SCImago Journal & Country Rank
This journal is a member of, and subscribes to the principles of, the Committee on Publication Ethics (COPE)
[ Abstract ] [ PDF ] [ Similar Articles ] [ E-Mail to Author ] [ E-Mail to Editor ]
DOI: 10.5146/tjpath.2018.01449
Pediatric Tonsillar Synovial Sarcoma- Very Rare Localization: A Case Report and Review of the Literature
Koray YALÇIN1, Gülen TÜYSÜZ1, Mine GENÇ2, İrem Hicran ÖZBUDAK3, Alper Tunga DERİN4, Kamil KARAALİ5, Alphan KÜPESİZ1, Elif GÜLER1
1Department of Pediatric Hematology and Oncology, Akdeniz University Medicine Faculty, ANTALYA, TURKEY
2Department of Radiation Oncology, Akdeniz University Medicine Faculty, ANTALYA, TURKEY
3Department of Pathology, Akdeniz University Medicine Faculty, ANTALYA, TURKEY
4Department of Nose Throat Ear Surgery, Akdeniz University Medicine Faculty, ANTALYA, TURKEY
5Department of Radiology, Akdeniz University Medicine Faculty, ANTALYA, TURKEY
Keywords: Synovial Sarcoma, Tonsillar neoplasm, Pediatric tumor , Dysphagia, Snoring
Abstract
Tonsillar synovial sarcoma is an extremely rare entity and only 9 adult patients have been reported up to now. Here, we describe the first pediatric tonsillar synovial sarcoma of the literature in a patient who presented with a 2-month history of dysphagia and snoring. Clinical and radiological examinations showed that the tumor arose from the right palatine tonsil and narrowed the parapharyngeal space. An incisional biopsy from the palatine tonsil revealed the diagnosis of synovial sarcoma. The patient has underwent total tonsillectomy and received radiotherapy and chemotherapy because of the positive surgical margins. The patient is clinically in good condition and free of tumor 30 months after the initial diagnosis. We achieved a long-term complete remission with a combination of surgery, radiotherapy and chemotherapy in our case. Tonsillar synovial sarcoma should be kept in mind while dealing with tonsillar masses. We can conclude that a multidisciplinary approach is warranted while treating synovial sarcoma with this localization.
Introduction
Synovial sarcoma (SS) is the most common soft tissue sarcoma after rhabdomyosarcoma in the pediatric population. The annual incidence rate is 0.5-07 /million in children and adolescents younger than 20 years of age 1,2. Synovial sarcoma primarily arises from deep soft tissues of the extremities, usually from the lower extremities followed by the upper extremities, trunk, and retroperitoneal/ abdominal region. Head and neck localization is rare with a percentage of only 3-10% of all SS cases 3,4. The most frequent areas in the head and neck region include the hypopharynx, followed by the parapharyngeal space and post pharyngeal area. Primary SS of the palatine tonsil is extremely rare and only 9 adult cases have been reported so far 5-11. As far as we are aware, we report the first pediatric case of tonsillar SS in the literature. Because of the unusual localization, the management and treatment of tonsillar SS is based on case reports 5-11. It is known that both the treatment and prognosis of SS differs among children and adults 2,12. We therefore believe this report may contribute to the existing literature with its rare location and treatment approach.
  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Case Presentation
    A 13-year-old boy presented with a 2-month history of dysphagia and snoring. His physical examination revealed an ulcerative, green colored mass in the right tonsil which narrowed the oropharynx. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 50x47x45 mm solitary mass originating from the right lateral oropharynx, extending to the hypopharynx and narrowing the oropharyngeal lumen irregularly (Figure 1,2). The Positron Emission Tomography (PET) scan showed metabolic activity (SUV max: 9.3) in the right tonsillar area. There was no evidence of distant metastases or regional lymph node involvement in the PET scan. To make a diagnosis, an incisional biopsy was performed from the right tonsil and the histopathological examination of the specimen revealed the presence of a biphasic SS. Immunohistochemical examination for PanCK, CK19, CK18, CK7 revealed positive staining in the epithelial component and staining for vimentin revealed positive staining in the mesenchymal component. The Hematoxylin-Eosin staining of tumor is illustrated in Figure 3 and the CK7 immunopositivity of the epithelial cells is illustrated in Figure 4. Muscle and neural markers were negative. The Ki67 proliferation index was 50%. The diagnosis was confirmed by additional cytogenetic analysis, which revealed the presence of SYTSSX1, a specific fusion gene for synovial sarcoma in the tumor cells.


