Main Article Content
Abstract
Oropharyngeal synovial sarcoma is a rare and aggressive soft tissue malignancy, warranting a comprehensive diagnostic approach involving clinical presentation, imaging, histological confirmation with molecular testing. Wide surgical excision with radiotherapy is the recommended treatment of choice, which is difficult to attain in this region due to close proximity to neurovascular structures.
SS (Synovial Sarcoma) is a rare malignant tumour of young adults typically arising in the deep soft tissues of the extremities, most commonly, the lower thigh-knee region. The occurrence of this tumour in unusual sites, distant from synovium-lined spaces, particularly, the abdominal wall[1] and head and neck region[2] has been highlighted in the literature, as case reports. The hypo-pharynx is the most common site of HNSS (Head and Neck Synovial Sarcoma).[3]
Oropharyngeal SS is extremely rare, and the subsite such as the tonsillo-lingual sulcus has not been reported in the literature yet.[4] The correct diagnosis, planning for surgery, and supportive therapies for synovial sarcoma at the oropharynx threatening the airway are always a challenge. Here we report a case of a female in her 4th decade, presenting with a mass obstructing the supra-glottic airway mimicking a vallecular cyst, which was finally diagnosed as a primary SS and treated with functional preservation.
We report a 40-year-old female with an oropharyngeal mass, mimicking a vallecular cyst, compromising the supraglottic airway and comprehensively diagnosed as synovial sarcoma. Treated by transoral excision with neoadjuvant radiotherapy which helped in functional preservation. At the five-year follow-up, the patient was disease-free.
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References
- Hale JE, Calder IM. Synovial sarcoma of the abdominal wall. Br J Cancer 1970;24:471–4. DOI: https://doi.org/10.1038/bjc.1970.56
- Kumar S, Shukla S, Avinash S, et al. Laryngeal synovial sarcoma: a rare clinical entity. Indian J Surg Oncol 2020;11(1):125–7. DOI: https://doi.org/10.1007/s13193-020-01097-4
- Pai S, Chinoy RF, Pradhan SA, et al. Head and neck synovial sarcomas. J Surg Oncol 1993;54(2):82–6. DOI: https://doi.org/10.1002/jso.2930540206
- Carrillo R, Rodriguez-Peralto JL, et al. Synovial sarcomas of the head and neck. Ann Otol Rhino lLaryngol 1992;101(4):367–70. DOI: https://doi.org/10.1177/000348949210100415
- Betal D, Babu R, Mehmet V. Monophasic synovial sarcoma of the pharynx: a case report Int Semin Surg Oncol 2009;6:9. DOI: https://doi.org/10.1186/1477-7800-6-9
- Russell WO, Cohen J, Enzinger F, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer. 1977;40:1562–70. DOI: https://doi.org/10.1002/1097-0142(197710)40:4<1562::AID-CNCR2820400428>3.0.CO;2-6
- Lockey MW. Rare tumors of the ear, nose and throat: synovial sarcoma of the head and neck. South Med J 1976;69:316–20. DOI: https://doi.org/10.1097/00007611-197603000-00021
- Doval DC, Kannan V, Mukherjee G, et al. Synovial sarcoma of the neck. Eur Arch Otorhinolaryngol 1997;254:246–250. DOI: https://doi.org/10.1007/BF00874097
- Lee N, Shin E. Treatment outcomes for patients with synovial sarcoma of the head and neck Expert Rev Anticancer Ther 2008;8(3):371-3. DOI: https://doi.org/10.1586/14737140.8.3.371
- Shukla PN, Pathy S, Sen S, et al. Primary orbital calcified synovial sarcoma: a case report. Orbit 2003;22(4):299-303. DOI: https://doi.org/10.1076/orbi.22.4.299.17246
