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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 25  |  Issue : 3  |  Page : 166-168

Osteosarcoma misdiagnosed as cholesteatoma


1 Department of Otorhinolaryngology, School of Health, Health Sciences Center, Mazandaran University of Medical Sciences, Sari, Iran
2 Department of Pathology, School of Medicine, Immunogenetics Research Center, Mazandaran University of Medical Sciences, Sari, Iran
3 Departments of Pathology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran

Date of Submission09-Jan-2019
Date of Acceptance26-Mar-2019
Date of Web Publication18-Oct-2019

Correspondence Address:
Dr. Maryam Ghasemi
Department of Pathology, School of Medicine, Immunogenetics Research Center, Mazandaran University of Medical Sciences, Sari
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_1_19

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  Abstract 


Osteosarcoma is a malignant neoplasm and the most common primary tumor of the bone. Craniofacial bones involvement is relatively rare, but if it occurs, most bone affected is the mandible and the maxillae. Osteosarcomas of extragnathic craniofacial bones are rare lesions, constituting fewer than 2% of all osteosarcomas. We report a rare case of de novo osteogenic sarcoma of the middle ear and mastoid and discuss the features associated with diagnosis and treatment.

Keywords: Cholesteatoma, de novo osteogenic sarcoma, middle ear, osteosarcoma


How to cite this article:
Nikkhah M, Ghasemi M, Poormoosa R, Sheidaei S, Akbari M. Osteosarcoma misdiagnosed as cholesteatoma. Indian J Otol 2019;25:166-8

How to cite this URL:
Nikkhah M, Ghasemi M, Poormoosa R, Sheidaei S, Akbari M. Osteosarcoma misdiagnosed as cholesteatoma. Indian J Otol [serial online] 2019 [cited 2019 Dec 15];25:166-8. Available from: http://www.indianjotol.org/text.asp?2019/25/3/166/269544




  Introduction Top


Sarcomas are malignant neoplasms originating from mesodermal tissues, which the neoplastic cells produce osteoid tissue. Sarcomas constitute <1% of body's tumors. Osteosarcomas are the most common primary malignant tumor of the bone.[1] The majority sites are the metaphyseal region of long bones of the limbs.[2] Osteosarcomas are relatively rare, account for approximately 1% or less of all head-and-neck cancers and represent <10% of all osteosarcomas.[2],[3] Here, we report a rare case of de novo osteogenic sarcoma of the middle ear and describe properties, symptoms, radiography and histopathology results, and therapeutic measures mentioned.


  Case Report Top


The patient is a 52-year-old male who had been referred to the educational and therapeutic center of Bu-Ali Sina (Sari) Hospital for the past 3 months with a tinnitus sign, nausea, earache, ear discharge, and facial nerve weakness. About 1 month before the referral, the patient had a tinnitus of the right ear, after which time nausea and difficulty in chewing were added to the patient's symptoms. With the severity of these symptoms and swelling in the head, the patient referred to this center for diagnosis and treatment.

According to the ear discharge and facial nerve weakness, the primary diagnosis was cholesteatoma. After the computed tomography scan was done [Figure 1], the patient underwent ear surgery. During surgery, middle ear and mastoid lesions with corrosion of the bone on the Dora and Dora's conflict were observed. The biopsy was done and the surgery ended. According to Dora's conflict, magnetic resonance imaging (MRI) of the brain and cervical soft tissue with and without Gadolinium injection were done [Figure 2] and [Figure 3]. The results indicated that there is heterogeneous ill-defined destructive enhancing mass involving the right mastoid extended auditory canal and periauricular soft tissue and caused temporal bone destruction extended to the right temporal lobe represent T1 hyperintensity. Right occipital condyle and jugular foramina have been involved by the mass.
Figure 1: Coronal computed tomography scan of the temporal bone

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Figure 2: Magnetic resonance imaging of the brain and cervical soft tissue with Gadolinium. Injection shows enhancing mass involving mastoid extended to external auditory canal, periauricular soft tissue, and temporal lobe

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Figure 3: Magnetic resonance imaging of the brain shows temporal lobe represent T1 hyperintensity

