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1 Chin Dent J( 中華牙誌 )25(4): December 2006 下顎骨緻密纖維瘤 - 病例報告陳玟秀 1,2 郭英雄 3,4 劉步遠 3,4 江俊斌 2,4 李正 3, 中山醫學大學牙醫學系國立台灣大學臨床牙醫學研究所國立台灣大學牙醫學系國立台大醫院牙科部緻密纖維瘤 (desmoplastic fibroma) 是一種罕見的良性骨頭腫瘤, 在組織學和生物行為上和軟組織的硬纖維瘤很相像, 多半發生在小於 30 歲的年輕人, 男女比例均等, 好犯下顎骨或身體其它的長骨它在臨床上的表現是局部侵犯性浸潤性和破壞性, 多半會侵入周圍的組織, 但不會轉移顎骨緻密纖維瘤由於較為少見, 目前對其治療方式仍有一些爭議本文將提出一例發生在下顎骨的緻密纖維瘤病例報告, 藉由其症狀治療方式及術後追蹤結果的呈現, 提供臨床醫師爾後面對相同病例時的參考關鍵詞 : 緻密纖維瘤, 顎骨, 刮除術緻密纖維瘤 (desmoplastic fibroma), 是一種罕見, 具局部侵犯性的腫瘤, 佔所有骨頭腫瘤的比例不到 1% 1,2 世界衛生組織對緻密纖維瘤所下的定義為良性腫瘤, 組織學上包含低到多變的細胞性, 腫瘤細胞可能是卵圓形或長形, 有一致的細胞核, 缺乏非典型多型性和有絲分裂的能力, 腫瘤細胞周 3,4 圍基質由膠原性透明的纖維結締組織所構成 1958 年,Jaffe 最早提到這種病變會出現在許多 5 部位, 像是脛骨肩胛和股骨而發生在顎骨的例 6 子, 最早是在 1965 年由 Griffith 和 Irby 所提出臨床上顎骨緻密纖維瘤的症狀有很多, 起初可能出現張口受限及無痛性腫脹 X 光片影像是多變的, 可能為多房性或單房性表現, 界線可能清楚或不清楚, 偶爾出現透射性或不透射性或兩者混合的影像皮 7~29 質骨可能穿通膨大或變薄, 若皮質骨穿通, 就很難分辦出病變是緻密纖維瘤 (desmoplastic fibroma) 往軟組織侵犯或是纖維瘤病 (fibromatosis) 往骨頭延伸所造成由於顎骨緻密纖維瘤在臨床及 X 光的表現上較不具特異性, 因此正確的診斷仍需靠病理組織學的檢查在治療上, 緻密纖維瘤因具局部侵犯性, 局部復發率高, 因此治療方式以手術切除為主但應採取較保守式的刮除術 (curettage) 或較積極性的顎骨片段切除術 (segmental resection), 由於提出的病例 30 報告仍不多, 目前仍存在著許多爭論因此, 本文將提出一例發生在幼童之顎骨緻密纖維瘤病例報告, 希望藉由其病程演變及治療結果的呈現, 提供醫師臨床面對相同病例時的參考病例九歲女孩, 因其母親發現下顎骨左側有一個約存在二個月的腫塊, 而來本科就診女孩先前並無創傷的病史, 她母親描述這個腫塊起初生長緩慢, 但最近一個月生長加速臨床檢查發現, 在下顎左側骨體近舌側區有一腫塊約 5 3 公分, 表面的皮膚顏色正常, 質地堅硬, 邊界清楚, 無活動性, 觸壓時不會疼痛 ( 圖 1, -C) 此外, 患者沒有張口困難的情形, 且可以正常的飲食說話和吞嚥環口 X 光和下顎咬合片的檢查, 發現在下顎骨左側骨體的下緣及舌側處, 均有反應性增生的影像 ( 圖 2,, ) 頭部電腦斷層掃描則可發現在下顎骨體 Chin Dent J 2006 Vol 25 No 4 281

2 W.H. Chen, Y.S. Kuo,.Y. Liu, et al. C 圖 1. 患者口外觀可見下顎骨左側骨體區有一約 4x3 平方公分大小的腫塊口內觀則無明顯腫大區域 ( 本照片為切片後手術前拍攝 ) Extraoral examination showing a 4 3-cm mass in the left mandibular body. The intraoral examination showed there to be no obviously swelling. 圖 2. 下顎骨左側骨體下緣及舌側緣有反應性增生影像 Panoramic radiograph displaying a periosteal reaction at the inferior border and lingual side of the left mandibular body. 左舌側骨板的地方, 呈現不規則骨表面, 在下顎骨體接近下顎骨角的地方, 有一密度較低的影像 ( 圖 3) 其後, 患者在全身麻醉下進行切片手術, 病理報告為纖維組織增生性病變 (fibrous proliferative lesion), 但仍無法完全排除為惡性腫瘤的可能性, 因此安排患者再次入院, 進行全病變切除手術手術時先標示出下顎骨下緣及腫塊的範圍, 沿著之前切片手術所留下的疤痕作切開線, 翻開皮瓣在二腹肌前腹下, 可見一軟組織腫瘤, 與顎下腺沾黏小心的將腫瘤剝離, 發現腫瘤往下顎骨的舌側面沿伸將腫瘤與骨面完全剝離, 並同時移除顎下腺後, 可見下顎骨下緣有骨頭破壞的情形, 呈 282 圖 3. 電腦斷層掃描可見下顎骨左側骨體舌側骨板的地方呈現不規則表面 Computed tomography displaying an irregular surface at the lingual plate of the left mandibular body. Chin Dent J 2006 Vol 25 No 4

