absent Occasional Common Grade 1+2 low grade, Grade 3+4 high grade, H-W HomerWright pseudorosette, +/- present or absent (52%), 비출혈(42%), 안구돌출증(19%), 두통(19%), 시 많았다. 남녀 비는 13 8로 남자가 많았다. 증상은 비폐색 력장애(14%), 비루(10%), 안구통증(5%), 안면통증(5%) 순 이었다. 이학적 검사소견으로는 비강내 종물(90%), 안 구 돌출(20%), 그리고 경부종물(15%) 순 이었다. 그리고, 증상발현으로부터 치료시작시점까지의 기간은 평균 5.5(0.5~ 24)개월이었다. 병기 분포 UCLA staging system에 따라 병기를 분류하였다. T1 stage가 3예, T2 stage가 5예, T3 stage가 6예, T4 stage 가 7예로 T1-2 stage를 early stage, T3-4 stage를 adfig. 2. Lobules of the small round cells are separated by fibrovascular stroma(right, 100, H & E). The tumor cells have round hyperchromatic nuclei and indistinct cell borders. Pseudorosette formation(black arrow) around fibrillary material is noted(left, 400, H & E). vanced stage라고 하였을 때, advanced stage의 환자가 많 아 조기에 발견되지 않고, 진행된 상태에서 발견되는 경우 가 많음을 알 수 있다. 생존율 특성에 따라 병합하여 시행하였다. 수술적 치료로 두개안면 절제술을 시행한 경우가 9예였으며, 두개강내 종양은 신경 전체 환자의 2년 생존율은 42.5%였고, 5년 생존율은 21.3% 였다. 그리고, 평균 생존기간은 28.9개월이었다(Fig. 3). 외과의사에 의해 개두술을 통한 경개두개접근법으로 절제하 였고, 비강내의 종양은 이비인후과의사에 의해 경안면접근 재발 및 전이 법으로 절제되었다. 상악전적출술(total maxillectomy)과 내 국소 재발은 6예(29%)에서 관찰되었다. 경부전이는 5예 측 상악절제술(medial maxillectomy)을 시행한 경우가 각 (24%)에서 관찰되었는데, 3예는 진단당시에 경부전이가 있 각 2예였고, 1예는 경구내 접근법(transpalatal approach)으 었고, 2예는 추적관찰 기간 중에 발견되었다. 원격전이는 J Korean Neurosurg Soc/ Volume 33/ May, 2003

4 % Mean survival time28.9 months Years0 Fig. 3. Kaplan-Meier survival curve. 예후인자 (Prognostic factor) Survival rate Survival vs Stage 1.0 Early stage Advanced stage p= Months Fig. 4. Survival vs tumor stage. Survival rate Table 4. Prognostic factors 합병증 Survival vs Primary therapy Months Factors Radiation or chemotherapy Radical surgical resection Fig. 5. Survival vs primary treatment modality. p=0.027 p-value Stage of tumor Radical surgical resection as primary treatment modality Age Sex Pathological grading system J Korean Neurosurg SocVolume 33May,

5 고찰 병리조직학적소견 - 임상양상 450 병기분류 System 치료결과 J Korean Neurosurg SocVolume 33May, 2003

6 - 두개안면절제술의합병증 생존율과재발및전이 J Korean Neurosurg SocVolume 33May,

7 예후인자 452 결 론 Acknowledgement References 1. Berger L, Luc R:L esthesionneuroepitheliome olfactif. Bull Assoc Fr Etude Cancer 13: , Biller HF, Lawson W, Sachdev VP, Som P:Esthesioneuroblastoma: surgical treatment without radiation. Laryngoscope 100: , Bilsky MH, Kraus DH, Strong EW, Harrison LB, Gutin PH, Shah JP:Extended anterior craniofacial resection for intracra-nial extension of malignant tumors. Am J Surg 174: , Broich G, Pagliari A, Ottaviani F:Esthesioneuroblastoma:A general review of the cases published since the discovery of the tumor in Anticancer Res 17: , Bumpous J, Janecka IP:Transorbital approaches to the cranial base. Clin Plast Surg 22: , Canty P:Olfactory neuroblastoma:long-term survival. J Laryngol Otol 93: , Catalano PJ, Hecht CS, Biller HF, Lawson W, Post KD, Sachdev V, et al:craniofacial resection. An analysis of 73 cases. Arch Otolaryngol Head Neck Surg 120: , Chapman P, Carter RL, Clifford P:The diagnosis and surgical management of olfactory neuroblastoma:the role of craniofacial resection. J Laryngol Otol 95: , Dulguerov P, Calcaterra T:Esthesioneuroblastoma:The UCLA Experience Laryngoscope 102: , Eden BV, Debo RF, Larner JM, Kelly MD, Levine PA, Stewart FM:Esthesioneuroblastoma Long-term outcome and patterns of failure--the University of Virginia experience. Cancer 73: , Eriksen JG, Bastholt L, Krogdahl AS, Hansen O, Joergensen KE: Esthesioneuroblastoma--what is the optimal treatment? Acta Oncol 39: , 2000 J Korean Neurosurg SocVolume 33May, 2003

