以头皮血肿为首发症状的原发性硬脑膜非霍奇金淋巴瘤1例

2019-03-21 阮航 吴京雷 段发亮 实用医学杂志

患者女,57岁。因“发现反复增大的额部头皮血肿2个月”入院。患者撞击硬物后发现左额部头皮肿物,逐渐增大,伴疼痛,穿刺抽吸后缩小,抽吸物为暗红色血液,但1周后血肿再次增大入院。查体:神经系统查体阴性,左额部见一大小约5 cm×3 cm包块,有波动感,轻压痛,无红肿及分泌物,移动度差。全身浅表淋巴结无肿大,心肺腹检查未见异常,血常规淋巴细胞分类正常。

患者女,57岁。因“发现反复增大的额部头皮血肿2个月”入院。患者撞击硬物后发现左额部头皮肿物,逐渐增大,伴疼痛,穿刺抽吸后缩小,抽吸物为暗红色血液,但1周后血肿再次增大入院。查体:神经系统查体阴性,左额部见一大小约5 cm×3 cm包块,有波动感,轻压痛,无红肿及分泌物,移动度差。全身浅表淋巴结无肿大,心肺腹检查未见异常,血常规淋巴细胞分类正常。
 
颅骨CT显示:双侧额骨受侵蚀破坏,骨板毛糙,板障可见斑片状低密度影,头皮软组织肿胀(图1A)。MRI显示:肿瘤位于额骨内板下窦镰旁硬脑膜上,以左侧为主,并跨中线向右侧生长,T1WI呈稍短信号,T2WI呈稍长信号(图1B、1C),增强扫描呈明显均匀强化,略呈团块状,边界清楚,可见脑膜尾征,周围脑膜强化增厚,临近额骨板障强化(图1D、1E),头皮下可见肿胀及血肿。
 




图1 57岁女性原发性硬脑膜非霍奇金淋巴瘤患者影像学图。注:A,颅骨CT示双侧额骨内斑片状低密度影;B、C,轴位T1WI及T2WI示稍短T1稍长T2信号;D、E,增强扫描示明显均匀强化
 
全麻下行肿瘤切除术,术中见肿瘤起源于大脑镰旁硬脑膜,呈灰白色,质地较韧,在硬膜内外及蛛网膜表面呈匍匐样生长,侵犯局部头皮内层,颅骨全层及软脑膜,肿瘤部位上矢状窦被侵犯闭塞,包膜不明显,与脑组织分界较清楚,血供较丰富。术中考虑为恶性脑膜瘤,切除受侵犯的头皮内层、颅骨全层、硬脑膜、上矢状窦、大脑镰及软脑膜。术后病理诊断:非霍奇金弥漫性大B细胞淋巴瘤
 
免疫组化:CD20弥漫(+),CD79a弥漫(+),PAX-5(+),CD10弱(+),BCL-6(+),CD43部分(+),CD21(+),Ki-67(约80%)。
 
术后3周患者出现腮腺包块及颈部淋巴节肿大,全身PET-CT提示:(1)双侧额骨(手术切口下方)及双侧颈部多发淋巴结代谢异常增高,考虑为恶性淋巴瘤浸润。(2)双侧腮腺结节代谢异常增高,考虑恶性淋巴瘤浸润可能性大。予鞘内注射化疗药物及全身化疗后患者腮腺包块及颈部淋巴节肿大消退出院。后经多次放化疗,患者3个月随访情况尚可。
 
讨论
 
原发性中枢神经系统淋巴瘤是一种特殊类型的结外非霍奇金淋巴瘤,约占颅内肿瘤的1%~3%,近年来发病率快速上升。其好发于脑实质内,以深部白质为主要发病部位,仅少数累计脑外组织,原发性于硬脑膜者则更为罕见。原发性硬脑膜淋巴瘤在生物学特性上与脑恶性淋巴瘤明显不同,其通常是低级别边缘区淋巴瘤,且女性明显多于男性。而脑恶性淋巴瘤多为中高级别弥漫性大B细胞淋巴瘤,男女比例约1.5∶1。本例患者为女性,但病理诊断为弥漫性大B细胞淋巴瘤,因此在原发性硬脑膜淋巴瘤中也较为少见。
 
原发性硬脑膜淋巴瘤临床表现缺乏特异性,大部分表现为颅内压升高症状和脑损伤部位定位体征,部分额叶受压致脑水肿患者有癫痫表现和精神症状,虽然很多硬脑膜淋巴瘤会侵蚀颅骨,但像本病例侵蚀颅骨全层及头皮内层,并以头皮血肿为首发症状者则极为罕见。
 
在影像学上,MRI较CT更能表现肿瘤范围和周围水肿,T1WI常为等或低信号,T2WI呈等信号或稍高信号,强化明显,常有脑膜尾征和周围脑膜强化。本病多发生于脑膜细胞丰富的部位,因此与脑膜瘤等源发于脑膜的病变鉴别较困难,确诊仍需依赖病理检查。原发性硬脑膜淋巴瘤手术效果较满意,且对放化疗较为敏感,治疗应以手术切除+放疗+化疗的综合治疗方案为主,但预后仍与组织生物学特性密切相关。
 
原始出处:

阮航,吴京雷,段发亮,何主强,陈晓斌.以头皮血肿为首发症状的原发性硬脑膜非霍奇金淋巴瘤1例[J].实用医学杂志,2018,34(06):1046.

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    2019-06-28 wrj0121
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