3 Desmoplastic fibroma of the mandible C D 圖 4. 手術過程與發現 Operative procedure and findings. 不規則表面, 將骨頭表面作刮除術, 最後將傷口縫合, 並放置一負壓引流管 ( 圖 4, -D) 手術標本約立方公分, 顏色呈暗紅色, 包含了腫瘤本身顎下腺及一些淋巴結, 另外下顎骨表面骨刮除術標本也一併送檢腫瘤質地具有彈性 ( 圖 5), 組織病理檢查下, 可見腫瘤本身無被膜 ( 圖 6), 周圍骨頭被破壞及肌肉變性 ( 圖 6) 腫瘤組織內顯示有大量的纖維母細胞增生, 交錯在大量不同方向的束狀膠原纖維當中, 大部分為低度細胞性 ( 圖 6C) 這些細胞無多形性外觀(pleomor- phism), 也無有絲分裂外觀 (mitoses) 或非典型細胞的出現 (cellular atypia) ( 圖 6D) 腫瘤顎下腺和一併送檢之骨碎片組織均無惡性變化表現最後病理診斷確定為下顎骨緻密纖維瘤病人術後恢復狀況良好, 無腫瘤復發跡象術後一年環口 X 光片追蹤, 原骨缺損處已見骨再生現象, 現仍持續於門診追蹤中 ( 圖 7-C) 圖 5. 腫瘤大小約立方公分 The approximately cm tumor. Chin Dent J 2006 Vol 25 No 4 283

4 W.H. Chen, Y.S. Kuo,.Y. Liu, et al. C D 圖 6. 組織病理檢查發現 Histopathologic features. 討論回顧過去的文獻緻密纖維瘤發生年齡廣但多侵犯青少年或年輕人年齡從12個月到46歲均有可能但84%的病人年齡小於30歲無性別上的明顯差異在發生部位上多位於長骨頭頸部則以 2,3.31, %的病例發生在後牙區本報下顎枝最多告提出的病例為發生在下顎骨後牙區的緻密纖維瘤患者年紀僅9歲這與過去報告之流行病學特徵類似此外過去提出發生在顎骨之緻密纖維瘤病例臨床上症狀多是無痛性腫脹少部份會有感到疼痛張口困難咬合異常牙齒動搖感覺異 32~34 常感染牙齒移位流血等症狀本病例臨床上的表現僅為無痛性的腫脹並不具特異性這也與過去報告之病例相似緻密纖維瘤病因目前未知可能與損傷內分 ,36 泌和基因有關大部分的報告認為緻密纖維瘤是 13,36~40 有軟組織纖維瘤病(fibromatosis)的骨相似物圖 7. 術後一年追蹤無腫瘤復發跡象缺損骨處有再生現象 Spontaneous regeneration of the bony defect noted after the patient had been followed-up in our clinic 1 year without tumor recurrence. Chin Dent J 2006 Vol 25 No 4

5 Desmoplastic fibroma of the mandible 些甚至將它歸類為骨之非硬化性纖維瘤的變異 (variant of nonossifying fibroma of bone) 41, 認為它生物學上的表現介於良性纖維病灶 (fibrous lesion) 和 42 惡性纖維肉瘤 (fibrosarcoma) 之間本病例發病之前並無創傷的病史, 無其它全身性的疾病, 亦無家族史, 確實的病因仍有待查證單靠臨床上的資訊診斷顎骨緻密纖維瘤, 仍有其困難度顎骨緻密纖維瘤鑑別診斷須與造釉細胞瘤齒源性黏液瘤動脈瘤性骨囊腫類軟骨黏液性纖維瘤骨內血管瘤嗜伊紅性肉芽腫等骨內腫瘤作區分核磁共振影像對鑑別診斷的貢獻不大, 但它可以區分骨內腫瘤和骨髓, 有助於手術計劃另外, 電腦斷層掃描則可看出皮質骨是否有穿通如同本病例 X 光之表現, 緻密纖維瘤由於臨床上生長快速, 具骨頭破壞性, 其 X 光影像呈日光狀 (sun-ray appearance) 表現 ; 再加以組織學上充滿梭狀細胞的特徵, 和低度纖維肉瘤 (low-grade fibrosarcoma) 很像, 因此容易被誤診成惡性病灶, 一個重要區分方法是緻密纖維瘤不會含有骨樣細胞 (osteoid cell) 因此建議切片檢查時, 須取靠近病灶中心的地方, 而非表面, 以免無法區分良性纖維病變和惡性骨肉瘤, 本病例手術前的切片, 由於腫瘤解剖位置的關係, 僅能取得下緣周邊之組織, 因此僅得到纖維組織增生性病變的診斷, 而不能完全排除有惡性病變的可能性顎骨緻密纖維瘤的治療方法包括刮除局部切除或廣泛性切除都曾被提出, 目前仍存在許多的爭 5 Jaffe 認為以片段切除術最佳 ; 反之,Freedman 30 論 15 等長期觀察後, 建議作刮除術最適合 Peede 及 Epker 43 則強調, 手術切除時需同時作冷凍切片, 以 25 確定邊緣是否為正常組織 Iwai 等的報告則指出, 如果採取廣泛切除則不會有復發的可能 ; 若採取局部切除則有 20~40% 的復發率 ; 如果採取刮除術則有高達 70% 的復發率然而, 有些報告卻指出緻密纖維瘤的高復發率不完全和手術方式有關, 而是它本身的生物特性使然, 甚至更進一步報告指出高 44,45 細胞性的腫瘤組織會有較高的復發率一般而言, 顎骨緻密纖維瘤手術方式的選擇可由臨床表現來決定, 若皮質骨未穿通或未往軟組織延伸, 作刮除術即可但若病變快速生長 X 光片影像界線不清楚皮質骨已穿通或往軟組織延伸, 則應考慮作片段切除術本病例在臨床上雖有局部破壞下顎骨之現象, 表示腫瘤已由下顎骨皮質骨穿通往軟組織延伸, 但考量到病人年紀較小, 以及顎骨發育未完成, 因此在和病人家長溝通後, 決定採取較保守的局部切除手術方式術後一年恢復狀況良好, 目前無復發徵兆, 但有鑑於緻密纖維瘤的高復發率, 過去學者建議術後追蹤至少要有三年, 本病例之長期追蹤結果, 仍有待觀察參考文獻 1. Weiss S, Goldblum J. Enzinger and Weiss s soft tissue tumors. 