|Year : 2021 | Volume
| Issue : 4 | Page : 161-163
Cerebrospinal fluid otorrhea and facial palsy – An unlikely presentation of metastatic nasopharyngeal carcinoma
Rachel C.A. Lim1, Bee See Goh1, Yin Peng Wong2
1 Department of Otorhinolaryngology and Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia
2 Department of Pathology, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia
|Date of Submission||13-Mar-2021|
|Date of Acceptance||26-Jun-2021|
|Date of Web Publication||01-Sep-2021|
Prof. Bee See Goh
Department of Otorhinolaryngology and Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Center, Jalan Yaacob Latif, 56000 Cheras, Kuala Lumpur
Source of Support: None, Conflict of Interest: None
Cerebrospinal fluid (CSF) otorrhea and facial nerve palsy as a result of nasopharyngeal cancer (NPC) metastasis to the mastoid postradiotherapy is rarely encountered. The diagnosis is not straightforward as the initial presentation could be mistaken for the middle ear effusion or chronic otitis media which are common complications following radiotherapy. Persistent symptoms despite adequate medical therapy should alert the clinician to a more ominous pathology. We report a rare and unusual case of CSF otorrhea and facial palsy secondary to temporal bone metastasis in a patient with NPC postradiotherapy.
Keywords: Cerebrospinal fluid otorrhea, facial nerve palsy, nasopharyngeal carcinoma, radiotherapy
|How to cite this article:|
Lim RC, Goh BS, Wong YP. Cerebrospinal fluid otorrhea and facial palsy – An unlikely presentation of metastatic nasopharyngeal carcinoma. Saudi J Otorhinolaryngol Head Neck Surg 2021;23:161-3
|How to cite this URL:|
Lim RC, Goh BS, Wong YP. Cerebrospinal fluid otorrhea and facial palsy – An unlikely presentation of metastatic nasopharyngeal carcinoma. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2021 [cited 2022 Jan 21];23:161-3. Available from: https://www.sjohns.org/text.asp?2021/23/4/161/325411
| Introduction|| |
Nasopharyngeal cancer (NPC) is an endemic disease common in Southeast Asia, with a racial predilection toward individuals of Chinese descent., Following the treatment with concurrent chemoradiotherapy, these patients undergo regular follow-up to monitor for recurrence. These patients may develop middle ear effusion (MEE), sensorineural hearing loss, or chronic otitis media which are known complications postradiotherapy. These seemingly innocuous conditions may point to a more serious pathology such as tumor recurrence. This is an unusual case of cerebrospinal fluid (CSF) otorrhea and facial palsy secondary to the temporal bone metastasis in a patient with NPC postradiotherapy. Based on the current English literature search, this is the only case reported.
| Case Report|| |
A 72-year-old Chinese gentleman with NPC (T2N2M0, WHO type 2), presented with 2-month history of right facial asymmetry 2 years postradiotherapy. He had previously undergone myringotomy and grommet insertion for MEE postradiotherapy. The patient developed unresolving serous ear discharge increasing in frequency and amount over the past 4 months. He also complained of worsening hearing but denied any otalgia, vertigo, or tinnitus. Otherwise, there were no significant nasal symptoms, neck swelling, meningism, or constitutional symptoms.
The clinical examination revealed right lower motor neuron facial nerve palsy (House-Brackmann Grade IV). On otoscopy, the right external auditory canal was narrowed and edematous with clear pulsatile discharge. The tympanic membrane was perforated with no grommet seen. Further examination of the oral cavity and neck was unremarkable. Except for right cranial nerves VII and VIII, other cranial nerves were intact. On nasendoscopy, bilateral fossa of Rosenmüller showed no evidence of tumor recurrence.
Computed tomography (CT) scan and magnetic resonance imaging of the brain and temporal bone detected an enhancing soft-tissue lesion occupying the right middle and external ear eroding the scutum, facial canal, mastoid, and tegmen tympani. The soft-tissue extended intracranially from the tegmen tympani into the right posterolateral temporal fossa with adjacent pachymeningeal enhancement and a small subdural collection [Figure 1] and [Figure 2]. There was no evidence of recurrence in the nasopharynx. CT cisternogram did not show any bony defect or contrast leak.
