Ewing's sarcoma

This patient had Ewing's sarcoma of the femur, which was originally misinterpreted by radiograph and by biopsy as osteomyelitis. For approximately 1 year the patient was treated for osteomyelitis. The lesion was slowly progressive and near the end of the first year became more active, and pulmonary metastases appeared. The natural behavior of this one case of Ewing's sarcoma demonstrates that it is not necessarily an overwhelmingly aggressive tumor. In addition, the point is made that pathologists should pay careful attention to necrotic material so that the suggestion of necrotic tumor can be made rather than dismissing the biopsy as nondiagnostic.     

Isolated CNS involvement in Ewing's sarcoma

Ewing's sarcoma, an uncommon malignant neoplasm of bone, represents about 10% of all the malignant primary bone tumors. The assumption that subclinical metastases are already present in patients with apparently localized tumor indicates the need for systemic therapy as an integral part of primary treatment. The usual sites of metastases are the lungs and skeletal system. Central nervous system (CNS) involvement is rare and is usually seen only in disseminated and fairly advanced disease. We report two patients in whom, after aggressive adjuvant chemotherapy, disease developed in the CNS without local recurrence. In one patient, meningeal involvement with malignant cells was identifiable in the cerebrospinal fluid; in the second patient, who presented with a space-occupying lesion, a diagnosis of arachnoid involvement was made histologically.     

Extraskeletal Ewing's sarcoma of primary cardiac origin

Summary A 13-year-old boy presented with cardiac tamponade. Echocardiography revealed a large mass extending from the right and left ventricles into a large pericardial effusion. Pathology confirmed the first reported case of a primary cardiac extraskeletal Ewing's sarcoma.     

尤文肉瘤家族治疗进展

尤文肉瘤家族(Ewing's sarcoma family tumor,ESFT)是儿童常见的恶性骨肿瘤,具有侵袭性、溶骨性和明显传播倾向.目前多学科综合治疗方法使患儿预后得到了显著改善.但即使积极治疗,仍有20％ ～40％的原发肿瘤患儿和近80％的转移肿瘤患儿难获显著疗效.随着生物靶向治疗的研究进展,一些晚期尤文肉瘤...     

Late metastasis from neuroblastoma mimicking Ewing's sarcoma

The case of an unusual late metastasis of neuroblastoma to the calcaneum, appearing as a sole recurrence 9 years after surgery, is reported. The radiographic features of the osseous lesion were very suggestive of primary bone malignancy, Ewing's sarcoma as the first option, with extraosseous spread to the soft tissues.     

Magnetic resonance imaging of primary orbital Ewing's sarcoma

The authors describe the MRI findings of a primary orbital Ewing's sarcoma in a 5-year-old boy, who underwent an extensive tumour surgery as these findings were mistaken for more chemoresistant sarcomas such as rhabdomyosarcoma. This case illustrates the fact that MR findings of primary orbital Ewing's sarcoma may be different from those of Ewing's sarcoma present elsewhere in the body. An accurate preoperative diagnosis may therefore result in a more conservative approach and prove extremely useful in such tumours.     

Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall

Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents. It is extremely malignant with a high frequency of both metastatic spread and of local recurrence. Cure requires intensive therapy to control both distant and local disease. Surgery and high-dose radiotherapy can achieve equivalent local control; however, radiation is associated with the additional morbidities of second malignancy and a significant adverse impact on both cardiac and pulmonary function. The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor. This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy. The chest wall is a rare site for tumors in children and adolescents. In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors. They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate. This report concentrates on the later tumors. They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve. Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.     

Acute nonlymphocytic leukemia developing during the course of Ewing's sarcoma

Two children with Ewing's sarcoma developed acute nonlymphocytic leukemia (ANLL) during the course of their illness. One patient developed ANLL after apparently successful treatment of his primary malignancy with radiation therapy and multiagent chemotherapy. In the second patient, acute leukemia developed before the administration of radiotherapy or systemic chemotherapy. The development of secondary ANLL after Ewing's sarcoma has been reported only twice previously, most likely representing a therapy-induced complication. The occurrence of ANLL in Patient 2 prior to therapy suggests that these two disorders may have a more than treatment-related association. Close follow-up of long-term survivors of Ewing's sarcoma with surveillance for secondary acute leukemia is advised.     

Ewing's sarcoma in the occipital bone. Case report.

The head is a very rare primary site for Ewing's sarcoma which occurs most often in the long bones of the extremities and in the pelvis. This report describes an unusual case of Ewing's sarcoma arising from the occipital bone in a seven year old girl. The tumour compressed the venous sinuses, thus lowering the intracranial pressure resulted in temporary recovery which made the diagnosis difficult.     

Solitary ivory vertebra due to primary Ewing's sarcoma

Dense sclerosis of most of the vertebral body has been termed as ivory vertebra. While this condition occurs rather frequently in adults it is not found very often in children.