Functional Outcomes After Scapulectomy and Glenoid Resection for Ewing Sarcoma
Removing the scapula and glenoid to treat Ewing sarcoma results in severe functional impairment with poor shoulder function (mean MSTS score 79%), though this aggressive surgery may be necessary for local control when the tumor extensively involves these structures. 1
Survival and Disease Control Outcomes
The prognosis for scapular Ewing sarcoma is notably poor despite aggressive multimodal treatment:
- Median survival is only 30 months with 5-year disease-specific survival of 38%, significantly worse than the 60-75% survival typically seen with extremity Ewing sarcoma 1, 2
- Scapular location represents an unfavorable anatomic site, similar to pelvic tumors, which are known adverse prognostic factors 3
- Even with complete surgical resection combined with chemotherapy, outcomes remain poor due to the aggressive nature of scapular Ewing sarcoma 1
Functional Consequences of Scapulectomy
The functional impact of removing the scapula and glenoid is substantial:
- Mean Musculoskeletal Tumor Society (MSTS) functional score is 79±14%, indicating significant functional limitation 1
- Complete scapulectomy eliminates the attachment point for 17 muscles critical for shoulder and upper extremity function
- Loss of the glenoid removes the glenohumeral articulation, resulting in a flail shoulder with severely limited active range of motion
- Patients typically retain some function through scapulothoracic motion and compensatory mechanisms, but overhead activities and heavy lifting are severely compromised
Decision-Making: Surgery vs. Radiotherapy
The choice between surgical resection and definitive radiotherapy for scapular Ewing sarcoma requires careful consideration:
Surgery is preferred when:
- Complete resection with wide margins is technically achievable 3
- The tumor shows good response to induction chemotherapy (>90% necrosis) 2
- Patient age and performance status permit major surgery 3
Definitive radiotherapy (50-60 Gy) should be considered when:
- Complete surgical removal would cause unacceptable morbidity, though this must be balanced against the already poor functional outcome of scapulectomy 3
- The tumor is judged inoperable due to extensive involvement of neurovascular structures 3
- Patient factors create unacceptable surgical risk 3
Notably, incomplete surgery followed by radiotherapy was not superior to radiotherapy alone in large series, emphasizing that if surgery is chosen, complete resection must be achieved 3
Treatment Response and Tumor Necrosis
Induction chemotherapy produces measurable tumor response in scapular Ewing sarcoma:
- Mean tumor size decreases from 10±2 cm to 6±3 cm (p=0.02) 1
- Mean tumor volume decreases from 181±112 cm³ to 20±12 cm³ (p<0.01) 1
- Mean tumor necrosis in resected specimens is 72±23%, though this is below the favorable threshold of >90% necrosis 1
Multimodal Treatment Protocol
All patients with scapular Ewing sarcoma require intensive multimodal therapy:
- Induction chemotherapy with 3-6 cycles using doxorubicin, vincristine, ifosfamide, etoposide, cyclophosphamide, and dactinomycin 3
- Local control via complete surgical resection or definitive radiotherapy (50-60 Gy for macroscopic disease) 3
- Consolidation chemotherapy with 8-10 additional cycles for total treatment duration of 8-12 months 3
Critical Caveats
- The scapula's flat bone anatomy is similar to the pelvis, another site associated with poor prognosis in Ewing sarcoma 3
- Despite aggressive treatment including scapulectomy, survival remains poor (38% at 5 years), suggesting that anatomic location itself is a major adverse prognostic factor 1
- All treatment should occur at specialized sarcoma centers with multidisciplinary expertise, as this rare presentation requires complex coordination 3, 2
- Long-term surveillance beyond 10 years is essential for detecting late relapses and treatment-related complications including secondary malignancies 2