The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the pat...

Ewing tumor family consists of Ewing tumor of bone, extraosseous Ewing tumor, primitive neurectodermal tumor and Askin tumor. All of them share genetic abnormality, reciprocal translocation (11; 22) (q24; q12), and originate from the same primordial stem cell. Ewing tumor is the most common form, found in 60% of cases. It is the second primary malignant bone tumor. Localized lesion is found in nearly 80% and metastatic disease in 20% of cases. Patients present mostly due to pain and palpable tumefaction, and pathological fracture as the initial problem develops in long bones. Ewing tumor can develop in virtually any bone of the body and in extraosseous localizations as well, while localization in the extremities occurs in 50% of patients. Head or neck localizations are extremely rare. Paraspinal, retroperitoneal or deep pelvic tumor localization is manifested by back pain. Systemic symptoms are also present, commonly fever or weight loss, which often indicates the presence of metast...

Ewing’s sarcoma (ES) is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells. Primary ES commonly occurs in early childhood or adolescence. It may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is a case report of sinonasal ES in a 13-year-old female patient who presented with a 7-month history of right nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss. Clinical examination revealed a right nasal mass pushing the septum to the left side and extending to the nasopharynx. Endoscopic biopsy and histopathological analysis showed a small blue cell tumor suggestive of ES. The patient was treated with surgery, radiotherapy, and chemotherapy. After a follow-up of 5 years, the patient remains recurrence-free with excellent functional status and quality of life.

Ewing's Sarcoma (ES) was first described by James Ewing in 1921 as a" Diffuse Endothelioma of Bone". It had been postulated to arise from primitive mesenchymal, reticulum, angioblastic and myelogenous cells of the bone marrow (1,2,3). It is now believed to belong to a larger group of Ewing Sarcoma Family of Tumors that share a common histogenesis from primitive neuroectodermal cells and includes ES of bone (ESB), extraosseous ES, Primitive neuroectodermal tumor (PNET) and Askins tumour (malignant small-cell tumor of the thoracopulmonary region.) (3). ESB is the second most common primary malignancy of bone after Osteosarcoma but it is relatively rare accounting for 4%-10% of all malignant neoplasms of bone. It is primarily a disease of children and adolescents in the second decade of life. The incidence in males and females is equal up to the age of 13 years, thereafter occurrence in males predominates. It is nine times more common in white children as compared to blac...