Ewing′s sarcoma of the mandible

Abstract

Ewing's sarcoma is a malignant tumor of bones that primarily affects children and young adults. The true origin of this small round cell lesion still remains controversial. It was originally described by James Ewing in 1921 as arising from undifferentiated osseous mesenchymal cells; however, recent studies suggest that Ewing's tumor might be neuroectodermally derived from various degrees of differentiation of the primitive neural tissues. This paper reports a rare case of ES of the mandible in an 11-year-old girl, which had been previously misdiagnosed and treated as a dental abscess. In the clinical examination, a hard immobile expansive mass of 2 cm diameter was observed on the left side of the mandible. Radiographic examination revealed a diffuse radiolucent lesion with ill-defined borders and wide vestibular bone plate destruction. Microscopically, the tumor was composed by monotonous small round cells that exhibited immunoreactivity for CD99, vimentin and desmin. Surgical resection of mandible followed by mandibular reconstruction was adopted. The patient was subjected to multiagent chemotherapy with Vincristine [VC], Dactinomycin [AC], Cyclophosphamide [CP] and Doxorubicin [AD]).

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

“…17, 18, 19 Two of the present cases had a similar presentation: 1 manifested as a dental cyst on panoramic radiograph and another case was initially considered a dental abscess refractory to treatment with medications. In the present series, 5 of 6 patients were female despite the aforementioned male predilection of ES of the jaw.…”

Ewing′s sarcoma of mandible: A case report and review of Indian literature

“…[78] Prasad in 2008, Deshingkar and Gupta in 2009 and Dadhe in 2010 reported cases with extensive nasomaxillary destruction, proptosis of eye and decreased nasal airway competence. [412–14] Rao and Pampori in 2011 reported cases of ES involving mandible and maxilla, respectively. [15] Till date only 19 cases of ES involving jaws have been described in Indian literature.…”

“…Identification of a common translocation t (11;22) (q24;q12) resulting in EWS–ETS fusion gene in above-mentioned tumors strongly supported their inter-relationship making them included in same group, the ESFT. [14]…”

“…Most ES shows characteristic chromosomal translocation between chromosomes 11 and 22, the t (11;22) (q24;q12) and trisomies 8 and/or 12 are observed in one-third to half of the cases. [214] Demonstration of EWS/FLI1 fusion is a better prognostic indicator than other variant gene fusions. [14] This could not be performed here due to unwillingness of the patient party.…”

“…[214] Demonstration of EWS/FLI1 fusion is a better prognostic indicator than other variant gene fusions. [14] This could not be performed here due to unwillingness of the patient party.…”