Le synovialosarcome de la sphère oto-rhino-laryngée: une localisation rare: à propos de deux cas

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Abstract

La localisation ORL du synovialosarcome est rare représentant moins de 5% des tumeurs de la région. Sa prise en charge est multidisciplinaire reposant sur une chirurgie large et complète suivie d'une radiothérapie externe. Nous rapportons deux cas de synovialosarcome de l'oropharynx et du sinus maxillaire chez deux adultes jeunes traités par une chirurgie et une radiothérapie externe avec une bonne réponse locorégionale.

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Most cited references 12

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Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis.

Louis Guillou, Jean Benhattar, Françoise Bonichon … (2004)

To assess the prognostic value of SYT-SSX fusion type, in comparison with other factors, in a population of 165 patients with synovial sarcoma (SS). Data on 165 patients with SS (141 with localized disease at diagnosis) were studied retrospectively. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumor site (extremities v proximal/truncal), size, histology, mitotic count, necrosis, histologic grade (Federation Nationale des Centres de Lutte Contre le Cancer system), stage (1997 tumor-node-metastasis system classification), surgical margin status (assessed histologically), and fusion type (SYT-SSX1 v SYT-SSX2). Median follow-up time was 37 months (range, 2 to 302 months). Among those patients with localized disease at diagnosis, median and 5-year disease-specific survivals (DSS) for the SYT-SSX1 and SYT-SSX2 subgroups were 126 months and 67.4% versus 82 months and 63.2%, respectively (P = .12). Median and 5-year metastasis-free survivals (MFS) were 84 months and 54.2% for SYT-SSX1 versus 50 months and 47.6% for SYT-SSX2 (P = .76). Univariate analyses showed that high histologic grade (grade 3), high mitotic count (>/= 10 mitoses/10 high-power fields), stage III disease, size greater than 7 cm, tumor necrosis, and presence of areas of poorly differentiated morphology were significant adverse prognostic factors for DSS and MFS, whereas SYT-SSX fusion type, tumor histology (biphasic v monophasic), and patient sex were not. Age greater than 35 years adversely affected DSS but not MFS. In multivariate analyses, histologic grade was the most significant prognostic factor for both DSS and MFS. For patients with localized SS, histologic grade but not SYT-SSX fusion type is a strong predictor of survival.

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Sarcomas of the head and neck region.

Erich M. Sturgis, Wyatt B. Potter (2003)

This review discusses the classification, etiology, diagnosis, evaluation, treatment, and prognosis of sarcoma of the head and neck region. Sarcomas account for less than 1% of all malignancies in the United States with only 5 to 15% of these sarcomas occurring in the head and neck region. However, about 1 in 3 pediatric sarcomas will occur in the head and neck region. Occasionally, these tumors are associated with genetic syndromes or previous radiation exposures, but, most commonly, no clear etiology exists. Pathologic classification is critical to the ultimate treatment and prognosis of sarcoma of the head and neck. Osteosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, and angiosarcoma are the most common types of sarcoma to occur in the head and neck region; however, up to 20% of head and neck sarcomas will remain unclassified. Surgery has been central to the management of these malignancies with some exceptions in the pediatric population. Adjuvant chemotherapy is being utilized and/or studied for most high-grade sarcomas and adjuvant radiotherapy is important for disease control in high-grade soft-tissue sarcomas. Prognosis is clearly related to tumor grade and margin status. Sarcomas of the head and neck region are rare malignancies often without a clear etiology. Expert pathologic review and classification is critical, as are quality imaging and multidisciplinary management.

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Head and neck synovial cell sarcoma.

F R Amble, K D Olsen, A Nascimento … (1992)

Our experience with 14 patients treated for synovial cell sarcoma during the past 30 years is described. These tumors were manifested in young people whose ages ranged from 12 to 43 years. The diagnosis of synovial cell sarcoma proved difficult because approximately one third of the patients initially received incorrect pathologic diagnoses. A painless neck mass was the most common presenting symptom. Initial treatment included surgical excision in five patients and surgical excision and radiation therapy in nine. Recurrent or metastatic tumor occurred from 4 months to 62 months later; thus, long-term followup is important. Four of the nine patients who were followed more than 5 years died of their disease. Favorable prognostic findings included early diagnosis and the performance of wide surgical removal.

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Journal

Journal ID (nlm-ta): Pan Afr Med J

Journal ID (iso-abbrev): Pan Afr Med J

Journal ID (publisher-id): PAMJ

Title: The Pan African Medical Journal

Publisher: The African Field Epidemiology Network

ISSN (Electronic): 1937-8688

Publication date (Electronic): 12 March 2015

Publication date Collection: 2015

Volume: 20

Electronic Location Identifier: 232

Affiliations

[1 ]Service de Radiothérapie, Institut National d'Oncologie, Université Mohammed V, Rabat, Maroc

[2 ]Service d'Oncologie Médicale, Institut National d'Oncologie, Université Mohammed V, Rabat, Maroc

[3 ]Service d'Anatomopathologie, Hôpital des Spécialités, Université Mohammed V, Rabat, Maroc

Author notes

[& ]Corresponding author: Kouhen Fadila, Service de Radiothérapie, Institut National d'Oncologie, Université Mohammed V, Rabat, Maroc

Article

Publisher ID: PAMJ-20-232

DOI: 10.11604/pamj.2015.20.232.6225

PMC ID: 4482523

PubMed ID: 26140075

SO-VID: 39314915-8b55-44e3-a73a-8dab35c8a21d

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The Pan African Medical Journal - ISSN 1937-8688. 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.

History

Date received : 28 January 2015

Date accepted : 06 February 2015

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Le synovialosarcome de la sphère oto-rhino-laryngée: une localisation rare: à propos de deux cas Translated title: Synovialosarcoma of the ENT sphere: a rare localization: report of two cases

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Abstract

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Pan African Medical Journal

Most cited references 12

Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis.

Sarcomas of the head and neck region.

Head and neck synovial cell sarcoma.

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Cited by 1

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