Title- Synovial Sarcoma; A Rare Entity In Submandibular Gland

Document Type : Case Study

Authors

1 department of otolaryngology and head and neck surgery, DOW University of Health and Sciences

2 department of otolaryngology and head and neck surgery, Dr Ziauddin University hospital, Karachi, Pakistan

10.32592/ARI.2025.80.1.635

Abstract

ABSTRACT:

INTRODUCTION: Salivary gland synovial sarcomas, constituting <1% of oral tumors, occurring mostly in young and adolescents age group, present diagnostic challenges. it clinically presents as a painless mass, often growing silently for months or even years. Grossly, synovial sarcoma presents as a tan or grey mass with multi-nodular or multi-cystic formations and is characterized by the presence of spindle cells and CKAE1/AE3, TLE-1, and CD99 positivity. Prognosis is influenced by patient demographics, tumor characteristics, and treatment modalities. Factors such as age over 35 at diagnosis, epithelioid type, and localization outside the head and neck region are associated with worse prognoses.

CASE SUMMARY: We present a 48-year-old male with a swelling in the left submandibular area with no other associated symptoms. An ultrasound of the lump showed a well-circumscribed, cystic cum solid mass, with internal vascularity. Fine needle aspiration cytology (FNAC) demonstrated nuclear pleomorphism and high mitotic activity. Excisional histopathology was done and diagnosis of unifocal synovial sarcoma was made, characterized by spindle cells, with a mitotic rate of 23 per 10 high-power fields, with positivity for CKAE1/AE3, TLE-1, and CD99 and negativity for S100 in tumor cells.

CONCLUSION: We here presents a case of synovial sarcoma in an adult patient, with an unlikely site of occurrence This report also contributes to limited literature regarding this rare malignancy, shedding light on their clinical and diagnostic characteristics, and emphasizing identification of specific immunohistochemical markers such as CKAE1/AE3, TLE-1, and CD99 add to the understanding of its molecular characteristics

KEY-WORDS: Synovial sarcomas, submandibular gland, spindle cells, immunohistochemical markers.

Keywords

Main Subjects

References

  1. Mariano FV, Oliveira Gondak R, da Costa MV, Corrêa MB, Lopes MA, de Almeida OP. Primary synovial sarcoma involving the submandibular gland. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(1):e61-65.
  2. Gazendam AM, Popovic S, Munir S, Parasu N, Wilson D, Ghert M. Synovial Sarcoma: A Clinical Review. Curr Oncol. 2021;28(3):1909-1920. doi:10.3390/curroncol28030177
  3. Aytekin MN, Öztürk R, Amer K, Yapar A. Epidemiology, incidence, and survival of synovial sarcoma subtypes: SEER database analysis. J Orthop Surg Hong Kong. 2020;28(2):2309499020936009.
  4. Srivastava A, Ghosh A, Saha S, Saha VP, Chakraborty D. Sarcomas of head and neck - A 10 yrs experience. Indian J Otolaryngol Head Neck Surg Off Publ Assoc Otolaryngol India. 2007;59(4):322-326. doi:10.1007/s12070-007-0093-3
  5. Alsanie I, Rajab S, Cottom H, et al. Distribution and Frequency of Salivary Gland Tumours: An International Multicenter Study. Head Neck Pathol. 2022;16(4):1043-1054.
  6. Jo VY, Fletcher CDM. WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology (Phila). 2014;46(2):95-104.
  7. Nielsen TO. Synovial Sarcoma. In: Maloy S, Hughes K, eds. Brenner’s Encyclopedia of Genetics (Second Edition). Academic Press; 2013:619-622.
  8. Terry J, Saito T, Subramanian S, et al. TLE1 as a diagnostic immunohistochemical marker for synovial sarcoma emerging from gene expression profiling studies. Am J Surg Pathol. 2007;31(2):240-246.
  9. Rekhi B, Basak R, Desai SB, Jambhekar NA. Immunohistochemical validation of TLE1, a novel marker, for synovial sarcomas. Indian J Med Res. 2012;136(5):766-775.
Archives of Razi Institute
Volume 80, Issue 2
March and April 2025
Pages 635-638

Files

Share

How to cite

Statistics