    Click Here to Zoom
    Figure 1: T2-weighted axial MR image shows hyperintense mass lesion within the right parapharyngeal area (long arrows). Maximum diameters in this plane are 24 x 38 mm. The mass is displacing the right submandibular gland (short arrow) but there is no invasion.


    Click Here to Zoom
    Figure 2: T1-weighted post contrast image shows diffuse homogeneous enhancement of the mass lesion (arrows).


    Click Here to Zoom
    Figure 3: Biphasic architecture of neoplasm composed of spindle cells and epithelial cells (H&E; 200).


    Click Here to Zoom
    Figure 4: CK7 positivity in neoplastic epithelial cells (IHC; x100).

    The patient was staged according to IRS staging as clinical group 3 and chemotherapy was initiated with vincristine (1,5 mg/m2; weekly), ifosfamide (3 g/m2; 1 in 3 weeks), and doxorubicin (30 mg/m2; 1 in 3 weeks) according to the treatment schema for the Pediatric Oncology Group trial 9553 13. The tumor remarkably regressed on the 10th week of treatment. The patient had undergone total tumor excision but a total of 6000 cGy conventional radiotherapy was administered to the primary tumor site in 30 fractions due to the positive surgical margins. Chemotherapy was completed at 23 weeks according to the protocol. At the last follow-up 30 months after the initial diagnosis, the patient was still in complete remission with a 100 Karnofsky performance score.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Discussion
    Synovial sarcoma is often associated with joints such as knee, but it can also arise from pluripotential mesenchymal stromal cells anywhere in the body 8. Histologically this tumor is classified based on epithelial and spindle cell components in two subtypes; monophasic and biphasic. Synovial Sarcoma rarely occurs in the head and neck area but the parapharyngeal space is the most commonly involved location for this region when it does. The monophasic subtype of SS is the most common histological pattern in the head and neck area 3,14,15.

    Primary SS of the tonsil is extremely rare and only nine cases have been reported in the English literature up to now 5-11 (Table I). All of these patients are adults (> 18 ages) with male gender. Our case is the youngest case and the first pediatric patient. Previous studies reported the dominancy of monophasic spindle cell variant for head and neck SS 3,14,15 but 7 of the reported tonsillar SS patients and ours had the biphasic subtype.

    More than 90% of all SS show a reciprocal translocation, t (X: 18) (p11:q11), with a resultant fusion gene of SYT and one of three SSX genes. It is considered a molecular hallmark for the diagnosis of SS in difficult cases 16. Three of the cases previously reported and ours had this diagnostic translocation.

    Complete surgical excision with a wide margin is the mainstay of the treatment in SS. In cases with positive surgical margins, a high incidence of local recurrence (60%- 90%) within 2 years has been reported 17. Radiotherapy is strongly recommended to prevent local recurrences in patients with microscopic residual disease 1-3,17. The role of chemotherapy in adults and children with SS remains controversial. Synovial sarcoma in adults generally has been considered a less chemosensitive tumor and patients have been treated with surgery and radiotherapy 18,19. In contrast, synovial sarcoma in pediatric patients is considered to be more chemosensitive than in adults and these patients have been treated according to rhabdomyosarcoma chemotherapy protocols. The chemotherapy response rate has been estimated to be 60-65% in children. Many drugs are active on this tumor, but the most commonly used regimens include doxorubicin and/or ifosfamide 2,20,21.

    It has been shown that a large tumor size (≥5 cm in diameter), presence of distant metastasis, gross residual disease, and omission of radiotherapy are all significantly associated with poor survival 1. Age is described as an independent prognostic factor for survival of localized SS. The 5-year overall survival has been reported as 89% in children and 43% in adults 22.

