Solitary Fibrous Tumors in Pediatric Patients: A Rare and Potentially Overdiagnosed Neoplasm, Confirmed by STAT6 Immunohistochemistry

Pediatr Dev Pathol. 2018 Jul-Aug;21(4):389-400. doi: 10.1177/1093526617745431. Epub 2017 Dec 11.


Pathological diagnosis of solitary fibrous tumor (SFT) in the pediatric population is challenging, as it occurs uncommonly in this age-group and resembles other spindle cell neoplasms. SFT contains a NAB2-STAT6 fusion gene, which can be reliably detected using STAT6 immunohistochemistry. Positive staining is highly sensitive and specific. We sought to investigate the utility of STAT6 immunohistochemistry, to show how commonly SFT was historically recognized at 3 academic pediatric institutions, to reclassify them when appropriate, and to demonstrate features of major mimics of SFT. Our series included cases with a previous diagnosis of SFT or for which SFT was among key considerations, from 3 major academic pediatric hospitals seen over the past 30 years. Of 18 tumors identified, only 3 tumors from 2 patients demonstrated positive STAT6 staining as well as the typical histology and immunophenotype seen in SFT. The remaining 15 tumors were reclassified based on morphology, additional immunohistochemistry and fluorescence in situ hybridization as desmoid-type fibromatosis (3 tumors), nerve sheath/neural tumors (3 tumors), low-grade fibromyxoid sarcoma, medallion-like dermal fibroma, poorly differentiated Sertoli cell tumor, nodular/proliferative fasciitis, calcifying fibrous tumor, aneurysmal bone cyst of soft tissue, STAT6-negative SFT with adipocytic differentiation, undifferentiated small round blue cell tumor, and scar (1 tumor each). Our study confirms that SFT is rare in the pediatric population and that it is potentially overdiagnosed. STAT6 immunohistochemistry is recommended to confirm the diagnosis of SFT in the pediatric population.

Keywords: STAT6 immunohistochemistry; soft tissue neoplasms; solitary fibrous tumor.

MeSH terms

  • Adolescent
  • Biomarkers, Tumor / metabolism*
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Diagnostic Errors / statistics & numerical data*
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Medical Overuse / statistics & numerical data*
  • Retrospective Studies
  • STAT6 Transcription Factor / metabolism*
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / pathology
  • Solitary Fibrous Tumors / diagnosis*
  • Solitary Fibrous Tumors / metabolism
  • Solitary Fibrous Tumors / pathology


  • Biomarkers, Tumor
  • STAT6 Transcription Factor
  • STAT6 protein, human