Ewing's sarcoma of the head and neck

Abstract

a b s t r a c tCONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual.CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

Ewing's sarcoma of the mandible in a young child

“…The intracytoplasmatic glycogen may be demonstrated by PAS stain in 75% of the cases, but it is not pathognomonic and conclusive because other small round cell may show the presence of glycogen as well (14,15). Due to similarity with many malignant tumors, the diagnosis of ES can be very difficult and the lesion must be differentiated from other small round cell tumors, such as small cell osteosarcoma, mesenchymal chondrosarcoma, embryonal rhabydomyosarcoma, neuroblastoma and lymphoma (1,16). The use of immunohistochemistry has helped in the diagnosis of this tumor.…”

“…It has been reported that combined therapy including surgery, radiotherapy and chemotherapy is the best approach for ES (16). The multidisciplinary treatment protocols has dramatically improved the 5-year survival rate of patients with ES from less than 16% to more than 75% of cases (1,5).…”

“…The multidisciplinary treatment protocols has dramatically improved the 5-year survival rate of patients with ES from less than 16% to more than 75% of cases (1,5). Radiotherapy alone must be undertaken only to treat non-resectable primary tumor or as a neoadjuvant therapy and chemotherapy to suppress potential micrometastasis and reduce the size of the tumor prior to surgery (16,18). The chemotherapeutic agents most commonly used are vincristine, doxorubicin, cyclophosphamide, ifosfamide, and actinomycin-D. Our patient was subjected to the multiagent chemotherapy before surgery because of the size of the primary tumor.…”

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

“…Secondly, and most importantly, ES/pPNETs were often not separated from ES of the jaw and were reported together in case series and literature reviews (37,38). Thirdly, immunohistochemical and molecular genetic data that enable separation of central PNETs from peripheral PNETs were not reported in most cases, particularly those published before 2000 (37,39). Pooling of patient data would, however, enable such a comparison to be made, thereby identifying possible differences between ES and ES/pPNETs.…”

Ewing’s Sarcoma of the Mandible: A Rare Case Report and Review of Literature

“…Intracytoplasmic glycogen is a definitive aspect, but not pathognomic because it is also present in other tumor cells, such as osteosarcomas, rhabdomyosarcomas and neuroblastomas. 12 Recent immunoperoxidase and cytogenic studies indicate that PNET and ES are the same entity showing varying degrees of neuroectodermal differentiaton and they are categorized into a group known as the Ewing family of tumors. CD 99/MIC2 is a cell surface glycoprotein found in virtually all ES and PNET.…”