8 12. Irish J, Dasgupta R, Freeman J, Gullane P, Gentili F, Brown D, et al:outcome and analysis of the surgical management of esthesioneuroblastoma. J Otolaryngol 26:1-7, Jackson IT, Somers P, Marsh WR:Esthesioneuroblastoma:treatment of skull-base recurrence. Plast Reconstr Surg 76: , Jekunen AP, Kairemo KJ, Lehtonen HP, Kajanti MJ:Treatment of olfactory neuroblastoma. A report of 11 cases. Am J Clin Oncol 19: , Kadish S, Goodman M, Wang CC:Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 37: , Kasler M, Banhidy FG, Trizna Z:Experience with the modified pectoralis major myocutaneous flap. Arch Otolaryngol Head Neck Surg 118: , Koka VN, Julier M, Bourhis J:Aesthesioneuroblastoma. J Laryngol Otol 112: , Levine PA, Debo RF, Meredith SD, Jane JA, Constable WC, Cantrell RW:Craniofacial resection at the University of Virginia( ):survival analysis. Head Neck 16: , Levine PA, Gallagher R, Cantrell RW:Esthesioneuroblastoma:reflections of a 21-year experience. Laryngoscope 109: , Lund VJ, Howard DJ, Wei WI, Cheesman AD:Craniofacial resection for tumors of the nasal cavity and paranasal sinuses--a 17-year experience. Head Neck 20:97-105, McCaffrey TV, Olsen KD, Yohanan JM, Lewis JE, Ebersold MJ, Piepgras DG:Factors affecting survival of patients with tumors of the anterior skull base. Laryngoscope 104: , McCutcheon IE, Blacklock JB, Weber RS, DeMonte F, Moser RP, Byers M, et al:anterior transcranial(craniofacial) resection of tumors of the paranasal sinuses:surgical technique and results. Neurosurgery 38:471-80, Meneses MS, Thurel C, Mikol J, Ramina R, Maniglia JJ, Arruda WO, et al:esthesioneuroblastoma with intracranial extension. Neurosurgery 27: , Miyamoto RC, Gleich LL, Biddinger PW, Gluckman JL:Esthesioneuroblastoma and sinonasal undifferentiated carcinoma:impact of histological grading and clinical staging on survival and prognosis. Laryngoscope 110: , Morita A, Ebersold MJ, Olsen KD, Foote RL, Lewis JE, Quast LM: Esthesioneuroblastoma:prognosis and management. Neurosurgery 32: , O Connor TA, McLean P, Juillard GJ, Parker RG:Olfactory neuroblastoma. Cancer 63: , Polin RS, Sheehan JP, Chenelle AG:The role of preoperative adjuvant treatment in the management of esthesioneuroblastoma:the University of Virginia experience. Neurosurgery 42: , Raveh J, Turk JB, Ladrach K, Seiler R, Godoy N, Chen J, et al: Extended anterior subcranial approach for skull base tumors:longterm results. J Neurosurg 82: , Shah JP, Kraus DH, Bilsky MH, Gutin PH, Harrison LH, Strong EW:Craniofacial resection for malignant tumors involving the anterior skull base. Arch Otolaryngol Head Neck Surg 123: , Shall LA, Lineback M:Primary intranasal neuroblastoma. Ann Otolaryngol 60: , Slevin NJ, Irwin CJ, Banerjee SS, Gupta NK, Farrington WT:Olfactory neural tumours--the role of external beam radiotherapy. J Laryngol Otol 110: , Som PM, Lidov M, Brandwein M, Catalano P, Biller HF:Sinonasal esthesioneuroblastoma with intracranial extension:marginal tumor cysts as a diagnostic MR finding. AJNR Am J Neuroradiol 15: , Vanaclocha V, Saiz-Sapena N:Surgical treatment of anterior skull base tumors. Acta Neurochir(Wien) 139: , 1997 J Korean Neurosurg SocVolume 33May,

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( )Jkstro011.hwp 비인강암의방사선치료결과및생존율에관한예후인자분석 2005 2 1 2005 3 28. :, Tel: 053)250-7665, Fax: 053)250-7984 E-mail: jhkim@dsmc.or.kr 정영연외 2 인 : 비인강암의예후인자분석 정영연외 2 인 : 비인강암의예후인자분석 Carcinoma of the nasopharynx treated by radiotherapy