Webmedcentral
WebmedCentral Oncology
2046-1690
WebmedCentral
1659
case-report
Oncology
Primary Synovial Sarcoma of the Inner Ear: A Case Report
Meryem
Glaoui
Resident,Department of Medical Oncology in the National Institute of Oncology,Morocco
Ikhoyaali
Siham
Resident,National Institute of Oncology , Medical Oncology,Morocco
Ait l haj
Meryem
Resident,National Institute of Oncology ,Morocco
Hamza
Faraj
Resident,National Institute of Oncology, Radiology ,Morocco
Mohamed
Mesmoudi
Medical Doctor,Medical Oncology, National Institute of Oncology,Morocco
Hassan
Errihani
Ph D,National Institute of Oncology, Medical Oncology,Morocco
* E-mail:m_glaoui@hotmail.com
Competing Interests:
Ruthors declares no competing interests
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Copyright: © 2011 Webmed Ltd
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This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Ear synovial sarcoma is thus rare, to our knowledge, there has been only one reported case in English medical literature. Here we report a second case of a locally advanced synovial sarcoma affecting the inner ear in a thirty-year-old female managed by chemotherapy and radiotherapy. The objective of the present paper was to describe and discuss the diagnosis and treatment of this rare malignant inner-ear tumor.
Introduction
Malignant tumor of the ear is infrequent, with an incidence of 1 per six million of the population. Whatever the histological type, an inner ear location is exceptional 1 .Diagnosis should not be delayed, so as to allow satisfactory tumor removal without risk of neurologic sequeles.
Case Report
A 30-year-old female presented in the medical oncology department of national institute of oncology with a painful contracture of facial muscles with a six months history of otorrhea associated with intermittent otalgia. Physical examination revealed a budding tumor filling the external auditory canal associated with complete peripheral right facial nerve palsy. Computed tomography scan of the face was performed and confirmed the presence of a 56*37 mass extending to the tympanum and external auditory canal with osteolysis of the mastoid [ figure 1,2] , there was no evidence of metastasis to lung or other sites.A biopsy was performed and the diagnosis of a monophoasic synovial sarcoma FNCLCC grade II was rendered .Histological analysis of the tumor revealed a massive infiltration by an elongated cells with spindleshaped nuclei. The reticulin pattern was heavy with prominent vascularity and some dilated vascular channels. There were several foci of distinct cartilaginous differentiation within the tumour. Immunohistochemical study revealed a positive reaction for EMA and PSA 100 but negative for CD34, HMB45, KL1 and desmine. A final diagnosis of monophoasic synovial sarcoma FNCLCC grade II was made.Surgical approach wasnât possible because of the extending of the tumor and the patient underwent three cycles of neo adjuvant chemotherapy consisting of doxorubicin (60mg/m2day1) and ifosfamide (3g/m2 day 1 to 3) regimen.CT scan after final courses showed a progressive disease [ figure 3] with skin ulceration, local bleeding and lung metastasis. A palliative radiotherapy was indicated followed by oralCyclophosphamide. The patient died after 6 months.
Discussion
Although rare, synovial sarcoma is one of the most common malignantsoft-tissue sarcomas in children and adolescents, the headand neck is involved in 6.8% of all synovial sarcomas2.Ear location is extremely rare , only one case of a primary synovial sarcoma of the middle ear has been reported by O'keefee et al 3 in 1993 .Synovial sarcomas often arise in areas remote from structures containing synovial membrane such as joints or bursae. Mackenzie et al suggested that synovial sarcomas arise from undifferentiated mesenchymal tissue which retains the potential for synovioblastic differentiation .Immunohistochemical studies by Abenoza et al support the theory of mesenchymal origin demonstrating a positive immunoreaction for the epithelial markers, epithelial membrane antigen [EMA] and cytokeratin [CK]The tumour is classically biphasic, containing both epithelial and spindle cells. The biphasic nature of the tumour is the only diagnostic histological criterion although a monophasic variety where either cell type predominates is recognized as in this case.Treatment of ear tumor depends on the degree of extension [4]. Classically, it associates a complete surgical resection of the primary tumor and/or radiochemotherapy. Surgeryshould follow the rules of cancer surgery rather than the general principles of ear surgery [5].removal is incomplete in 50% of cases, accounting for the high rate of local recurrence at 10 months [4]. Prognosis is generally poor but depends on the time to diagnosis [6].
Conclusion
Inner-ear synovial sarcoma is a rare malignant tumor that requires early diagnosis to allowa complete surgical resection which is the mainstay of TreatmentA multidisciplinary approach associating otologist ,surgeons,radiologist and oncologist is essential for efficient management.
References
1.Alvarez CN, DA Silva CV, Luisi A. Carcinomes primitifs de lâoreille externe et de lâoreille moyenne. Ann Otolaryngol 1981;98:613â92.McCarville MB, Spunt SL, Skapek SX, Pappo AS. Synovial sarcoma in pediatric patients. AJR Am J Roentgenol2002; 179 :797 â8013.O'keei .L, Ramsden .R and Birzgalis.A .Primary synovial sarcoma of the middle ear. The Journal of Laryngology & Otology (1993), 107: 1070-10724.Depondt J, Bouccara D, Enaux M, et al. Tumeurs malignes du rocher : à propos de 25 cas. Ann Otolaryngol Chir Cervicofac 1995;112:309â16.5.Moffat DA, Wagstaff SA, Hardy DG. The outcome of radical surgery and postoperative radiotherapy for squamous cell car- cinoma of the temporal bone. Laryngoscope 2005;115:341â7.6.Yeung P, Bridger A, Smee R. Malignancies of the external auditory canal and temporal bone: a review. ANZ J Surg 2002;72:114â20.