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The biopsy was taken and performed the histopathologic examination. By microscopy, a malignant neoplasm composed of sheets of atypical tumor cells producing osteoid was found [Figure 4]. By immunohistochemistry (IHC), tumoral cells were immunoreactive for vimentin and nonreactive for smooth muscle actin (SMA), S100, CK5/6, desmin, and myogenic markers. Final diagnosis was osteosarcoma and fibrohistiocytic type.
Figure 4: Hematoxylin and eosin slide shows atypical tumor cells with hyperchromatic nuclei, high nucleus: cytoplasm ratio and eosinophilic cytoplasm with mitotic figures and osteoid formation (400 × 0)

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The results of radiology and histopathology led to the diagnosis of de novo osteogenic sarcoma of the middle ear with the right side mastoid involvement that was rare between osteosarcomas of the craniofacial bones. The patient was referred to the radiotherapy and chemotherapy center for treatment.


  Discussion Top


Osteosarcoma is one of the primary skeletal malignant tumors that are determined by immature bone tissue formation by tumor cells.[4] This type of malignancy, which is rare (1 in 100,000 for each year), affects 6%–13% of craniofacial bones, and usually, secondary osteosarcomas occur in the skull bones.[5] Although the cause of osteosarcoma is still unclear, the only environmental factor affecting osteosarcoma in humans is ionization radiation. However, it is a rare complication of radiation therapy and usually occurs after a long latent period.[6] There is also strong evidence that indicates the possibility of an increasing incidence of osteosarcoma in genetic diseases such as hereditary retinoblastoma and Li–Fraumeni syndrome. Paget's disease, history of fibrous dysplasia, or trauma constitute is other predisposing features.[6] The present case appears to have developed de novo, as no history of any predisposing factors to be elicited from this patient.

In cases of large sarcomas, pathological fractures can occur. Sensory neural abnormalities may occur in cases where the lesion involves the course of peripheral nerves trismus can occur if mastication muscles or temporomandibular joint involvement.[7],[8] As Bielack et al. stated in her study, pain, and swelling in the affected area, could be the most common presenting symptom of osteosarcoma.[9] The pain was the presenting complaint in approximately 50% of the patients.[10] Here, the patient presented a history of pain and swelling in the head area. Despite what has been mentioned in Picci study, the patient did not provide any record of repeated exposure to ionization radiation.[4] In addition, nausea and difficulty in chewing were observed.

In contrary to the above findings, our patient presented with pain, nausea, tinnitus, ear discharge, and facial nerve weakness which are more in favor of chronic ear disease. Therefore, the primary physicians thought in favor of chronic media otitis and cholesteatoma.

Middle ear as the site of osteogenic sarcoma is a rare presentation. Due to the rarity, no comparative study exists. According to the literature, there are limited reports of detected osteosarcoma in the ear and often in mastoid. Brusić et al. reported a case of osteosarcoma of the mastoid. A 75-year-old female with a history of radiation therapy in 12 years ago, for the treatment of mucoepidermoid carcinoma of the parotid gland after total parotidectomy and radical neck dissection.[11] Hsieh et al. reported a case of parosteal osteosarcoma of the mastoid bone the following radiotherapy for nasopharyngeal carcinoma.[12]

The proposed treatment is often multistep method consisted primarily of surgery, preoperative chemotherapy, radical resection, and adjuvant treatment.[2],[3] Furthermore, Ferguson and Goorin reported that surgery is a vital factor in treating osteosarcoma and chemotherapy as a complementary therapy plays an important role in the control of subclinical metastatic disease.[13] They also suggested that the addition of radiation therapy allows better local control for the patients with complete surgical excision is not possible.[13] Kassir et al., in a meta-analysis study about the osteosarcoma in the head and neck, reported that surgery alone has the highest survival rate.[14] According to the Mendenhall et al. report, the prognosis of osteosarcoma patients was statistically better with the absence of paresthesia, smaller tumor size, younger age, adequacy of surgical removal, and a more differential histological grade of the lesion.[15] In our case, due to the spread of the tumor, the patient was referred to the radiotherapy and chemotherapy center for follow-up treatment.


