Bone Neoplasms/therapy* ; Child ; Humans ; Sarcoma, Ewing/therapy*

Extraskeletal Ewing's sarcoma is a very rare tumor which was first reported by Angervall and Enzinger in 1975. The common sites of extraskeletal Ewing s sarcoma are bony structures of lower extremities, paravertebral region, and pelvis, but rarely chest walL Microscopically, extraskeletal Ewing's sarcoma is indistinguishable from the Ewing's sarcoma of bone. We present here a case of extraskeletal Ewing's sarcoma of the left lateral chest wall in a 19-year-old male. Wide extirpation and postoperative combined chemotherapy were done, and we discuss the clinical picture, histopathology, therapeutic management, and prognosis with review of the literature.
Drug Therapy ; Humans ; Lower Extremity ; Male ; Pelvis ; Prognosis ; Sarcoma* ; Sarcoma, Ewing* ; Thoracic Wall* ; Thorax* ; Young Adult

OBJECTIVETo recognize and improve the outcome of childhood Ewing's sarcoma family tumors, and to identify the efficacy and safety of the chemotherapy using RS-2002 Protocol.

METHODSFrom September 1997 to September 2006, 14 newly diagnosed patients with the tumors were admitted, 9 were boys, and 5 were girls, the median age was 7.04 years, ranging from 1.58 years to 11.67 years. Among them, 9 patients were younger than 10 years. By the time of diagnosis, 9 patients had local diseases, and the other 5 patients had metastatic diseases. All the patients' diagnoses were confirmed by pathological studies. Nine patients had Ewing's sarcoma by histology, and the other 5 patients had peripheral primitive neuroectodermal tumors (PPNET). All of the patients were treated with multidisciplinary therapy, and RS-2002 Protocol for chemotherapy was used to treat patients with rhabdomyosarcoma in our hospital. Surgery and irradiation were performed for local control. Imaging studies were used for evaluation, reevaluation and follow-up.

RESULTSTill April 30th 2007, 13/14 patients survived. The median follow-up time was 41 months (range: 7 months-115 months). The 10-year overall survival (OS) was 88.9%+/-10.5%, and the 10-year disease-free-survival (DFS) was 72.2%+/-13.8%; 3/14 patients had disease relapse, the median time to relapse from initial diagnosis was 23 months (range: 16-30 months). One patient developed second malignancy. No therapy related death was documented.

CONCLUSIONSChildhood Ewing's sarcoma family tumors were not very rare, and the prognosis was acceptable with optimal treatment. RS-2002 Protocol was effective and safe in treating such patients.

Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Humans ; Infant ; Male ; Sarcoma, Ewing ; therapy ; Treatment Outcome

Extraskeletal Ewing sarcoma is a rare event, and extraskeletal Ewing sarcoma of the thyroid gland is even rarer. It has non-specific clinical manifestation and difficulty in early diagnosis. The diagnosis mainly depends on histology and immunohistochemistry. It possesses the features of high malignancy, high rate of local recurrence, and distant metastasis. Currently, the aggressive multimodal treatment contains surgery, chemotherapy, and radiotherapy. This study presented a case of extraskeletal Ewing sarcoma arising in the thyroid gland of a 30-year-old woman, who presented with supraclavicular mass and sense of dysphagia obstruction in Department of Otolaryngology, Head and Neck Surgery, Second Xiangya Hospital, Central South University in 2018. Imaging studies demonstrated a cystic-solid mass in inferior of the left leaf of thyroid gland and in the posterior of the trachea and esophagus. The patient underwent localized tumor resection. The pathological diagnosis revealed that it was a small round cell tumor, and the immunohistochemistry results were considered to be extraskeletal Ewing sarcoma. Subsequently, the patient was given chemotherapy and local radiation therapy. There was no evidence of tumor recurrence or metastasis.
Adult ; Combined Modality Therapy ; Female ; Humans ; Immunohistochemistry ; Neoplasm Recurrence, Local ; Sarcoma, Ewing/therapy* ; Thyroid Gland

We present a case of primary Ewing's sarcoma involved the cranium in a young child. The tumor originated in the temporal bone. The patient was acutely compromised by signs and symptoms of raised intracranial pressure. Radiographic studies including brain magnetic resonance image showed a huge mass affecting the temporal bone. Systemic examination found no evidence of other primary site or metastasis. Following aggressive surgical resection, the patient received intensive chemotherapy without radiotherapy. Nine months after surgery, there has been no recurrence of the tumor. The principles of the management in children with primary cranial Ewing's sarcoma are discussed.
Brain ; Child ; Drug Therapy ; Humans ; Intracranial Pressure ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Sarcoma, Ewing* ; Skull ; Temporal Bone

Ewing's sarcoma is rarely found occurs in the bones of the hands and feet. We report a case of Ewing's sarcoma of the left calcaneus in a 15-year-old girl who complained of left heel pain and swelling. An open biopsy was performed and histological examination showed the proliferation of uniform small round cells. Immunohistochemical staining for CD99 showed diffuse strong positivity in the cytoplasmic membrane of the tumor cells. After preoperative chemotherapy, a below knee amputation was performed.
Adolescent ; Amputation ; Biopsy ; Calcaneus* ; Cell Membrane ; Drug Therapy ; Female ; Foot ; Hand ; Heel ; Humans ; Knee ; Sarcoma, Ewing*

