Primary Subcutaneous Synovial Sarcoma: First Reported Subcutaneous Case Showing TLE1 Immunoreactivity

Am J Dermatopathol. 2018 Oct;40(10):772-777. doi: 10.1097/DAD.0000000000001172.

Abstract

Synovial sarcoma (SS) accounts for 5%-10% of all soft tissue sarcomas. It is a well-defined soft tissue neoplasm with biphasic and monophasic histologic subtypes and unknown histogenesis. It usually occurs in the extremities, especially the thigh-knee region of young adults. Recurrences are frequent and distant metastasis developed in approximately half of the patients. SSs are characterized by a recurrent nonrandom chromosomal translocation, t(X; 18) (p11; q11), which is considered the primary genetic event in more than 90% of cases. Only 4 cases of cutaneous and subcutaneous SSs have been published in the literature so far. We report a case of primary subcutaneous SS in the forearm of a young woman and discuss the histopathologic differential diagnosis with other similar neoplasms. This is the first reported case of primary cutaneous SS showing immunoreactivity for TLE1 in the nuclei of neoplastic cells, supporting the use of this marker for diagnosis of this rare cutaneous neoplasm.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / immunology*
  • Biopsy
  • Co-Repressor Proteins
  • Diagnosis, Differential
  • Female
  • Forearm
  • Gene Rearrangement
  • Humans
  • Immunohistochemistry*
  • In Situ Hybridization, Fluorescence
  • Predictive Value of Tests
  • Proto-Oncogene Proteins / genetics
  • Repressor Proteins / genetics
  • Repressor Proteins / immunology*
  • Sarcoma, Synovial / genetics
  • Sarcoma, Synovial / immunology*
  • Sarcoma, Synovial / pathology
  • Sarcoma, Synovial / surgery
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / immunology*
  • Soft Tissue Neoplasms / pathology
  • Soft Tissue Neoplasms / surgery
  • Subcutaneous Tissue / immunology*
  • Subcutaneous Tissue / pathology
  • Subcutaneous Tissue / surgery

Substances

  • Biomarkers, Tumor
  • Co-Repressor Proteins
  • Proto-Oncogene Proteins
  • Repressor Proteins
  • SS18 protein, human
  • TLE1 protein, human