Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children.

Ewing sarcoma (ES) of the jaw bones comprises a small fraction of ES at all sites. Due to their rarity, a specific policy for local treatment is lacking. The aim of this study was to evaluate the local therapy for ES and recommend measures to individualize treatment options. Patients with primary non-metastatic ES of the jaw bones treated between August 2005 and February 2015 were analyzed. All patients received primary induction chemotherapy, following which lesions amenable to resection based on specific radiological criteria were resected; those with unresectable lesions were offered definitive radiotherapy. The maxilla was the primary site in 13 patients and the mandible in eight. The median age of patients was 11.6 years (range 5-17 years). Overall, surgery was performed in 17 patients and definitive radiotherapy was used in four patients. Postoperative radiotherapy was administered to 12 patients and was avoided in five patients with 100% tumour necrosis. The 3-year overall survival, event-free survival, and local control were 68.1%, 63.6%, and 80.2%, respectively. Mandible primary and a histological response to chemotherapy were significant prognostic factors. The stratification of patients based on radiological criteria aids in selecting local therapy. In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcomes. Surgery also has the added advantage of identifying patients who may not need radiotherapy.

Qureshi SS ，Bhagat M ，Laskar S ，Kembhavi S ，Vora T ，Ramadwar M ，Chinnaswamy G ，Prasad M ，Khanna N ，Shah S ，Talole S ... -  《-》

Primary non-metastatic Ewing sarcoma of the jaw in children: results of surgical resection and primary reconstruction.

The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non-metastatic ES of the mandible and maxilla in children. Consecutive patients (mandible = 6, maxilla = 5) treated with surgery from August 2005 to January 2013 were selected. All patients received induction chemotherapy and were selected for surgical resection based on the presence of specific criteria for operability. The median age was 11.5 years (range 5-16 years). Free fibular osteocutaneous flap was commonly used for reconstruction. There were no complications related to microvascular anastomosis or flap loss. Five patients had 100% tumor necrosis and did not receive radiotherapy. Teeth alignment, chewing, swallowing, and speech were normal in all and donor site morbidity occurred in one. The 5-year overall, event-free survival, and local control are 87.5%, 72.9%, and 90%, respectively. In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcome. Surgery also has the added advantage of identifying patients who may not need radiotherapy.

Qureshi SS ，Kembhavi S ，Bhagat M ，Laskar S ，Chinnaswamy G ，Vora T ，Prasad M ，Ramadwar M ，Desai S ，Khanna N ，Kurkure P ，Shah S ，Shankdhar V ，Yadav P ... -  《-》

Ewing's sarcoma of the maxillofacial region in Greek children: Report of 6 cases and literature review.

The rarity of Ewing's sarcoma (ES) in the maxillofacial region of children, coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data regarding the optimal local control of the disease. To describe our experience in the management of primary maxillofacial ES in children, focusing on the therapeutic modalities for local control of the disease. Single institution observational study. This is a single institution review of patients, treated between 2007 and 2016. Six primary maxillofacial ES were treated according to the EURO-EWING 99 protocol, consisting of a uniform chemotherapy regimen, combined selectively with surgery and radiotherapy as local treatment. Patients' mean age was 9.42 years (range 6-12.5 years). One patient initially suffered from metastasis and succumbed to the disease; another refused further treatment following chemotherapy and was lost to follow-up. Four patients underwent surgery and adjuvant radiotherapy successfully. At a mean follow-up of 3.78 years relapse-free and overall survival rates were 60% and 80% respectively. The aesthetic and functional outcome was satisfactory in all treated patients. In eligible cases the combination of chemotherapy with surgery and adjuvant radiotherapy results in optimal oncological and functional outcome for children with ES of the maxillofacial region. Metastasis and poor response to chemotherapy are the most important adverse prognostic factors.

Iatrou I ，Theologie-Lygidakis N ，Schoinohoriti O ，Tzermpos F ，Mylonas AI ... -  《-》

Management and Outcome of Ewing Sarcoma of the Head and Neck.

Ewing sarcoma (EWS) of the head and neck is rare. Multimodal treatment consists of chemotherapy and local treatment; however, local treatment for EWS of the head and neck is challenging. The first objective was to describe local treatment administered to the patients with localized EWS of the head and neck according to the EURO-E.W.I.N.G.99-trial, and to assess the impact on survival. The second objective was to systematically review the scientific literature available for this topic. Fifty-one patients were included. Local control consisted of surgery and/or radiotherapy (RT). Event-free survival (EFS) and overall survival (OS) were determined. Outcome was analyzed by comparing local treatment approaches. A Medline search was performed for EWS of the head and neck. Eighty-six percent of patients had localized disease. Most common primary sites included the skull (45%), maxilla (14%), and mandible (12%). Three-year EFS was 74% and 3-year OS was 87% for patients with localized disease. EFS was 40% for patients >15 years compared to 81% for patients <15 years. Local control consisted of surgery (S; 33%), RT (18%), or S + RT (45%). Related 3-year EFS was 81% (S), 80% (RT), and 72% (S + RT); 3-year OS was 80%, 76%, and 81%, respectively. In patients with EWS of the head and neck, age, and stage are important prognostic factors. Although not statistically significant, large tumor volume seems to be a negative prognostic factor. No difference in EFS and OS could be found when comparing patients treated with surgery, RT, or combined surgery and RT.

Grevener K ，Haveman LM ，Ranft A ，van den Berg H ，Jung S ，Ladenstein R ，Klco-Brosius S ，Juergens H ，Merks JH ，Dirksen U ... -  《-》

Prognosis of Ewing's sarcoma of the head and neck.

Mamede RM ，Mello FV ，Barbieri J 《OTOLARYNGOLOGY-HEAD AND NECK SURGERY》