|Figure 1: Magnetic resonance imaging scan (coronal view) showing right subdural collection (arrow) and soft-tissue lesion at the temporal bone causing narrowing of the external auditory canal|
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|Figure 2: (a) Computed tomography of the temporal bone in axial view showing bony erosion of the right mastoid bone (arrow). (b): Erosion of the tympanic segment of facial nerve canal|
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The patient was diagnosed as having right CSF otorrhea and facial nerve palsy secondary to chronic mastoiditis with underlying nasopharyngeal carcinoma postradiotherapy. A lumbar drain was inserted but symptoms persisted. Thus, he came to our center for second opinion. After a multidisciplinary discussion with the neurosurgeon and radiologist, we proceeded with a right cortical mastoidectomy, extended posterior tympanotomy, and CSF leak repair. Intraoperatively, unhealthy tissue was noted at the periosteum of the postauricular region with granulation tissue occupying the mastoid antrum and middle ear cavity. A 2 mm × 2 mm defect over the tegmen tympani was discovered which demonstrated minimal CSF leak on Valsalva maneuver. Bony erosion of the root of zygoma and tegmen mastoideum was also noted. Tissues from the mastoid, middle ear, and periosteum were biopsied. Histopathological examination revealed tissue infiltration by sheets of malignant epithelial cells arranged in syncytial pattern [Figure 3]. Histology confirmed metastatic nonkeratinizing NPC with the establishment of Epstein–Barr virus infection in the tumor cells by in situ hybridization technique. The diagnosis was hence revised to CSF otorrhea and facial nerve palsy secondary to the temporal bone metastasis from NPC.
|Figure 3: (a) Tissue infiltration by sheets of malignant cells exhibiting pleomorphic, hyperchromatic nuclei with inconspicuous nucleoli, and moderate cytoplasm. Mitotic figure (arrow) is seen. Immunohistochemically, the malignant cells show strong and diffuse (b) CK AE1/AE3 and (c) p63 immunopositivity. They display immunonegativity for (d) chromogranin and (e) synaptophysin. (f) The malignant cells show Epstein–Barr virus-encoded RNA in situ hybridization positivity|
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Follow-up imaging with positron emission tomography showed metabolic activity at the right middle cranial fossa-temporal region indicative of infection or metastasis. After discussion with oncologist, the patient opted for palliative chemotherapy and is well on follow-up 6 months later.
| Discussion|| |
The patients with NPC postradiotherapy routinely undergo endoscopic surveillance to detect local recurrence. Patients may complain of ear block, otorrhea, and reduced hearing postradiation which are common complications of radiotherapy secondary to damage to the Eustachian tube More Details (ET). The effects of radiation are evidenced by ciliary loss, intracellular vacuolation, and ciliary dysmorphism noted in the ET mucosa on pathologic examination. In addition, ET dysfunction can occur secondary to tumor involvement causing functional and mechanical obstruction. Tumor cell invasion can be found within the submucosal layers of the ET causing luminal blockage. Recent reports have highlighted functional obstruction being a major factor in ET dysfunction where there is tumor infiltration of the ET musculature with paralysis of the tensor veli palatini and erosion of the ET cartilage., Based on this, a diagnosis of MEE in these individuals should always be approached with caution to avoid missing a metastatic tumor such as in the present case.
A few pathways of spread of NPC to the middle ear have been suggested: (1) through the ET; (2) direct infiltration of the parapharyngeal space; and (3) from cavernous sinus through carotid canal.,, In our case, the tumor spread to the middle ear had caused bony erosion of the facial nerve and tegmen tympani. Facial nerve palsy in NPC is a rare finding with an incidence of <1%. The facial palsy had been erroneously thought to be a complication of mastoiditis before further investigation proved a metastatic tumor. Other diagnoses that need to be considered are Bell's palsy and metastasis to the parotid or cerebellopontine angle.
The extensive bony erosion involving the tegmen tympani seen on imaging and CSF leak that was demonstrated intraoperatively suggested possible osteoradionecrosis, which is a rare complication postradiotherapy. Reported cases of CSF leak postradiotherapy for NPC are few with one such case reported by Lim et al. where the CSF otorrhea was secondary to the temporal bone osteoradionecrosis. In our patient, surgical findings raised suspicion of a malignant or infective process due to extensive granulation tissue in the middle ear. Histopathological analysis confirmed a metastatic carcinoma. In conclusion, most patients with NPC develop seemingly benign ear complications postradiation. Follow-up visits focus on detecting local recurrence on scope and neck metastasis. Despite normal findings on scope and neck examination, it is crucial to closely monitor a patient with persistent ear symptoms warranting further investigation. This case emphasizes the importance of having a high degree of clinical suspicion coupled with pathologic diagnosis to avoid misdiagnosis and delayed treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to acknowledge all involved in this case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]