- Crowson MG, Lalich I, Keeney MG, et al. Clinicopathologic factors and adjuvant treatment effects on survival in adult head and neck synovial cell sarcoma. Head. Neck 2015;37(3):375–80. DOI: https://doi.org/10.1002/hed.23605
- Danninger R, Humer U, Stammberger H. Synovial sarcoma, a rare tumor of the larynx. Case report and differential diagnostic considerations. Laryngorhinootologie 1994;73(8):442–4. DOI: https://doi.org/10.1055/s-2007-997169
- Ameerally PJ, Sira SK, Barrett AW, et al. Synovial sarcoma of hard palate. Br J Oral Maxillofac Surg 2004;42:261-3. DOI: https://doi.org/10.1016/j.bjoms.2003.12.005
- Harb WJ, Luna MA, Patel SR, et al. Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension Head Neck 2007;29:731-740. DOI: https://doi.org/10.1002/hed.20564
- Sultan I, Rodriguez-Galindo C, Saab R, et al. Ferrari Comparing children and adults with synovial sarcoma in the surveillance, epidemiology and end results program 1983–2005 Cancer 2009;115:3537-47. DOI: https://doi.org/10.1002/cncr.24424
- Cihak RA, Lydiatt WM, Lydiatt DD, et al. Synovial sarcoma of the head and neck: chromosomal (translocation)(X;18)as a diagnostic aid. Head Neck 1997;19:549–53. DOI: https://doi.org/10.1002/(SICI)1097-0347(199709)19:6<549::AID-HED14>3.3.CO;2-3
- Villaroel-Salinas J, Campos-Martinez J, Ortiz-Hidalgo C. Synovial sarcoma of the tongue confirmed by molecular detection of the SYT-SSX2 fusion gene transcript. Int J Surg Pathol 2012;20(4):386-9. DOI: https://doi.org/10.1177/1066896911424897
- Yoshida A, Sekine S, Tsuta K, et al. NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma. Am J Surg Pathol 2012;36(7):993-9. DOI: https://doi.org/10.1097/PAS.0b013e31824ee43c
- Giaoui L, Salvan D, Casiraghi O, et al. Primary synovial sarcoma of head and neck. Materials of the gustaveroussy institute. Report of 13 cases. Ann Otolaryngol Chir Cervicofac 1999;116:71-7.
- O’Malley BW, Weinstein GS, Snyder W, et al. Transoral robotic surgery (TORS) for base of tongue neoplasms Laryngoscope 2006;116:1465-72. DOI: https://doi.org/10.1097/01.mlg.0000227184.90514.1a
- Italiano A, Penel N, Robin YM, et al. Neoadjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group Ann Oncol 2009;20:425-30. DOI: https://doi.org/10.1093/annonc/mdn678
- Amble FR, Olsen KD, Nascimento AG, et al. Head and neck synovial cell sarcoma. Otolaryngol Head Neck Surg 1992;107:631-7. DOI: https://doi.org/10.1177/019459989210700504
- Sharif MA, Mushtaq S, Mamoon N, et al. Biphasic synovial sarcoma of oral cavity. J Coll Physicians Surg Pak 2008;18(11):713-5.
References
Hale JE, Calder IM. Synovial sarcoma of the abdominal wall. Br J Cancer 1970;24:471–4. DOI: https://doi.org/10.1038/bjc.1970.56
Kumar S, Shukla S, Avinash S, et al. Laryngeal synovial sarcoma: a rare clinical entity. Indian J Surg Oncol 2020;11(1):125–7. DOI: https://doi.org/10.1007/s13193-020-01097-4
Pai S, Chinoy RF, Pradhan SA, et al. Head and neck synovial sarcomas. J Surg Oncol 1993;54(2):82–6. DOI: https://doi.org/10.1002/jso.2930540206
Carrillo R, Rodriguez-Peralto JL, et al. Synovial sarcomas of the head and neck. Ann Otol Rhino lLaryngol 1992;101(4):367–70. DOI: https://doi.org/10.1177/000348949210100415
Betal D, Babu R, Mehmet V. Monophasic synovial sarcoma of the pharynx: a case report Int Semin Surg Oncol 2009;6:9. DOI: https://doi.org/10.1186/1477-7800-6-9