    Head and neck localization of SS is rare and the treatment approach to tonsillar SS is based on case reports. There are nine case reports in the literature (Table I). All of the reported patients are adults and they were treated according to adult protocols. Six of the previously reported cases had initial surgery and the tumor was totally removed 5-11. Radiotherapy was applied to 2 of these patients and chemotherapy was administered to one of them. All of these patients were in complete remission within a median of 3.8 years of follow up when they were published as case reports. Two cases reported by Khademi et al. 11 had no chance for total excision because of an extended tumor. They underwent debulking surgery and then received radiotherapy and chemotherapy respectively. They both had local recurrence; one of them died because of progressive disease, and the other had progressive disease despite salvage treatment. Our case also presented with a large and extensive tumor. After biopsy, he first received neoadjuvant chemotherapy and underwent surgery when the tumor regressed. Radiotherapy was applied for microscopic residue and chemotherapy was continued up to 23 weeks according to the protocol 13.


    Click Here to Zoom
    Table I: The review of previous patients with Tonsillar Synovial Sarcoma.

    Although there are limited data about tonsillar synovial sarcoma in the literature, it is obvious that total tumor excision is the most crucial part of the treatment. Neoadjuvant chemotherapy is recommended for all patients with SS with a head and neck localization who have tumors > 5 cm in size, extensive or recurrent disease, and high risk localization, such as the skull base or paraspinal area 3,14,15,23. Pediatric oncologists have changed the treatment of SS depending on the patient’s risk stratification. Patients with macroscopic disease, N1 tumor and axial localization (head-neck, trunk, lung-pleura, retro peritoneum) are classified as a high risk group regardless of any other clinical parameters. These patients are now more prone to be treated with neoadjuvant chemotherapy, delayed surgery and radiotherapy 20. It has however been suggested that adjuvant chemotherapy could be omitted for low-risk pediatric patients 20,24.

    In conclusion, a review of the literature shows that surgery could be sufficient for low risk and totally excisable tonsillar SS cases. However, we believe that high risk patients such as our case deserve multimodal therapy. The treatment approach should include a multidisciplinary team consisting of a head and neck surgeon, radiation oncologist and pediatric oncologist to ensure full recovery in these patients.

    FUNDING
    This study did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

    CONFLICT of INTEREST
    The authors declare no conflict of interest.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • References

    1) Okcu MF, Munsell M, Treuner J, Mattke A, Pappo A, Cain A, Ferrari A, Casanova M, Ozkan A, Raney B. Synovial sarcoma of childhood and adolescence: A multicenter, multivariate analysis of outcome. J Clin Oncol. 2003;21:1602-11.

    2) Ferrari A, Gronchi A, Casanova M, Meazza C, Gandola L, Collini P, Lozza L, Bertulli R, Olmi P, Casali PG. Synovial sarcoma: A retrospective analysis of 271 patients of all ages treated at a single institution. Cancer. 2004;101:627-34.

    3) Mallen-St Clair J, Arshi A, Abemayor E, St John M. Factors associated with survival in patients with synovial cell sarcoma of the head and neck: An analysis of 167 cases using the SEER (Surveillance, Epidemiology, and End Results) Database. JAMA Otolaryngol Head Neck Surg. 2016;142:576-83.

    4) Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15:239-52.

    5) Rangheard AS, Vanel D, Viala J, Schwaab G, Casiraghi O, Sigal R. Synovial sarcomas of the head and neck: CT and MR imaging findings of eight patients. AJNR Am J Neuroradiol. 2001;22:851-7.

    6) Soria-Cespedes D, Galván-Linares AI, Oros-Ovalle C, Gaitan- Gaona F, Ortiz-Hidalgo C. Primary monophasic synovial sarcoma of the tonsil: Immunohistochemical and molecular study of a case and review of the literature Head Neck Pathol. 2013;7:400-3.

    7) Vogel U, Wehrmann M, Eichhorn W, Bültmann B, Stiegler M, Wagner W. Molecular and clinicopathological findings in a tonsillar synovial sarcoma. A case study and review of the literature. Head Neck Pathol. 2010;4:257-60.

