Zhang Michael, Phung Daniel, Gupta Ruta, Wykes James, Wu Raymond, Lee Jenny, Elliott Michael, Palme Carsten E, Clark Jonathan, Low Tsu-Hui Hubert
Sydney Head and Neck Cancer Institute, Chris O'Brien Lifehouse, Sydney, New South Wales, Australia.
Sydney Medical School, Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.
ANZ J Surg. 2023 Oct;93(10):2394-2401. doi: 10.1111/ans.18625. Epub 2023 Jul 23.
Perineural spread (PNS) of head and neck cutaneous squamous cell carcinoma (HNcSCC) is a unique diagnostic challenge, presenting with insidious trigeminal (CN V) or facial nerve (CN VII) neuropathies without clinically discernible primary masses. These patients are often sub-optimally investigated and misdiagnosed as Bell's palsy or trigeminal neuralgia. This case series highlights the red flags in history and pitfalls that lead to delays to diagnosis and treatment.
A retrospective case series of 19 consecutive patients with complete clinical histories with HNcSCC PNS without an obvious cutaneous primary lesion at time of presentation to a quaternary head and neck centre in Australia were identified and included for analysis.
Fifteen had CN VII PNS, 17 had CN V PNS, and 13 had both. The overall median symptom-to-diagnosis time was 12-months (IQR-15 months). Eight patients had CN VII PNS and described progressive segmental facial nerve palsy with a median symptom-to-diagnosis time of 9-months (IQR-11.75 months). Eleven patients had primary CN V PNS and described well localized parathesia, formication or neuralgia with a median symptom-to-diagnosis time of 19-months (IQR 27.5 months).
PNS is often mistaken for benign cranial nerve dysfunction with delays in diagnosis worsening prognosis. Red flags such as progressive CN VII palsy or persistent CN V paraesthesia, numbness, formication or pain, particularly in the presence of immuno-compromise and/or a history of facial actinopathy should raise suspicion for PNS. Gadolinium-enhanced MR Neurography should be obtained expediently in patients with persistent/progressive CN V/CN VII palsies in patients with red flags, with low threshold for referral to a Head and Neck Surgeon.
头颈部皮肤鳞状细胞癌（HNcSCC）的神经周围扩散（PNS）是一项独特的诊断挑战，表现为隐匿性三叉神经（CN V）或面神经（CN VII）神经病变，而临床上无法识别原发性肿块。这些患者的检查往往不够完善，常被误诊为贝尔麻痹或三叉神经痛。本病例系列突出了病史中的警示信号以及导致诊断和治疗延误的陷阱。
对澳大利亚一家四级头颈中心收治的19例连续的HNcSCC PNS患者进行回顾性病例系列研究，这些患者在就诊时无明显皮肤原发性病变且有完整的临床病史，纳入分析。
15例有CN VII PNS，17例有CN V PNS，13例两者皆有。总体症状至诊断时间的中位数为12个月（四分位间距 - 15个月）。8例有CN VII PNS，表现为进行性节段性面神经麻痹，症状至诊断时间的中位数为9个月（四分位间距 - 11.75个月）。11例有原发性CN V PNS，表现为定位良好的感觉异常、蚁走感或神经痛，症状至诊断时间的中位数为19个月（四分位间距27.5个月）。
PNS常被误诊为良性颅神经功能障碍，诊断延迟会使预后恶化。进行性CN VII麻痹或持续性CN V感觉异常、麻木、蚁走感或疼痛等警示信号，特别是在存在免疫功能低下和/或面部放线菌病病史的情况下，应引起对PNS 的怀疑。对于有警示信号且存在持续性/进行性CN V/CN VII麻痹的患者，应尽快进行钆增强磁共振神经成像检查，转诊至头颈外科医生的阈值要低。