  Conclusion Top


The present case appears to have developed de novo, as no history of any predisposing factors to be elicited from this patient. Our patient presented with pain, nausea, tinnitus, ear discharge, and facial nerve weakness which are more in favor of chronic ear disease and cholesteatoma. The patient underwent ear surgery. During surgery, middle ear and mastoid lesions and Dora's conflict were observed. The biopsy was done and the surgery was ended. According to the Dora's conflict, MRI of the brain and cervical soft tissue was done. Thus, de novo osteogenic sarcoma of the middle ear with right side mastoid was diagnosed. Then, the patient was referred to the radiotherapy and chemotherapy center for follow-up treatment.

Acknowledgment

The report adhered to the ethical principles outlined in the Declaration of Helsinki as amended in 2013. Written consent is obtained from the patient to access the details of the case and to publish the images and the results. The authors would like to thank the Clinical Research Development Unit of Bu-Ali Sina Hospital, Mazandaran University of Medical Sciences, Sari, Iran, for their support, cooperation, and assistance throughout the period of study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Amaral MB, Buchholz I, Freire-Maia B, Reher P, de Souza PE, Marigo Hde A, et al. Advanced osteosarcoma of the maxilla: A case report. Med Oral Patol Oral Cir Bucal 2008;13:E492-5.  Back to cited text no. 1
    
2.
Işikdogan A, Büyükçelik A, Erekul S, Pamir A. De novo osteogenic sarcoma of mastoid bone. Sarcoma 2002;6:79-81.  Back to cited text no. 2
    
3.
Wu G, Liang Q, Liu Y. Primary osteosarcoma of frontal bone: A case report and review of literature. Medicine (Baltimore) 2017;96:e9392.  Back to cited text no. 3
    
4.
Picci P. Osteosarcoma (osteogenic sarcoma). Orphanet J Rare Dis 2007;2:6.  Back to cited text no. 4
    
5.
Ha PK, Eisele DW, Frassica FJ, Zahurak ML, McCarthy EF. Osteosarcoma of the head and neck: A review of the Johns Hopkins experience. Laryngoscope 1999;109:964-9.  Back to cited text no. 5
    
6.
Ogunlewe MO, Ajayi OF, Adeyemo WL, Ladeinde AL, James O. Osteogenic sarcoma of the jaw bones: A single institution experience over a 21-year period. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:76-81.  Back to cited text no. 6
    
7.
Adekeye EO, Chau KK, Edwards MB, Williams HK. Osteosarcoma of the jaws – A series from Kaduna, Nigeria. Int J Oral Maxillofac Surg 1987;16:205-13.  Back to cited text no. 7
    
8.
Bianchi SD, Boccardi A. Radiological aspects of osteosarcoma of the jaws. Dentomaxillofac Radiol 1999;28:42-7.  Back to cited text no. 8
    
9.
Bielack S, Carrle D, Casali PG; ESMO Guidelines Working Group. Osteosarcoma: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2009;20 Suppl 4:137-9.  Back to cited text no. 9
    
10.
Nissanka EH, Amaratunge EA, Tilakaratne WM. Clinicopathological analysis of osteosarcoma of jaw bones. Oral Dis 2007;13:82-7.  Back to cited text no. 10
    
11.
Brusić SK, Pusić M, Cvjetković N, Karnjus R, Candrlić B, Kukuljan M, et al. Osteosarcoma of the mastoid process following radiation therapy of mucoepidermoid carcinoma of the parotid gland – A case report. Coll Antropol 2012;36 Suppl 2:223-5.  Back to cited text no. 11
    
12.
Hsieh ST, Guo YC, Tsai TL, Li WY, Lin CZ. Parosteal osteosarcoma of the mastoid bone following radiotherapy for nasopharyngeal carcinoma. J Chin Med Assoc 2004;67:314-6.  Back to cited text no. 12
    
13.
Ferguson WS, Goorin AM. Current treatment of osteosarcoma. Cancer Invest 2001;19:292-315.  Back to cited text no. 13
    
14.
Kassir RR, Rassekh CH, Kinsella JB, Segas J, Carrau RL, Hokanson JA. Osteosarcoma of the head and neck: Meta-analysis of nonrandomized studies. Laryngoscope 1997;107:56-61.  Back to cited text no. 14
    
15.
Mendenhall WM, Fernandes R, Werning JW, Vaysberg M, Malyapa RS, Mendenhall NP. Head and neck osteosarcoma. Am J Otolaryngol 2011;32:597-600.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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