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Introduction

Otolaryngologists are keenly aware of the fact that parotid carcinoma may initially present with facial weakness . When facial paresis occurs in association with parotid malignancy, it is ordinarily slow in onset, progressive in nature, and without evidence of spontaneous recovery . Although parotid malignancy may present with acute-onset, complete facial paralysis, few well-documented cases exist in the literature . Most importantly, when a patient continues to have a flaccid face 6 months after acute-onset facial paralysis, a thorough evaluation through an algorithmic approach must be initiated to prevent diagnostic delays.

We present 4 additional cases of acute-onset, complete facial paralysis due to occult parotid malignancy, in which there was a significant diagnostic delay. We analyze the presentation and diagnostic evaluation and discuss ways to avoid diagnostic delays in this specific subset of patients with facial paralysis. This study was approved by the institutional review board at the Massachusetts Eye and Ear Infirmary.

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Case 1

A 51-year-old man presented with a history of acute-onset right facial paralysis that developed over 8 hours without evidence of emergence from the flaccid state after 9 months. Within 24 hours of symptom onset, he was treated with prednisone and valacyclovir for a presumptive diagnosis of Bell’s palsy. He was then treated with doxycycline for the possibility of seronegative Lyme disease 1 month later. An extensive hematologic evaluation and a noncontrast brain magnetic resonance imaging (MRI) were unremarkable. Upon referral to the senior author at 9 months after onset, he had right flaccid facial paralysis with brow ptosis, incomplete eye closure, complete effacement of the nasolabial fold, and malposition of the oral commissure. There was no palpable facial or neck mass. A gadolinium-enhanced temporal bone and neck MRI and a high-resolution fine-cut temporal bone computed tomography scan showed an infiltrative mass involving the right parotid gland and facial nerve, extending to the stylomastoid foramen and into the infratemporal fossa. Pathology revealed high-grade adenocarcinoma with extensive perineural spread.

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Case 1

A 51-year-old man presented with a history of acute-onset right facial paralysis that developed over 8 hours without evidence of emergence from the flaccid state after 9 months. Within 24 hours of symptom onset, he was treated with prednisone and valacyclovir for a presumptive diagnosis of Bell’s palsy. He was then treated with doxycycline for the possibility of seronegative Lyme disease 1 month later. An extensive hematologic evaluation and a noncontrast brain magnetic resonance imaging (MRI) were unremarkable. Upon referral to the senior author at 9 months after onset, he had right flaccid facial paralysis with brow ptosis, incomplete eye closure, complete effacement of the nasolabial fold, and malposition of the oral commissure. There was no palpable facial or neck mass. A gadolinium-enhanced temporal bone and neck MRI and a high-resolution fine-cut temporal bone computed tomography scan showed an infiltrative mass involving the right parotid gland and facial nerve, extending to the stylomastoid foramen and into the infratemporal fossa. Pathology revealed high-grade adenocarcinoma with extensive perineural spread.

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Case 2

An 84-year-old man presented with a history of left-sided facial paralysis that developed over a 3-day period without evidence of any recovery by 6 months after onset. He had been diagnosed with Bell’s palsy and treated with steroids and antiviral medication at onset. After 6 months without recovery, he had a contrast-enhanced computed tomography (CT) of the temporal bone and brain that was negative for tumor. On examination, he had left flaccid facial paralysis, with brow ptosis at rest, no brow elevation, incomplete eye closure with full effort, midfacial flaccidity, oral incompetence, and a firm, fixed, palpable mass in the immediate infra-auricular region. A contrast-enhanced CT of the neck and parotid showed a 2.0×2.5×1.8-cm infiltrative mass lesion of the parotid, extending to the stylomastoid foramen, with evidence of metastatic disease in 3 cervical lymph nodes. Pathology showed a primary ductal adenocarcinoma in the parotid with multiple metastatic lymph nodes.

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Case 3

An 84-year-old man presented with dense right facial paralysis that developed over the course of 1 day 3 years before referral. At onset, he was diagnosed with Bell palsy and treated with antibiotics and pain medication. Two years after onset, he had right facial numbness and continued flaccid facial paralysis. A contrast-enhanced MRI of the brain was performed, which showed enhancement of the facial nerve from the cannalicular segment to the stylomastoid foramen, as well as diffuse enhancement of the right trigeminal nerve. He presented to the facial nerve center 36 months after onset of facial paralysis. On examination, he had severe right brow ptosis, incomplete eye closure with effort, dense midfacial paralysis, and flaccid lower lip weakness. Subsequent MRI of the head and neck with gadolinium showed a deep right parotid tumor with perineural infiltration of the facial and trigeminal nerves. Biopsy revealed poorly differentiated squamous cell carcinoma, likely from a cutaneous primary lesion.

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Case 4

A 39-year-old woman presented with right facial weakness of rapid onset that had developed 14 months earlier, 2 days after experiencing tingling and twitching in the right face. At onset, she was treated with a 6-day prednisone taper for presumed Bell’s palsy, had an unremarkable noncontrast head CT, and negative Lyme titer. At 4 months, she had little to no recovery, and a repeat head CT with contrast was normal. A gadolinium-enhanced MRI of the head and neck, however, showed 2 small areas of T2 hyperintensity in the superior portion of the parotid gland and within the mandibular condyle. On examination, she had a palpable nodule in the immediate pretragal area with overlying peau d’orange skin changes, trace right brow ptosis, no volitional brow movement, and incomplete eye closure with effort. There was midfacial weakness but detectable movement of the oral commissure. Review of her prior MRI with a head and neck radiologist revealed replacement of the right parotid with abnormal tissue. Needle biopsy was suspicious for malignancy. She underwent total parotidectomy, facial nerve sacrifice, excision of involved pretragal skin, segmental mandibulectomy, neck dissection, and mastoidectomy. For facial reanimation, the medial antebrachial cutaneous nerve was used as a branched cable graft from the tumor-free proximal mastoid segment of the facial nerve to 4 distal branches. Pathology showed a poorly differentiated, invasive, adenoid cystic carcinoma with extensive perineural invasion, and metastatic disease in 3 of 30 lymph nodes.