Russell WO, Cohen J, Enzinger F, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer. 1977;40:1562–70. DOI: https://doi.org/10.1002/1097-0142(197710)40:4<1562::AID-CNCR2820400428>3.0.CO;2-6
Lockey MW. Rare tumors of the ear, nose and throat: synovial sarcoma of the head and neck. South Med J 1976;69:316–20. DOI: https://doi.org/10.1097/00007611-197603000-00021
Doval DC, Kannan V, Mukherjee G, et al. Synovial sarcoma of the neck. Eur Arch Otorhinolaryngol 1997;254:246–250. DOI: https://doi.org/10.1007/BF00874097
Lee N, Shin E. Treatment outcomes for patients with synovial sarcoma of the head and neck Expert Rev Anticancer Ther 2008;8(3):371-3. DOI: https://doi.org/10.1586/14737140.8.3.371
Shukla PN, Pathy S, Sen S, et al. Primary orbital calcified synovial sarcoma: a case report. Orbit 2003;22(4):299-303. DOI: https://doi.org/10.1076/orbi.22.4.299.17246
Crowson MG, Lalich I, Keeney MG, et al. Clinicopathologic factors and adjuvant treatment effects on survival in adult head and neck synovial cell sarcoma. Head. Neck 2015;37(3):375–80. DOI: https://doi.org/10.1002/hed.23605
Danninger R, Humer U, Stammberger H. Synovial sarcoma, a rare tumor of the larynx. Case report and differential diagnostic considerations. Laryngorhinootologie 1994;73(8):442–4. DOI: https://doi.org/10.1055/s-2007-997169
Ameerally PJ, Sira SK, Barrett AW, et al. Synovial sarcoma of hard palate. Br J Oral Maxillofac Surg 2004;42:261-3. DOI: https://doi.org/10.1016/j.bjoms.2003.12.005
Harb WJ, Luna MA, Patel SR, et al. Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension Head Neck 2007;29:731-740. DOI: https://doi.org/10.1002/hed.20564
Sultan I, Rodriguez-Galindo C, Saab R, et al. Ferrari Comparing children and adults with synovial sarcoma in the surveillance, epidemiology and end results program 1983–2005 Cancer 2009;115:3537-47. DOI: https://doi.org/10.1002/cncr.24424
Cihak RA, Lydiatt WM, Lydiatt DD, et al. Synovial sarcoma of the head and neck: chromosomal (translocation)(X;18)as a diagnostic aid. Head Neck 1997;19:549–53. DOI: https://doi.org/10.1002/(SICI)1097-0347(199709)19:6<549::AID-HED14>3.3.CO;2-3
Villaroel-Salinas J, Campos-Martinez J, Ortiz-Hidalgo C. Synovial sarcoma of the tongue confirmed by molecular detection of the SYT-SSX2 fusion gene transcript. Int J Surg Pathol 2012;20(4):386-9. DOI: https://doi.org/10.1177/1066896911424897
Yoshida A, Sekine S, Tsuta K, et al. NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma. Am J Surg Pathol 2012;36(7):993-9. DOI: https://doi.org/10.1097/PAS.0b013e31824ee43c
Giaoui L, Salvan D, Casiraghi O, et al. Primary synovial sarcoma of head and neck. Materials of the gustaveroussy institute. Report of 13 cases. Ann Otolaryngol Chir Cervicofac 1999;116:71-7.
O’Malley BW, Weinstein GS, Snyder W, et al. Transoral robotic surgery (TORS) for base of tongue neoplasms Laryngoscope 2006;116:1465-72. DOI: https://doi.org/10.1097/01.mlg.0000227184.90514.1a
Italiano A, Penel N, Robin YM, et al. Neoadjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group Ann Oncol 2009;20:425-30. DOI: https://doi.org/10.1093/annonc/mdn678
Amble FR, Olsen KD, Nascimento AG, et al. Head and neck synovial cell sarcoma. Otolaryngol Head Neck Surg 1992;107:631-7. DOI: https://doi.org/10.1177/019459989210700504
Sharif MA, Mushtaq S, Mamoon N, et al. Biphasic synovial sarcoma of oral cavity. J Coll Physicians Surg Pak 2008;18(11):713-5.