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Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade's Experience From a Single Centre in India
Sudha S Murthy1, S Challa1, Kvvn Raju2
1Department of Pathology, Basavatarakam Indo American Cancer Hospital & Research Institute, Hyderabad, India.
Clinical Pathology (Thousand Oaks, Ventura County, Calif.)
|November 26, 2020
Summary
Diagnosing Ewing sarcoma family of tumours (ESFT) is complex, particularly in adults and non-bone locations. This study highlights significant differences in age, sex, and tumor site between skeletal and extra-skeletal ESFT, aiding diagnosis.
Area of Science:
- Oncology
- Pathology
- Molecular Diagnostics
Background:
- Diagnosis of Ewing sarcoma family of tumours (ESFT) presents challenges, especially in adults and extra-skeletal or visceral locations.
- Morphologic mimics and varied prognoses complicate ESFT diagnosis, necessitating advanced diagnostic tools.
- Ancillary diagnostic modalities in surgical pathology are crucial for accurate ESFT diagnosis in various locations.
Purpose of the Study:
- To evaluate clinicopathological features of ESFT, focusing on molecular results.
- To analyze ESFT cases concerning sex, age, and primary tumor location.
- To specifically emphasize extra-skeletal soft tissue and visceral ESFT characteristics.
Main Methods:
- Retrospective review of 302 ESFT cases diagnosed over ten years.
- Analysis of clinicopathological data and molecular test results (FISH, RT-PCR).
- Statistical comparison of skeletal versus extra-skeletal ESFT regarding age and sex using SPSS (P<.05 significant).
Main Results:
- The cohort comprised 302 ESFTs: 49% skeletal and 51% extra-skeletal.
- Common sites: thigh (skeletal); chest wall, paraspinal, retroperitoneum (soft tissue); kidney, ovary, cervix (visceral).
- Skeletal ESFT showed male predominance, younger age, and extremity location; extra-skeletal ESFT had no gender predilection, higher age, and axial location (statistically significant).
- Molecular tests (EWSR1 rearrangement) were positive in 54 (FISH) and 19 (RT-PCR) patients, more frequent in extra-skeletal/visceral tumors.
Conclusions:
- Statistically significant differences exist in age, sex, and location between skeletal and extra-skeletal ESFT.
- Increased incidence of extra-skeletal ESFT, particularly in visceral sites, is linked to heightened awareness and improved ancillary diagnostic techniques.
- Accurate diagnosis of ESFT in diverse locations is facilitated by molecular testing and awareness of clinicopathological variations.
