鼻咽部侵袭性纤维瘤病1例

2019-03-17 尚紫薇 谷佳 魏宏权 中国耳鼻咽喉颅底杂志

患者,男,47岁,因头痛、左耳闷3月余,加重伴伸舌偏斜1周,于2015年6月门诊以鼻咽部占位性病变收住院。患者诉3个月前无明显诱因出现头痛、左耳闷,近1周来伸舌向左偏斜(图1)。否认家族史及外伤史。

侵袭性纤维瘤病(Aggressive fibromatosis,AF) 是一种起源于肌肉、深筋膜和腱膜等处的富含胶原纤维成分的纤维组织肿瘤,几乎可见于全身各部位,高复发性和不发生远处转移是其显著特征。该病在形态学上表现为良性,但呈浸润性生长,有局部复发倾向,生物学行为明显类似恶性肿瘤。临床上多见于腹壁、腹内及下肢,发生在头颈部的侵袭性纤维瘤病较少见,其中位于鼻咽部者国内未见有报道。

病例报告

患者,男,47岁,因头痛、左耳闷3月余,加重伴伸舌偏斜1周,于2015年6月门诊以鼻咽部占位性病变收住院。患者诉3个月前无明显诱因出现头痛、左耳闷,近1周来伸舌向左偏斜(图1)。否认家族史及外伤史。查体:伸舌向左偏斜,鼻腔内未见肿物,颈淋巴结未触及。常规化验检查正常。首诊于我院神经内科,临床诊断舌下神经疾患,行颅脑增强 MRI发现鼻咽部占位。鼻窦增强3D-CT扫描示鼻咽后壁软组织增厚,累及双侧咽鼓管圆枕及头长肌,平扫CT值77HU,增强扫描中度强化,CT值96HU,双侧咽隐窝不清,鼻咽腔变形(图2)。鼻咽部增强MRI表现为鼻咽后顶壁软组织增厚且欠规整,增强后明显强化,异常信号累及头长肌间及表面,左鼻咽旁、邻近枕骨斜坡、左侧破裂孔增强亦可见斑片状强化灶,左侧乳突他信号增高。患者于6月26日行”鼻内镜下鼻咽部探查取病理术”,术中发现鼻咽部黏膜下软骨样、白色质硬韧肿物,不易出血。



取部分瘤组织行病理检查,示“慢性炎症伴纤维组织增生,考虑为侵袭性纤维瘤病”。明确病理诊断后就诊于国内多家医院,曾以“炎性病变”为诊断治疗,但头痛、耳闷及伸舌偏斜无缓解,严重影响睡眠和生活质量。再次于同年8月3日于我院在全麻下行“鼻内镜下鼻咽部肿物切除术”。术中见病变性质特点同前,为质地硬韧软骨样组织,沿头长肌表面筋膜呈侵袭性生长,与周围组织无明显界限,向后达下斜坡骨面,向前上方达鼻咽顶和后鼻孔上缘,向两侧接近枕骨髁。术中在影像导航系统辅助下,完全切除病灶,深度至暴露出头长肌或斜坡骨面,向两侧达舌下神经孔周围。术后再次送病理确诊为侵袭性纤维瘤病 (图3)。显微镜下瘤细胞梭形、束状分布,浸润周围肌肉组织,细胞排列稀疏,核梭形异型不明显。免疫组织化学示:CK(上皮+) Vimentin(+) SMA(血管+) S-100(-) CD34(血管+) VOB-Catenin(散在+) CD56(-) Ki-67(局灶2%+)。术后恢复良好,无吞咽困难等并发症。于2015年9月3日至11月21 日在我院放疗科行调强放射治疗,每次照射剂量2 Gy,总剂量50 Gy,放疗中出现口腔黏膜反应Ⅱ度,无鼻咽黏膜坏死、颞叶坏死等严重并发症。术后头痛、耳闷症状消失,但伸舌偏斜无缓解,定期复查,随访28个月未见复发,影像学复查并与术前对比见图4。

讨论 

侵袭性纤维瘤病又称韧带样瘤、硬纤维瘤、肌腱膜纤维瘤病等,是一种起源于问质干细胞的软组织 肿瘤,发病中位年龄30-40岁,女性患者多见,且多发于育龄期女性。病因及发病机制目前尚未明确,可能与外伤、手术、遗传和内分泌异常等因素有关。本病较为少见,其发病率仅占所有肿瘤的0.03%,发生于腹外者罕见,预后较差,复发率达40%~60%,主要见于颈肩部、胸壁和下肢,头颈 部较少见(8%~25%)。在头颈部,侵袭性纤维瘤病最常见于颈部,颅面部次之,按照发生率高低,依次是下颌骨、口腔、鼻窦和眼眶等。位于鼻咽部者更为罕见,此方面的文献报道极少,国内未见有报道,国外仅Gnepp等于1996年有提及,一例54岁鼻咽侵袭性纤维瘤病患者,手术部分切除后未辅助治疗,随访20年死亡,死因不明。

本病临床表现不特异,常常导致诊断困难或误诊。增强MRI及CT检查有助于了解肿瘤的大小、形态、信号特征及骨破坏的程度,还能评估与周围重要结构如神经、血管等的关系,可作出提示性诊断。头颈部侵袭性纤维瘤病主要与其他纤维瘤病、纤维肉瘤、特发性纤维化等相鉴别,明确诊断需依靠病理及免疫组化检查。

侵袭性纤维瘤病的最佳治疗策略至今未确定,治疗方法有手术、放射治疗、化学疗法和激素治疗等。由于本病具有侵袭性强的特点,大多数学者认为完整的手术切除是首选治疗方法。然而在实际临床工作中,位于头颈部的侵袭性纤维瘤病,由于解剖位置的限制,肿瘤生长空间小,且与周围血管、神经及重要器官黏连,关系密切,常因必须保留器官功能或美容需求,而不能完全切除或手术安全界不足,故术后复发率高达70%,且危及生命。有学者认为,不应强调积极的扩大切除,应在难以达到根治性切除时尽量保留器官结构及功能性,术后补充放疗。大多数人认为术后辅助放射治疗可以大大降低局部复发的风险,不仅可以有效的控制肿瘤进展,显著提高手术的治愈率,且能促进大部分肿瘤部分或完全消退。其他治疗方式还有抗雌激素治疗、抗炎治疗、干扰素治疗、化疗、靶向治疗等,治疗机制仍需进一步研究。本病例在影像导航系统引导下,采用内镜经鼻扩大径路完整切除鼻咽部肿瘤,结合术后局部调强放疗,随访28个月无复发。但由于侵袭性纤维瘤病易复发,应长期密切随访,90%肿瘤的复发发生在5年内,故随访时间不应 少于5年。鉴于本病散发且少见,目前很多研究结论不确切甚至互相矛盾,因此有必要进行大范围前瞻性多中心联合研究,以建立一套完善确切的诊治标准。

原始出处:

尚紫薇,谷佳,魏宏权.鼻咽部侵袭性纤维瘤病1例[J].中国耳鼻咽喉颅底杂志,2018,4(3):288-289.

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