CHAPTER 28 Squamous Cell Carcinoma (Middle Ear)
Epidemiology
Primary middle ear squamous cell carcinoma is a rare neoplasm seen in less than 1 in 10,000 patients presenting with otologic disease. It is less common than primary external auditory canal squamous cell carcinoma.
This rare carcinoma is most often seen in 50- to 60-year-old men. It is associated with chronic otitis media, which is associated with increased cellular metaplasia due to ongoing inflammation/otorrhea possibly mediated through human papilloma virus (which is present in many of these patients). These carcinomas are also associated with prior radiation therapy.
These patients have a poor prognosis, as most present with advanced disease (e.g., temporal bone or intracranial extension) and it is difficult, if not impossible, to achieve complete neoplasm resection because many crucial structures are nearby (brain, skull base internal carotid artery, cavernous sinus, dural venous sinuses, etc.). Five-year survival is less than 50% in patients who are treated with surgery and radiation therapy.
Clinical Features
These patients present with a long history of ear symptoms, namely pain, discharge, bleeding, and refractory granulation formation. These patients may also have facial palsy reflecting involvement of the tympanic portion of cranial nerve VII. Sudden deafness can be seen late in the course of the disease. The clinical scenario of seventh nerve palsy, deafness, and continuing otorrhea should suggest a middle ear process. The diagnosis is often initially overlooked, as middle ear squamous cell carcinoma is a rare entity and all of the symptoms seen in these patients (except for the facial nerve palsy) are typical of chronic otitis media.
Pathology
Middle ear squamous cell carcinoma probably arises from tympanic mucosa. Temporal bone squamous cell carcinomas are considered stage 1 and 2 when limited to the external auditory canal. By definition, they are considered stage 3 when they involve the middle ear (stage 4 lesions involve the temporal bone more extensively). Cases of primary middle ear squamous cell carcinoma are so rare that there are no reliable statistics as to whether these lesions metastasize. As mentioned above, these patients have a poor prognosis primarily due to their overall late presentation.
Treatment
Treatment for middle ear (and all temporal bone) squamous cell carcinomas includes surgery, chemotherapy, and postsurgical radiation therapy. In cases of extensive disease, palliative radiation therapy only may be performed.
Imaging Findings
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