Ewing Sarcoma in Older Adults: A Clinicopathologic Study of 50 Cases Occurring in Patients Aged ≥40 Years, With Emphasis on Histologic Mimics

Abstract

Objective. We explore the clinicopathologic features of Ewing sarcoma (ES) presenting in older adulthood. Methods. Cases of molecularly confirmed ES arising in patients aged ≥40 years were evaluated. Results. Fifty patients were identified (33 males/17 females; 41-86 years). The majority of tumors (41) arose at extraskeletal sites, while 9 were bone primaries. Twenty-eight cases showed nested architecture, while the remaining cases showed sheet-like growth. Tumor cytology was categorized as conventional (n = 39), crushed (n = 5), clear cell (n = 4), rhabdoid (n = 3), and epithelioid (n = 2). Fifty percent had necrosis, while rosettes were noted in 1 case. Immunostains performed ranged from 1 to 28 (median = 10). Follow-up (n = 43, 1-147 months) revealed 15 patients with metastasis. Conclusion. Although rare, ES should be considered in the differential diagnosis for round cell malignancies in older adult patients. In this cohort, ES is most often extraskeletal, and may show unusual morphologic features, closely simulating more common neoplasms in this age group.

Comorbidities rather than older age define outcome in adult patients with tumors of the Ewing sarcoma family

“…This observation is in‐line with previous studies suggesting an adverse effect of comorbidity on survival of sarcoma patients 25–27 . In addition, in accordance with previously published case series, we found a significant higher frequency of extra‐skeletal primary tumors in the older patient which could also explain part of our observation 3,28 …”

“…[25][26][27] In addition, in accordance with previously published case series, we found a significant higher frequency of extra-skeletal primary tumors in the older patient which could also explain part of our observation. 3,28 Furthermore, as expected, patients with metastatic disease had a significantly shorter OS and PFS than those with localized disease. The prominent role of stage in our cohort is consistent with previous analyses in adult patients with EFT and even was described as the sole predictor of survival by Martin II et al [29][30][31][32] In addition, some adult patient populations have linked tumor size, gender, and non-extremity bone location as well as the differing treatment regimen to impact outcome.…”

“…Published data suggest a rate of only <20% of all ES occurring over the age of 40. 3 Furthermore, there is evidence that ES in patients ≥41 years tend to be more aggressive as patients may be more likely to show extra‐skeletal and metastatic disease. Additionally, the survival rate in older patients seems to be generally lower.…”

Ewing’s Sarcoma

“…1); extraosseous Ewing's sarcoma occurs much more frequently in adults than in children. 4,5 The discovery of an undifferentiated roundcell tumor in the soft tissues of an adult warrants inclusion of Ewing's sarcoma in the differential diagnosis.…”

Ewing Sarcoma of Phalanx—A Common Tumor with an Uncommon Presentation

“…In 20% of patients, tumors are extraosseous and this occurs more frequently in adults. 6,7 Patients present with localized pain and swelling. A pathological fracture is reported in 10 to 15% of cases, and nonspecific constitutive symptoms, including fever, weight loss, night sweats, and fatigue, may be present.…”