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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 1  |  Issue : 2  |  Page : 26-28

Diagnostic dilemma in a case of temporal bone carcinoma in a young man


1 Department of ENT, NEIGRIHMS, Shillong, Meghalaya, India
2 Department of Radiodiagnosis, NEIGRIHMS, Shillong, Meghalaya, India

Date of Web Publication26-Sep-2018

Correspondence Address:
Dr. Deepanava Jyoti Das
Department of ENT, NEIGRIHMS, Shillong, Meghalaya
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aiao.aiao_5_17

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  Abstract 


Malignancy of temporal bone is a rare entity with an incidence of <0.2% of all head-and-neck cancer with an average age of occurrence at 60 years. We present here a case of a 21-year-old boy with temporal bone cancer who presented with clinical features of complicated chronic suppurative otitis media, radiologically as chronic osteomyelitis creating a diagnostic dilemma as there was no predisposing factor for osteomyelitis. The diagnosis was confirmed by open biopsy which revealed squamous cell carcinoma. The best survival rate is obtained with total temporal bone resection and post-operative radiotherapy.

Keywords: Cancer temporal region, diagnostic dilemma, young age


How to cite this article:
Das DJ, Bhatia A, Phukan P, Baruah C. Diagnostic dilemma in a case of temporal bone carcinoma in a young man. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2017;1:26-8

How to cite this URL:
Das DJ, Bhatia A, Phukan P, Baruah C. Diagnostic dilemma in a case of temporal bone carcinoma in a young man. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2017 [cited 2018 Nov 18];1:26-8. Available from: http://www.aiaohns.in/text.asp?2017/1/2/26/242235




  Introduction Top


Malignancy of temporal bone is a rare entity. Incidence is about 0.2% of all head-and-neck cancer.[1] Despite the surgical and radiographic advancements, the prognosis remains guarded for extensive lesions. Early diagnosis is rare and often coexists with chronic otitis media. Age of occurrence is 34–85 years with an average age of 60 years.[2] Occasional cases with earlier presentation have been reported.[3],[4] Some cases have been reported to have occurred in childhood.[5] We report a case of squamous cell carcinoma occurring in early adulthood and the diagnostic dilemma we encountered.


  Case Report Top


A 21-year-old boy presented to the Department of ENT, NEIGRIHMS with complaints of deviation of mouth to the right for the last 1 month with a history of intermittent discharge from the left ear since childhood that had increased for the past month. The discharge was foul smelling, bloodstained, with hearing loss, and pain in the post-aural region.

On examination, the patient had Grade 5/6 facial nerve palsy of the left side (house Brockmann classification).[6] The post-aural region was swollen, tender with the local rise of temperature. Otoscopy revealed foul smelling, nonblood stained purulent discharge. Examination under microscope revealed purulent discharge with an eroded posterior bony wall of the external auditory canal. The skin was friable and bled briskly on touch. The tympanic membrane could not be visualized. Pus swab was sent for bacterial staining, culture, and sensitivity. Provisionally, it was diagnosed with a case of complicated chronic suppurative otitis media.

Computed tomography (CT) scan of the temporal bone showed soft-tissue density within the left mastoid air cells, external auditory canal, the middle ear with the erosion of the ossicles and posterior fossa with the extensive permeative destruction of the occipital bone and relative sparing of the petrous apex [Figure 1] and [Figure 2].
Figure 1: Coronal section of the NCCT showing permeative destruction of the occipital condyle on the left side

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Figure 2: Axial section of the NCCT showing permeative bone destruction of the inner and outer table of the squamous part mastoid and occipital bone

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A differential diagnosis of complicated CSOM and chronic osteomyelitis was given.

The patient was admitted started on intravenous ciprofloxacin given suspicion of skull base osteomyelitis. Routine blood investigations were normal. There was no evidence of any immunocompromised condition. Pus culture revealed methicillin-resistant Staphylococcus aureus sensitive to ciprofloxacin, and hence, intravenous ciprofloxacin was continued. Neurosurgery opinion was sought in view of suspected skull base osteomyelitis, advised an Contrast-enhanced magnetic resonance imaging (MRI) which revealed enhancing collection in the left middle ear cavity with extension into the left mastoid air cells, petrous apex, destruction of ossicles, scutum, and mastoid air cells with surrounding soft-tissue inflammatory change and adjacent pachymeningitis of the tentorium. The left parotid gland was also involved [Figure 3].
Figure 3: An enhancing soft tissue with minimal peripherally enhancing collection seen within the left mastoid cavity, middle ear and external auditory canal, extending to involve the left parotid gland

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Features were suggestive of infective etiology. However, given the suggestive clinical picture, malignant growth was also considered in the differential diagnosis, along with skull base osteomyelitis and complicated chronic otitis media.

The patient was planned for open biopsy given lack of adequate visualization of the lesion. Intraoperatively, the posterior wall of the EAC was eroded with growth extending up to the epitympanum. The mastoid region was also infiltrated by growth. Histopathology report suggested moderately differentiated squamous cell carcinoma.