    8) Ishiki H, Miyajima C, Nakao K, Asakage T, Sugasawa M, Motoi T. Synovial sarcoma of the head and neck: Rare case of cervical metastasis. Head Neck. 2009;31:131-5.

    9) Engelhardt J, Leafstedt SW. Synovial sarcoma of tonsil and tongue base. South Med J. 1983;76:243-4.

    10) Shmookler BM, Enzinger FM, Brannon RB.Orofacial synovial sarcoma: A clinicopathologic study of 11 new cases and review of the literature. Cancer. 1982;50:269-76.

    11) Khademi B, Bahranifard H, Mohammadianpanah M, Ashraf MJ, Azarpira N, Dehghani M. Synovial Sarcoma of the Palatine Tonsil: Report of two cases and review of the literature. Middle East Journal of Cancer. 2010;1:141-6.

    12) van der Graaf WT, Orbach D, Judson IR, Ferrari A. Soft tissue sarcomas in adolescents and young adults: A comparison with their paediatric and adult counterparts. Lancet Oncol. 2017;18:166-75.

    13) Pappo AS, Devidas M, Jenkins J, Rao B, Marcus R, Thomas P, Gebhardt M, Pratt C, Grier HE. Phase II trial of neoadjuvant vincristine, ifosfamide, and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: A Pediatric Oncology Group Study. J Clin Oncol. 2005;23:4031-8.

    14) Harb WJ, Luna MA, Patel SR, Ballo MT, Roberts DB, Sturgis EM. Survival in patients with synovial sarcoma of the head and neck: Association with tumor location, size and extension. Head Neck. 2007;29:731-40.

    15) Crowson MG, Lalich I, Keeney MG, Garcia JJ, Price DL. Clinicopathologic factors and adjuvant treatment effects on survival in adult head and neck synovial cell sarcoma. Head Neck. 2015;37:375-80.

    16) Kawai A Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. 1998;338:153-60.

    17) Carrillo R, Rodriguez-Peralto JL, Batsakis JG. Synovial sarcomas of the head and neck. Ann Otol Rhinol Laryngol. 1992;101:367-70.

    18) Trassard M, Le Doussal V, Hacène K, Terrier P, Ranchère D, Guillou L, Fiche M, Collin F, Vilain MO, Bertrand G, Jacquemier J, Sastre-Garau X, Bui NB, Bonichon F, Coindre JM. Prognostic factors in localized primary synovial sarcoma: A multicenter study of 128 adult patients. J Clin Oncol. 2001;19:525-34.

    19) Spurrell EL, Fisher C, Thomas JM, Judson IR. Prognostic factors in advanced synovial sarcoma: An analysis of 104 patients treated at the Royal Marsden Hospital. Ann Oncol. 2005;16:437-44.

    20) Ferrari A, De Salvo GL, Brennan B, van Noesel MM, De Paoli A, Casanova M, Francotte N, Kelsey A, Alaggio R, Oberlin O, Carli M, Ben-Arush M, Bergeron C, Merks JH, Jenney M, Stevens MC, Bisogno G, Orbach D. Synovial sarcoma in children and adolescents: The European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann Oncol. 2015;26:567-72.

    21) Orbach D, Mc Dowell H, Rey A, Bouvet N, Kelsey A, Stevens MC. Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: Experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group. Pediatr Blood Cancer. 2011;57:1130-6.

    22) Vlenterie M, Ho VK, Kaal SE, Vlenterie R, Haas R, van der Graaf WT. Age as an independent prognostic factor for survival of localised synovial sarcoma patients. Br J Cancer. 2015;113:1602-6.

    23) Lee N, Shin E. Treatment outcomes for patients with synovial sarcoma of the head and neck. Expert Rev Anticancer Ther. 2008;8:371-3.

    24) Ferrari A, Casanova M. New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther. 2005;5:307-18.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • [ Top ] [ Abstract ] [ PDF ] [ Similar Articles ] [ E-Mail to Author ] [ E-Mail to Editor ]