Zhu Jay-Jiguang, Padillo Osvaldo, Duff John, Hsi Bae-Li, Fletcher Jonathan A, Querfurth Henry
Division of Neurology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, 736 Cambridge Street, Boston, MA 02135, USA.
Neurosurgery. 2004 Feb;54(2):492-8; discussion 498-9. doi: 10.1227/01.neu.0000103674.30974.69.
Invasion of trigeminal and facial perineural spaces is a recognized complication of cutaneous malignancies. Centripetal spread along the trigeminal nerve axis and into the cavernous sinus and the gasserian ganglion is rare. Metastasis to the leptomeninges and cauda equina has not been reported. We report a unique case of perineural spread and central dissemination from an epithelial squamous cell carcinoma (SCC) associated with a tumor biomarker.
After excision of multiple cutaneous SCCs and basal cell carcinomas of the head and neck, a 70-year-old male patient developed successive, right-side, V1 and V2 trigeminal neuropathies and complete right cavernous sinus syndrome during a 5-year period. Concurrently, the right face became paralyzed. Left facial paresis developed during the latter half of this period. Two months before admission, subacute left lower-extremity radicular weakness resulted in falls. Serial magnetic resonance imaging scans obtained in the previous 4 years were unrevealing. At the time of admission, enhancing masses were found in the 1) right cavernous sinus and dura, foramina ovale and rotundum, and Meckel's cave, 2) right subtemporal region and orbital rectus muscles, and 3) cauda equina. Cerebrospinal fluid analysis demonstrated mild pleocytosis and rare carcinoma cells.
Biopsy of the right cavernous sinus mass confirmed moderately differentiated, metastatic SCC. Immunohistochemical staining and fluorescence in situ hybridization revealed epidermal growth factor receptor overexpression and genomic amplification.
The indolent progression of cranial nerve palsy among patients with resected cutaneous SCCs of the head and neck must raise clinical suspicion of perineural spread, even in the absence of radiological changes. Biomarkers predicting aggressive SCC behavior, illustrated here by epidermal growth factor receptor amplification and central invasion, have the potential to guide early therapy.
三叉神经和面神经周围间隙受侵是皮肤恶性肿瘤公认的并发症。沿三叉神经轴向往心性扩散并累及海绵窦和三叉神经节较为罕见。向软脑膜和马尾转移尚未见报道。我们报告1例伴有肿瘤生物标志物的上皮性鳞状细胞癌(SCC)发生神经周围扩散和中枢播散的独特病例。
1例70岁男性患者在切除头颈部多发皮肤SCC和基底细胞癌后,5年内相继出现右侧V1和V2三叉神经病变及完全性右侧海绵窦综合征。同时,右侧面部瘫痪。在此期间后半段出现左侧面部轻瘫。入院前2个月,亚急性左下肢神经根性无力导致跌倒。前4年的系列磁共振成像扫描均未发现异常。入院时,在以下部位发现强化肿块:1)右侧海绵窦、硬脑膜、卵圆孔和圆孔以及梅克尔腔;2)右侧颞下区和眶直肌;3)马尾。脑脊液分析显示轻度细胞增多且偶见癌细胞。
右侧海绵窦肿块活检证实为中分化转移性SCC。免疫组织化学染色和荧光原位杂交显示表皮生长因子受体过表达和基因组扩增。
头颈部皮肤SCC切除术后患者出现的隐匿性进展性脑神经麻痹,即使在无影像学改变的情况下,也必须引起对神经周围扩散的临床怀疑。预测侵袭性SCC行为的生物标志物,如此处所示的表皮生长因子受体扩增和中枢侵犯,有可能指导早期治疗。