4th ed, Mosby Co, St Louis, pp , ohm P, Krober S, Greschniok, Loniado M, Kaiserling E. Desmoplastic fibroma of the bone: a report of two patients, review of the literature, and therapeutic implications. Cancer, 78: , Fletcher CDM, Uni KK, Mertens F. WHO classification of tumors. Pathology and genetics of tumors of soft tissue and bone. IRC Press, Lyon, p. 288, Schajowucz F, ckerman LV, Sisson H. Histological typing of bone tumors. International histological classification of tumours. No. 6. Geneva, WHO, Jaffe HL. Tumors and tumorous conditions of the bones and joints. Lea & Febiger, Philadelphia, p. 298, Griffith JG, Irby W. Desmoplastic fibroma: report of a rare tumor of the oral structures. Oral Surg Oral Med Oral Pathol, 20: , Marlette RH, Gerhard RC. Intraosseous fibroma and fibromyxoma of the mandible. Report of three cases. Oral Surg Oral Med Oral Pathol, 25: , Hinds Ed, Kent JN, Fechner RE. Desmoplastic fibroma of the mandible: report of case. J Oral Surg, 27: , Gosserez M, Stricker M, Rauber G, Pierson. Fibrous tumours of the facial skeleton. Trans Int Conf Oral Surg, 1: , ear SE. Therapy for central non-odontogenic lesions of the jaws. Trans Int Conf Oral Surg, 4: 33-39, Ferguson JW. Central fibroma of the jaws. r J Oral Surg, 12: , adger G, Syed, Malby FC. Desmoplastic fibroma of the mandible. Can J Otolaryngol, 3: , Cunningham CD, Smith RO, Enriquez P, Singleton GT. Desmoplastic fibroma of the mandible. case report. nn Otol Rhinol Laryngol, 84 (1 Pt 1): , Wagner JE, Lorandi CS, Ebling H. Desmoplastic fibroma of bone. case in the mandible. Oral Surg Oral Med Oral Pathol, 43: , Freedman P, Cardo V, Kerpel SM, Lumerman H. Desmoplastic fibroma (fibromatosis) of the jaw bones: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol, 46: , Taguchi N, Kaneda T. Desmoplastic fibroma of the mandible: report of case. J Oral Surg, 38: , ddante RR, Laskin JL. Case 55: large right mandibular Chin Dent J 2006 Vol 25 No 4 285

6 W.H. Chen, Y.S. Kuo,.Y. Liu, et al. mass: clinicopathological conference. J Oral Maxillofac Surg, 43: , George DI, Gould R, Miller RL, Strull NJ. Desmoplastic fibroma of the maxilla. J Oral Maxillofac Surg, 43: , ertoni F, Present D, Marchetti C, acchini P, Stea G. Desmoplastic fibroma of the jaw: the experience of the Instituto eretta. Oral Surg Oral Med Oral Pathol, 61: , Schultz E, Hermann G, Irwin G, Shih H. Case report 380: desmoplastic fibroma of the mandible. Skeletal Radiol, 15: , De Vito M, Tom LW, oran TV, Quinn PD. Desmoplastic fibroma of the mandible. Ear Nose Throat J, 68: , Christiansen RL. Desmoplastic fibroma of the ramus and body of the mandible. Cranio, 8: , Hashimoto K, Mase N, Iwai K, Shinoda K, Sairenji E. Desmoplastic fibroma of the maxillary sinus. