Primary Ewing's Sarcoma Arising from the Mandible: A Case Report.
- Author:
Joong Wha KOH
1
;
Sang Hoon CHUN
;
Young Taek OH
;
Jin Hyuk CHOI
Author Information
1. Department of Otolaryngology, Ajou University School of Medicine, Suwon, Korea. ent50@madang.ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Ewing's sarcoma;
Mandible;
Combined therapy
- MeSH:
Bone Marrow;
Burkitt Lymphoma;
Child;
Cyclophosphamide;
Cytogenetics;
Cytoplasm;
Diagnosis, Differential;
Doxorubicin;
Drug Therapy;
Humans;
Ifosfamide;
Incidence;
Jaw;
Male;
Mandible*;
Neural Plate;
Neuroblastoma;
Neuroectodermal Tumors, Primitive;
Osteosarcoma;
Pelvis;
Retinoblastoma;
Sarcoma, Ewing*;
Young Adult
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
1999;42(2):249-254
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Ewing's sarcoma is an uncommon malignancy that usually occurs in children. It is composed of monotonous population of small cells with large nuclei and little cytoplasm. These cells fall into the category of the "small, round, blue cell tumors of children". Based on cytogenetic studies, Ewing's sarcoma appears to be of neuroectodermal origin and shares the same reciprocal 11:22 translocation as primitive neuroectodermal tumor. Long bones and pelvis are the preferential sites. The incidence of Ewing's sarcoma in all primary malignant bone tumors is four-seven percent. Only seven percent of Ewing's sarcoma occurs in the mandible either as primary or metastatic disease. The rarity is due to the paucity of hematopoietic marrow of the mandible. Soft tissue swelling and pain are the most common presenting symptoms. Differential diagnosis of Ewing's sarcoma of the mandible includes other mass lesions of the jaw such as osteosarcoma, Burkitt's lymphoma, retinoblastoma and neuroblastoma. Treatment for mandibular Ewing's sarcoma consists of various single and combination modalities including radiation, chemotherapy, and surgery. We report a case of a 24-year-old male with mandibular Ewing's sarcoma who was treated with chemotherapy (vincristine, adriamycin, cyclophosphamide and ifosfamide, etoposide) and concurrent radiation therapy (6MV, 180-200 cGy/d, 32 fractination, total dose: 5900 cGy).