  Discussion Top


Squamous cell carcinoma of the temporal region is a rare malignancy with considerable morbidity and mortality. Despite the diagnostic advances and therapeutic refinement, these malignancies still bear an ominous prognosis.

Squamous cell carcinoma accounts for 60% to 80% of all temporal bone malignancies followed by basal cell carcinoma.[7] The disease affects men and women equally, with an age range of 34–85 years and a mean age of 60 years.[2] The occurrence of squamous cell carcinoma of the temporal bone at the age of 18–20 years has been reported.[3] A 12-year-old child with syndactyly and deaf-mutism with squamous cell cancer of temporal bone has been reported.[5] Another article mentions cases occurring at the age of 8 years, but a published case report of this case could not be found.[8] The rarity of the disease limits the performance of large risk assessment studies. Hence, the etiology is not well defined. Chronic otitis media is frequently argued a risk factor for temporal bone malignancy, especially squamous cell carcinoma.[7]

The patient presented with a clinical picture of CSOM with facial nerve palsy. CT scan and MRI were suggestive of infective pathology involving a large region usually seen in skull base osteomyelitis. However, investigations did not reveal any immunocompromised state, thereby leaving the diagnosis of skull base osteomyelitis in doubt. Inability to satisfactorily examine the external auditory canal due to edema and tendency of the tissue to bleed on touch preempted a reliable diagnosis. The final resort was an open biopsy which revealed squamous cell carcinoma.

The early symptoms of temporal bone carcinoma closely resemble those of CSOM, including purulent discharge, bleeding, and also they often coexist.[7] High-resolution CT provides detail of invasion into bony structures but cannot distinguish mucosal inflammation from tumor. This is best differentiated by MRI scan, which could also show the evidence of a tumor without bony erosion and tumor with dural and brain involvement. Ongoing secondary infection in a tumorous lesion, both will show enhancement on CT and MRI and restricted diffusion on MRI making it difficult to distinguish on imaging. In this case, there is a collection with minimal soft tissue on MRI which appeared like an abscess.[9]

In patients with carcinoma that is confined to the external auditory canal have similar survival, regardless of whether mastoidectomy, lateral temporal bone resection, or subtotal temporal bone resection is performed. Postoperative radiotherapy does not appear to improve survival. Survival of patients treated with subtotal temporal bone resection appears to improve compared to those treated with lateral resection or mastoidectomy when middle ear is involved. Adjunctive radiotherapy has uncertain value. The results of surgical resection with petrous apex involvement are bad. Resection of the involved dura, involved brain parenchyma or internal carotid artery leads to limited or no clinical improvement. The best survival rate is obtained with total temporal bone resection and postoperative radiotherapy. However, this technique cannot be used for all the patients.[10]

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Antonio SM, Hirsch BE. Malignant Tumours of the Temporal Bone. New York (NY): WebMD LLC; 25 February, 2016. Available from: http://www.emedicine.medscape.com/article/ 845777-overview#showall [Last accessed on 2017 Jul 21].  Back to cited text no. 1
    
2.
Michaels L, Soucek S, Beale T. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon: World Health Organization; 2005. p. 349.  Back to cited text no. 2
    
3.
Deka RC. Middle-ear carcinoma in a girl of eighteen. J Laryngol Otol 1980;94:429-31.  Back to cited text no. 3
    
4.
Lasisi O, Ogunleye A, Akang E. Squamous cell carcinoma of the mastoid – A report of two cases. Ghana Med J 2005;39:28-32.  Back to cited text no. 4
    
5.
Pippal S, Arora S, Yadav A, Singhal D. A rare association of deaf mutism, syndactyly and squamous cell carcinoma of the temporal bone. Internet J Otolaryngol 2006;7:12-6.  Back to cited text no. 5
    
6.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146-7.  Back to cited text no. 6
    
7.
Gustafson ML, Pensak ML. Surgery for malignant lesions. Glasscock-Shambaugh Surgery of the Ear. 5th ed., Cha. 37. Hamilton (ON): Decker, BC; 2003. p. 743-54.  Back to cited text no. 7
    
8.
Agada FO, Gnananandha C, Wickham M. Squamous cell carcinoma of the middle ear: Case report and literature review. Ann Afr Med 2004;3:90-2.  Back to cited text no. 8
    
9.
Attakil A, Thorawade V, Jagade M, Kar R, Parelkar K, Khairnar P, et al. Squamous cell carcinoma of the middle ear mimicking CSOM with intracranial complications: A diagnostic dilemma. Int J otolaryngol 2014;3:376-81.  Back to cited text no. 9
    
10.
Weiss MD, Kattah JC, Jones R, Manz HJ. Isolated facial nerve palsy from metastasis to the temporal bone: Report of two cases and a review of the literature. Am J Clin Oncol 1997;20:19-23.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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