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol, 72: , Miyamoto Y, Satomura K, Rikimaru K, Hayashi Y. Desmoplastic fibroma of the mandible associated with tuberous sclerosis. J Oral Pathol Med, 24: 93-96, Iwai S, Matsumoto K, Sakuda M. Desmoplastic fibroma of the mandible mimicking osteogenic sarcoma: report of a case. J Oral Maxillofac Surg, 54: , Hopkins KM, Huttula CS, Kahn M, lbright JE. Desmoplastic fibroma of the mandible: review and report of two cases. J Oral Maxillofac Surg, 54: , Templeton K, Glass N, Young SK. Desmoplastic fibroma of the mandible in a child: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 84: , akaeen G, Rajab LD. Desmoplastic fibroma of the mandible: report of a case. Int J Paediatr Dent, 9: , Kaplan KJ, Torske KR. Pathologic quiz case: a 3-year-old boy with swelling of the right mandible. rch Pathol Lab Med, 126: , Nasser Said-l-Naief, Rui Fernandes, Patrick Louis, Walter ell, Gene P. Siegal. Desmoplastic fibroma of the jaw: case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 101: 82-94, Lagace R, Delage C, ouchard HL, Seemayer T. Desmoplastic fibroma of bone. n ultrastructural study. m J Surg Pathol, 3: , Graudal N. Desmoplastic fibroma of bone. Case report and literature review. cta Orthop Scand, 55: , Crim JR, Gold RH, Mirra JM, Eckardt JJ, assett LW. Desmoplastic fibroma of bone: radiographic analysis. Radiology, 172: , Inwards CY, Unni KK, eabout JW, Sim FH. Desmoplastic fibroma of bone. Cancer, 68: , ridge J, Swarts SJ, uresh C, Nelson M, Degenhardt JM, Spanier S. Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. m J Pathol, 154: , Triantafyllou NM, Triantafyllou DN, ntonados DN. Desmoid tumors of the bone. Int Surg, 57: , Fisker V, Philipsen HP. Desmoplastic fibroma of the jaw bones. Int J Oral Surg, 5: , Osguthorpe JD, dkins WY Jr, Rawe SE. Combined extracranial- intracranial resection of a maxillary desmoid tumor. Otolaryngol Head Neck Surg, 89: , Green TL, Gaffney E. Desmoplastic fibroma of the mandible. J Oral Med, 36: 47-49, Slootweg PJ, Muller H. Central fibroma of the jaw, odontogenic or desmoplastic. Oral Surg Oral Med Oral Pathol, 56: 61-70, Siemssen SJ, nagnostaki T. ggressive fibromatosis (extraabdominal desmoids) of the head and neck. r J Plast Surg, 37: , Neville, Damm D, llen C, ouquot J. Textbook of oral and maxillofacial pathology. 