Citation: Glaoui M , Siham Ik , Meryem Ai , Faraj Ha , Mesmoudi Mo , Errihani Ha .Primary Synovial Sarcoma of the Inner Ear: A Case Report . WebmedCentral ONCOLOGY 2011;2(3):WMC001659
Wafaa
Kaikani
Dr, Medical Oncology, National Institute of Oncology, , Morocco
Primary Synovial Sarcoma of the Inner Ear: A Case Report
ligne 15 : the presence of a 56*37 mm mass extending..
ligne 24 : preoperative chemotherapy rather than neoadjuvant chemotherapy
ligne 33:Â Synovial sarcomas often arises
ligne 41: O'keefee et al in 1993 (3)Â
ligne 43:  Mackenzie et al  suggested .. reference ??
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Concerning imaging: is MRI better ??
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Youssef
Bensouda
Medical Doctor, Medical Oncology, Huceima, Centre regional d\'oncologie, Huceima, Morocco
Synovial sarcoma ORL
Nice and original artcile,
I join the comments made by the other authors
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Esther
Una Cidon
Oncology, Oncology Department; Clinical University Hospital and Faculty of Medicine , , Spain
A very rare clinical entity: Sarcoma of the year
I think this is a very interesting article. It shows us a very rare clinical case of a primary synovial sarcoma arising of the inner ear. The communication of these kind of rare clinical cases is understandable from the point of view of increasing the doctors´ awareness about the relevance of expedite diagnosis and treatment and also to increase the knowledge of these diseases to make doctors suspect about their existence. Despite of the comments above, I think this article could be somewhat improved. Sometimes it needs a language polishment.
In the introduction the authors should include more data about the synovial sarcomas in general, frequency, most usual locations and therapeutic approaches. Then, authors could focus (as they have done) on the ear tumours and the relevance of early diagnosis and treatment to get the best results.
In the section âCase reportâ, authors should explain the abbreviation they have used FNCLCC (French Federation of Cancer Centers Sarcoma Group).
I would like to have seen if this case was treated in a multidisciplinary committee and if radiation therapy was considered as neoadjuvant treatment as chemotherapy was.
I miss some pictures about histology.
I would like also to know why this patient had received oral cyclophosphamide as palliative treatment once she had got progressive disease with the combination of ifosfamide and doxorubicin and for how long was she receiving this treatment and also the evolution she presented (stable disease, partial remission or progressive disease). On the other hand the dosis of palliative radiotherapy and the clinical benefits she got from this technique.
I think all these points would improve our knowledge about this rare disease.
In the end the references need to be reviewed (there is a mistake in reference 3).
Esther Una Cidon, MD, PhD, Professor
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Thomas
Heston
Physician, Internet Medical Association, 848 N Rainbow Blvd #1289, United States of America
Review of a Case Report on Primary Synovial Sarcoma of the Inner Ear
Thank you for this interesting case report.
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I was only able to see the CT image before treatment, not afterwards. Also, do you have an MRI? Do you have a PET/CT image? In your description of the CT, please give units of measurement.
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Some of the abbreviations you used were unfamiliar to me. Could you please give the full description instead of just abbreviations?
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Eman
El-Abd
Molecular Oncology & Nuclear Medicine, Medical Research Institute, Radiation Science Department, Alexandria University, Egypt
Primary Synovial Sarcoma of the Inner Ear: A Case Report
I suggest to:
refere to the grading system that was used
add the illustrations for the IHC for EMA, PSA100, CD34, HMB45, KL1, and desmine with the proper positive and negative controls
add the scoring system for these markers and illustrate why they have been used (e.g. desmine as myoepithelial marker, KL1 as epithelial marker .....etc)
Explain why diagnosis was not confirmed by molecular markers
Follow consistent method in writing refereces
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Narendra
Hulikal
Associate Professor, Surgical Oncology, Kasturba Medical College, Manipal University, , India
Primary Synovial sarcoma of the inner ear?!
I wish authors should elaborate on certain issues and also calrify few of them. Can they clarify the exact site of origin of the tumor as it appears clinically as well as on CT films to be involving external and middle ear, mastoid and temperomandibular area and not inner ear. There are case reports of the tumor arising from temperomandibular region (atleast 2). Authors should have provided illustration of microscopic findings. It is difficult to diagnose these tumors in small specimens even by experienced sarcoma pathologist. At present time diagnosis of monphasic synovial sarcoma needs demonstration of X;18 translocation which is characteristic of these tumors. Extensive articles on synovial sarcoma of head and neck and orbit can be found.
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Juan
Yakisich
Assistant Professor, Department of Clinical Neuroscience, Karolinska Institute, Department of Clinical Neuroscience R54, Sweden
Primary Synovial Sarcoma of the Inner Ear: A Case Report
This manuscript reports a rare case of a primary case of synovial sarcoma localized in the middle ear.
The manuscript may need the addition of the histological analysis.
Minor corrections are also needed (e.g. consistency in the references).
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