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

Abstract

Background, MethodsTo describe the characteristics, treatments (systemic/local), and outcome (oncological/functional) of French patients with head and neck Ewing's sarcomas (HNES) registered in the Euro‐Ewing 99 (EE99) database. Specific patient‐level data were reviewed retrospective.ResultsForty‐seven HNES patients in the EE99 database had a median age of 11 years, 89% had bone tumors (skull 55%, mandible 21%, maxilla 11%), 89% had small tumors (<200 mL), and they were rarely metastatic (9%). Local treatment was surgery radiotherapy (55%), exclusively surgery (28%), or radiotherapy (17%). Metastatic relapses occurred in five patients with high relapse risk factors (metastasis at diagnosis, poor histological response, large tumors). Local progression/relapses (LR) after exclusive radiotherapy occurred in three patients with persistent extra‐osseous residue and in four patients considered R0 margins (postchemotherapy surgery, without postoperative radiotherapy [PORT]), reclassified by pathological review as R1a. Pathological review reclassified 72% of R0 margins: 11/18 to R1a and 2/18 to R2. Five patients had confirmed R0 margins after postchemotherapy surgery without PORT and had no LR Eight patients had R2 margins (initial surgery without previous chemotherapy, with PORT) and had no LR With a median follow‐up of 9.3 years, the 3‐year LR rate, EFS, and OS were 84.8%, 78.6%, and 89.3%, respectively. Among the 5‐year survivors, 88% had long‐term sequelae.ConclusionTo optimize HNES management, patients should be treated from diagnosis in expert centers with multidisciplinary committees to discuss treatment strategy (type of surgery, need for PORT) and validate surgical margins.

The epidemiology, surgical management, and impact of margins in skull and mandibular osseous‐site tumors

“…Chordomas and cartilage tumors are almost entirely nonmandibular (>98.7% and 93.3%, respectively), consistent with previous studies 19‐22 . However, our study and the literature 5,15,23‐25 suggest a greater portion of ES and osteosarcoma are mandibular.…”

“…This large range may be due to varying age distribution, as previous work from our institution suggests that younger patients have a better prognosis 40 . While we elected not to analyze the effect of margins due to sample size, current research suggests that while clear margins render RT unnecessary, 5 they may not be necessary in the setting of adjuvant therapy 41 …”

“…Few studies within the otolaryngologic literature have examined primary bone tumors. According to the National Comprehensive Cancer Network (NCCN), primary osseous neoplasms make up <0.2% of all cancers, the majority of which occur outside the HN 2 (1%‐12% of chondromas, 3 25%‐35% of chordomas, 4 1%‐15% of Ewing sarcomas 5 [ES], 3%‐10% of fibrous histiocytomas, 6 and <10% of osteosarcomas 7 occur within the HN). Given their rarity, treatment strategies are guided by paradigms established outside of the HN.…”

Entrapment of the Greater Occipital Nerve with Chronic Migraine and Severe Facial Pain: A Case Report

“…1,2 Primary Ewing's sarcoma of the skull is very rare, occurring in only $1% of cases. 3,4 We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the therapy for this type of tumor. Our case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull.…”

Comment to: “Head and neck osteosarcomas: An analysis of treatment trends and survival outcomes in the United States (2004–2016)”

“…we have recently reported the importance of considering the initial tumor extension and not the post-neoCT residual tumoral volume for head and neck sarcoma resection. 7,8 Indeed, planned postchemotherapy surgery of the head and neck sarcoma initial tumor bed limits resections in postchemotherapy fibrosis, which is associated with fatal local relapses, and thus improve prognosis. 7,9 In conclusion, Shim et al reported survival analysis of large cohort of a rare disease, HNOS.…”