2nd ed, W Saunders Co, Philadelphia, P, US, pp , Peede LF, Epker N. ggressive juvenile fibromatosis involving the mandible: surgical excision with immediate reconstruction. Oral Surg Oral Med Oral Pathol, 43: , Kwon PH, Horswell, Gatto DJ. Desmoplastic fibroma of the jaws: surgical management and review of the literature. Head Neck, 11: 67-75, Rabhan WN, Rosai J. Desmoplastic fibroma: report of ten cases and review of the literature. J one Joint Surg, 50 : , Chin Dent J 2006 Vol 25 No 4

7 Desmoplastic fibroma of the mandible Desmoplastic fibroma of the mandible-case report WEN-HSIU CHEN 1,2 YING-SHIUNG KUO 3,4 U-YUN LIU 3,4 CHUN-PIN CHING 2,4 JNG-JER LEE 3,4 1 School of Dentistry, Chung Shan Medical University, Taichung, Taiwan, ROC. 2 Institute of Clinical Dentistry, College of Medicine, National Taiwan University, Taipei, ROC. 3 School of Dentistry, National Taiwan University, Taipei, ROC. 4 Department of Dentistry, National Taiwan University Hospital, Taipei, ROC. Desmoplastic fibromas are rare, benign bone tumors that histologically resemble desmoid tumors of the soft tissue. Most examples of desmoplastic fibromas of the bone are discovered in patients younger than 30 years, and there is no gender predilection. desmoplastic fibroma may involve any bone but is most frequent in the mandible. It often behaves in a locally aggressive fashion with extensive bone destruction and soft-tissue extension. Metastases do not occur, and the long-term prognosis is good. The surgical approach to the tumor has been a source of controversy. The following is a report of a case of desmoplastic fibroma of the mandible. We present the features, treatment, and prognosis of the lesion which can be used as a reference. Key words: desmoplastic fibroma, mandible, curettage. Received: September 24, 2006 ccepted: November 21, 2006 Reprint requests to: Dr. Jang-Jaer Lee, Department of Dentistry, National Taiwan University Hospital, No. 1, Chang-Te Street, Taipei, Taiwan 10048, ROC. Chin Dent J 2006 Vol 25 No 4 287

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z-rb tpf 台灣獸醫誌林正忠病例報告 : 犬鼻軟骨肉瘤與顏面部及咽部侵犯林正忠詹宥瑜羅登源李衛民國立中興大學獸醫病理學研究所台中市國立嘉義大學獸醫學系嘉義市國立中興大學獸醫系台中市 ( 收稿日期 :95 年 4 月 28 日接受日期 :95