Primary bone tumors in pediatric age group are uncommon, and even when they do occur, they are usually benign. The primary malignant tumors that occur predominantly in children are two bone tumors, namely, osteosarcoma and Ewing's sarcoma. An adequate history and physical examination are the first and most important steps in evaluating a patient with a bone tumor. All suspected bone tumors should be evaluated initially with plain roentgenograms. Then the additional diagnostic studies, such as computed tomography(CT), magnetic resonance imaging(MRI) and technetium bone scan can be used, if necessary. Biopsy should be the last step in evaluation. Most of benign bone tumors usually do not require treatment other than a periodic follow-up evaluation. The optimal treatment of the malignant bone tumor often requires a combination of radiation therapy, chemotherapy, and wide surgical excision or amputation. Early detection of a malignant bone tumor not only may make the difference between life and death but also may allow successful salvage surgery rather than amputation of the limb.
Amputation ; Biopsy ; Child* ; Drug Therapy ; Extremities ; Follow-Up Studies ; Humans ; Osteosarcoma ; Physical Examination ; Sarcoma, Ewing ; Technetium

Extraskeletal Ewing's sarcomas (EES) are rare. Recently, Ewing's sarcoma of the bone, primitive neuroectodermal tumor (PNET), Askin tumor and EES have been included into the family of Ewing's tumors, due to the overlapping features relating to their clinico-pathological and cytogenetic appearance. We experienced a case of an EES arising from the duodenum in a 14-year-old girl who presented with hematemesis and epigastric discomfort. A duodenal biopsy specimen revealed the infiltration of small round cells and rich vasculatures, with immunohistochemical finding of MIC-2 (CD99) (+), vimentin (+), CD56 (NCAM) (+), LCA (-), T-cell (-), B-cell (-), CD43 (-) and CD68 (-). She was treated with several cycles of multiagent chemotherapy, and achieved an initial partial response, but rapid progression of tumor followed, so she was treated with surgical excision. This is the first case report of an EES arising from the duodenum in the literature.
Adolescent ; B-Lymphocytes ; Biopsy ; Cytogenetics ; Drug Therapy ; Duodenum* ; Female ; Hematemesis ; Humans ; Neuroectodermal Tumors, Primitive ; Sarcoma ; Sarcoma, Ewing* ; T-Lymphocytes ; Vimentin

A total 73 cases of primary malignant bone tumors was reviewed and analysed clinically at the department of orthopaedic surgery, Kosin medicsl center, Pusan, Kores for 11 years from January, 1975 to December, 1985. The results were obtained as follows ; l. In the 73 cases of primsry malignant bone tumors, osteogenic sarcoma was the most common primary malignant bone tumor (57%) and followed by chondrossrcoma (10%), multiple myeloma (8%). 2. Average survival times according to each primary malignant bone tumors was more than 3 years in chondrosarcoma, reticulum cell sarcoma, and synovial sarcoma, 28 months in osteogenic sarcoma, and 7 months in Ewings sarcoma. Ewings sarcoma had the worse prognosis and the slowly progressing tumors-chondrosarcoma, reticulum cell sarcoma and synovial sarcoma are needed long term follow up. 3. In osteogenic sarcoma, the prognosis was better when developed in their 3rd decsde than when developed in their 2nd decade. 4. There is a slight difference in average survival time on the location of the site, for example when tumor is located in the distal femur, the prognosis was worst. 5. There is no difference in the prognosis. The mode of treatment did not effect to their prognosis. 6. It was clear that the tumors which had not been responded to chemotherapy or radiation therapy had poorer prognosis. Chondrosarcoma, fibrosarcoma and synovisl sarcoma were considered as slowly progressed tumors, and so it may be benefit to the patients that chemotherapy and/or radiotherapy were prescribed.
Busan ; Chondrosarcoma ; Drug Therapy ; Femur ; Fibrosarcoma ; Follow-Up Studies ; Humans ; Lymphoma, Non-Hodgkin ; Multiple Myeloma ; Osteosarcoma ; Prognosis ; Radiotherapy ; Sarcoma ; Sarcoma, Ewing ; Sarcoma, Synovial

PURPOSE: Fusion genes(EWS-Fli-1 and EW.S-erg) function as transcription activators and are essential for maintaining tumorigenic properties in Ewing's sarcoma cells. Several reports have noted that Ets family transcription factors bind with CBP(CREB binding protein) in vitro. To understand the interaction of fusion proteins and CBP, we studied the CBP protein in TC135 cells expressing the EWS-Fli-1 gene. We also studied the hypothesis that downregulation of fusion gene expression may induce susceptibility to apoptosis in Ewing's sarcoma cells. METHODS: For targeting fusion proteins, we reconstructed the antisense EWS-fli-l, EWS-erg and CBP genes in pcDNA3, and transfected these genes to Ewing's sarcoma cells showing high levels of expression for Ve3 and 5838 genes. These vectors were transfected to cells by the calcium phosphate method, and transformed cells were selected using G418. We measured DNA fragments for apoptosis using FACScan. We used crystal violet staining and MTT assay to evaluate cell viability, and Western blot analysis was used to assess CBP gene expression. RESULTS: Cells transfected with antisense fusion genes Ve3 and 5838 showed inhibition of fusion protein expression. These cells also showed decreased cell viability. Susceptibility to apoptosis was induced by treatment with chemotherapeutic agents at low concentrations. Antisense CBP- transfected cells showed loss of cell viability in O.l% and 0.5% serum. This loss of cell viability was similar to the response by antisense fusion protein-transfected cells treated with chemotherapeutic agents at low concentrations. CONCLUSION: Our results suggest that fusion proteins and CBP co-regulate apoptosis in Ewing's sarcoma cells. Antisense fusion gene therapy may be an useful adjunct in combining with chemotherapeutic regimens to downregulate the expression of fusion proteins in Ewing's sarcoma.
Apoptosis* ; Blotting, Western ; Calcium ; Cell Survival ; DNA ; Down-Regulation ; Gene Expression ; Genetic Therapy ; Gentian Violet ; Humans ; Sarcoma, Ewing* ; Transcription Factors