Temporal Bone CarcinomaTemporal bone carcinoma
Section snippets
Historical Considerations
Carcinomas of the external auditory canal and middle ear probably were first described around 1775, but without pathologic confirmation. Credit for the first comprehensive description was given to Politizer in 1883 in his classic Textbook of Disease of the Ear.53 Early attempts at piecemeal removal of temporal bone carcinoma soon followed, highlighted by Heyer's report29, 35 and Zeroni's report in the German literature of 121 cases described between 1804 and 1899,43 both written in 1899.
Demographics
Although studies vary regarding whether men or women show a higher incidence of temporal bone carcinoma, pooled data show an approximately even gender distribution.35 As with most epithelial cancers, the majority of cancers of the external auditory canal and middle ear occur in the fifth and sixth decades of life. The tumor can be present in children, however. In his 1965 report of 50 cases, Conley12 had four patients under the age of 4 and two teenage patients. Squamous cell carcinoma is the
Clinical Presentation
The common clinical symptoms and signs are presented in Table 1. In most series, otorrhea is the primary symptom, and pain, facial nerve palsy, and an external auditory canal mass occur later with tumor extension.1, 31, 35, 36, 42, 43, 50 Diagnosis usually is delayed because symptoms mimic more common otologic conditions. In Leonetti et al's review,36 all 26 patients presented with stage III or IV disease and a mass occluding the external auditory canal. A review of Moffat43 et al's list of
Pathogenesis
There are several etiologic factors suspected in the development of squamous cell carcinoma of the temporal bone. The most important factor may be exposure to ultraviolet radiation, especially in fair-skinned people.43 A susceptibility to develop squamous cell carcinoma in unexposed skin, such as the external auditory canal, is believed to be determined genetically.43 The association of chemicals, such as chlorinated disinfectants, has been proposed.45 Chronic suppurative otitis media was
Differential Diagnosis
The primary differential diagnosis of squamous cell carcinoma of the temporal bone is not one of the tumors listed, but includes benign conditions that cause otorrhea. The conditions listed as follows are not exhaustive:
Benign
Otitis externa
Tympanic membrane perforation with polyp or cholesteatoma
External auditory canal cholesteatoma
Post-tympanostomy tube otorrhea
Exostosis or osteoma
Psuedoepitheliomatous hyperplasia
Malignant
Squamous cell carcinoma
Adenoid cystic carcinoma
Staging Systems
Unfortunately, there are no universal staging systems for cancers of the external auditory canal, middle ear, and mastoid. This impedes comparison of studies between institutions and negatively affects treatment planning and prognosis for the individual patient. Three current systems are summarized in Table 2.2, 11, 42, 47, 60
Goodwin and Jesse18 in 1980 mentioned an early three-stage system derived from involvement of the external auditory canal, tympanic membrane, or extension to the middle
Evaluation
Accurate staging and treatment planning for temporal bone cancer is dependent on CT scanning assessment of the extent of bone erosion, whereas MR imaging provides better soft tissue assessment. This presumes that biopsy and clinical stagings of the ear, surrounding structures, and lymph nodes have been accomplished already. As noted previously, Arriaga et al3 found excellent correlation between CT scans and histologic specimens. Though Leonetti et al36 also found fairly good general correlation
Management
In this section, the discussion is directed primarily toward squamous cell carcinoma because it is the most common temporal bone tumor of epithelial origin. Specific comments on basal cell carcinoma and auricular squamous cell carcinoma with extension to the temporal bone are mentioned elsewhere.
Treatment Outcomes
The survival rates for many studies are summarized in Table 4. The trend has been for survival to increase from a rate of approximately 25% 50 years ago to a current rate of 50%. The survival rates of T1 and T2 carcinomas are 80% to 100%.46 The main controversy is whether a larger operation gives better results for advanced disease (T3 and T4 lesions). As with smaller lesions, there are advocates of a limited surgical procedure coupled with postoperative radiation for advanced disease.
Chao et al
Prognostic Factors
Several factors are associated with decreased survival rates, including extent of disease, facial nerve paralysis, positive margins, dural involvement, and lymph node involvement. Facial nerve paralysis is associated with decreased survival rates, as noted by several authors: in a study by Birzgalis et al,7 survival rate decreased from 46% to 24%, Liu et al's40 survival rate decreased from 56% to 10%, and Manolidis et al's42 study reported a decrease from 66% to 29%. Facial nerve involvement
Survey of Otologists
To give readers the most current information about controversies in the management of temporal bone squamous cell carcinoma, a survey was sent in early 2001 to 14 prominent otologists, many of whom have written articles cited in this article. Significant comments are quoted verbatim, without specific reference to author.
Question 1: For advanced carcinoma, T3–T4, do you ever perform a procedure that is less than a TTBR?
Response: Twelve otologists would do a piecemeal or subtotal temporal bone
Summary
Temporal bone malignancy is rare, with squamous cell carcinoma the most common cancer of the external auditory canal, middle ear, and mastoid. There is no gender predominance, and most cases occur in the fifth and sixth decades of life.
Otorrhea is the most common symptom of external auditory canal cancer. Diagnosis can be delayed because cancer mimics more common conditions of the external auditory canal, such as otitis externa.
There is no universally accepted staging system, but several
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Cited by (55)
Carcinomas of the external auditory canal: Management and results: A multicenter REFCOR propensity score matching study
2024, European Journal of CancerIndications for and extent of elective neck dissection for lymph node metastasis from external auditory canal carcinoma
2021, Auris Nasus LarynxCitation Excerpt :External auditory canal (EAC) carcinoma is considered rare, with an incidence of 1 in 1 million populations [1].
Clear cell variant squamous cell carcinoma of temporal bone: Case report and literature review
2018, International Journal of Surgery Case ReportsCitation Excerpt :Temporal bone malignancies are extremely rare, and SCC is the most common type of tumors to occur in the temporal bone. Temporal bone SCC is usually diagnosed late due to delayed presentation [4,11]. Our SCC case was aggressive in nature and presentation.
Temporal bone carcinoma: Classical prognostic variables revisited and modern clinico-pathological evidence
2016, Reports of Practical Oncology and RadiotherapyCitation Excerpt :A role has been hypothesized for chlorinated disinfectants in the etiopathogenesis of middle ear carcinoma and for human papillomavirus in cases of temporal bone squamous cell carcinoma (TBSCC) associated with inverted papilloma.6,7 TBSCC is usually diagnosed with delay1,4 since clinical signs (otorrhea, polyps or granulation tissue, hearing loss, bleeding) and patients complaints (ear pain) are similar to other common inflammatory diseases of the ear. When present, facial nerve paralysis is a sign of advanced disease.
Malignant tumors of the temporal bone – our experience
2016, Brazilian Journal of Otorhinolaryngology
Address reprint requests to, David M. Barrs, MD, 3404 Wake Forest Road, Suite 303, Raleigh, NC 27609