Primary Ewing Family of Tumors of the Jaw Has a Better Prognosis Compared to Tumors of Extragnathic Sites

Abstract

Purpose Primary Ewing sarcoma of the jaw is rare. The aim of this study was to describe new cases of primary Ewing sarcoma of the jaw and investigate reported prognostic factors of Ewing sarcoma in this series and treatment outcome. Materials and Methods Six patients with primary Ewing sarcoma of the jaw were treated at the Memorial Sloan Kettering Cancer Center (MSKCC) from 1992 through 2013. Clinical data, pathology reports, treatment prescribed, treatment regimens, outcome, and follow-up information were reviewed. Results Five of 6 patients were female and 5 cases were in the mandible. No patient presented with metastatic disease at diagnosis. All cases were positive for CD99, and 3 patients with genetic confirmation were positive for EWS-FLI1 fusion or EWSR1 gene rearrangement. All patients received induction multiagent chemotherapy and surgical resection and 2 patients received adjuvant radiotherapy. Total (grade IV) or nearly total (grade III) tumor necrosis in 3 of 5 patients (60%) assessed for histologic response to chemotherapy indicated intense sensitivity. All patients were alive and free of disease, with no history of local recurrence, at a median follow-up period of 6.5 years. Conclusion Patients with primary Ewing sarcoma of the jaw have a good prognosis and metastasis is an uncommon occurrence at initial presentation.

Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review

“…The final diagnosis of ES/pPNET was only confirmed after microscopy associated with immunohistochemical analysis. Other authors have also misdiagnosed cases with clinical features similar to those of our case as being cysts (3,(19)(20)(21). Moreover, trauma (22)(23)(24) and acute inflammatory lesions (1, 3, 24-29, 30, 31) were also mentioned several times as provisional diagnosis.…”

“…The medical literature contains only single case reports or small case series including six patients (3,24,25,36). To determine the exact number of children/young adults with primary ES or ES/pPNET of the jaws in the medical literature is very difficult, and in fact, the data reported are confusing.…”

Ewing’s Sarcoma of the Head and Neck: Margins are not just for surgeons

“…We confirmed that HNES are rare (4.1% of all ES) (Data ), arising mainly from bones (skull, mandible, maxillary) in children/adolescents with persistent growing potential (median age 11 years; only 26% postpuberty). Most HNES patients have low ES relapse risk factors :91% with nonmetastatic tumors, 89% with small primary tumors at diagnosis, and 81% with good histological response to chemotherapy and a favorable outcome (3y‐EFS and OS of 78.6% and 89.3%, respectively). However, for the minority of HNES patients with high ES relapse risk factors (metastatic disease and/or poor histological response), the outcome is impaired by metastatic relapses, as in other ES localizations .…”

Head and Neck Round Cell Sarcomas: A Comparative Clinicopathologic Analysis of 2 Molecular Subsets: Ewing and CIC-Rearranged Sarcomas

“…A recent study using the Surveillance, Epidemiology, and End Results (SEER) database, showed that patients with HN-ES have a better OS compared to ES of other sites (p = 0.015), possibly related to the lower disease burden [smaller tumor size and lower initial metastasis at diagnosis (p < 0.001)] at presentation compared to ES of other sites [37]. Moreover, in our prior study of primary jaw ES, all 6 patients were alive with no evidence of disease at last follow-up and none presented with initial metastasis at diagnosis [38].…”