Diagnosis of osteosarcoma in a patient previously treated for Ewing sarcoma

Primary malignant bone tumors, whether Ewing sarcoma or osteosarcoma, are a rare type of tumor. The sequential occurrence of two bone sarcomas, Ewing sarcoma and high-grade osteosarcoma, in the same patient at two different locations is an exceptionally rare phenomenon. We present the case of a 13-year-old girl who presented with a high-grade osteoblastic osteosarcoma of the distal femur, 7 years after treatment for Ewing sarcoma of the left pelvis. She did not receive radiation therapy. Following the recent developing multidisciplinary therapy, long-term follow-up for monitoring latent treatment-related adverse effects may be necessary for survivors of primary malignant bone tumors.     

Primary Ewing sarcoma of rib

Ewing sarcoma is a relatively common, highly malignant bone tumor that typically occurs in adolescents and young adults aged 10-25 years. Our archives contain 328 cases of histologically proved and radiologically correlated Ewing sarcoma collected in consultation over 40 years. From this series, we identified 34 lesions (10%) arising in ribs. Radiographically, the affected rib was predominantly lytic in most (82%) cases, but mixed lytic-sclerotic (9%) and even predominantly sclerotic (9%) patterns were also encountered. The affected rib was "expanded" in 35% of cases, although the contour change was usually mild. Abnormalities of the affected ipsilateral hemithorax varied from subtle, isolated rib involvement to solitary rib involvement accompanied by complete opacification of the hemithorax. We describe the spectrum of radiologic findings of primary Ewing sarcoma of rib, augmented where appropriate by accompanying pathologic material.     

Ewing’s sarcoma of the thumb

 The case of a 51-year-old man with Ewing’s sarcoma of the thumb is presented. The tumor involved the distal phalanx of the right thumb, associated with an impressive extraskeletal mass. Histology revealed a round cell sarcoma with a positive immunoreactivity with monoclonal antibody O13. Five years after disarticulation at the metacarpophalangeal joint, the patient is alive without recurrence or metastasis.     

Ewing’s sarcoma of the larynx

Extraskeletal Ewing’s sarcoma arising in the head and neck region is an extremely rare malignant neoplasm. We describe the unusual case of a tumor originating in the larynx of a 68-year-old male with hoarseness and occasional aphonia. We report successful treatment with sequential chemo- and radiotherapy. Complete regression and larynx preservation with voice function recovery was achieved. To our knowledge, this is the first report of this type of tumor in the larynx with cartilage invasion that documents the effectiveness of radiotherapy as an alternative to surgical management. At present, after 30 months of follow-up, the patient is free of tumor relapse and in very good condition.     

Ewing’s sarcoma of the sacrum

 A lytic lesion with soft-tissue extension in the sacrum of a 47-year-old man was needle-biopsied under computed tomographic (CT) guidance using an 18-gauge cutting needle. The cytologic appearance of the lesion and immunohistochemical staining were diagnostic of Ewing’s sarcoma. Specifically, a new marker (O13) for the presence of glycoprotein p30/32 mic2 in Ewing’s sarcoma was utilized.     

Extraskeletal Ewing sarcoma of the buccal space

Extraskeletal Ewing sarcoma (EES) is a rare soft tissue tumor, and EES of the head and neck is particularly rare. Radiographic imaging of these lesions is crucial given their anatomical complexity and infrequent incidence. Conventional EES imaging features include a large, hyperintense, heterogeneously enhancing lesion, with frequent invasion of local structures. In this case report, a 19-year old male presented with left facial swelling and pain. He underwent sclerotherapy and bleomycin treatment for a presumed lymphatic malformation. Initial imaging demonstrated a rim-enhancing lesion within the left buccal space with no muscle invasion or bony erosion present. Two years later, imaging identified an enlarging buccal mass with destruction of the zygomatic arch and inferolateral orbital wall. The patient underwent surgical resection of the mass. Pathology confirmed the mass to be a small round blue cell tumor and FISH testing confirmed the presence of the EWSR1 gene arrangement that is consistent with EES. The patient tolerated the procedure well and underwent chemoradiation therapy. At three years postoperatively, the patient remains disease free. The presented case demonstrates an unusual presentation of a buccal space EES as a rim-enhancing, centrally hypointense mass with no bony erosion or muscular invasion. The location and atypical imaging appearance of this case offer insight for future diagnosis of EES.     

Radiological features of extraskeletal Ewing sarcoma

The radiological features of extraskeletal Ewing sarcoma were reviewed in 22 patients whose average age was 22 years. Tumours were located in the extremities (11 patients), abdomen or pelvis (six patients) and the chest (five patients). The tumours ranged in size from 2 cm to 20 cm, were mainly well circumscribed and showed no evidence of calcification prior to treatment. Most tumours (13 out of 14) were of low attenuation or contained areas of lower attenuation than muscle on computed tomographic examination, and in six out of seven patients studied by ultrasound the tumours were hypoechoic or partly anechoic. No distinctive post-contrast medium enhancement pattern on CT examination (11 patients) or angiographic features (three patients) were evident. Tumour haemorrhage was a frequent microscopic finding and changes consistent with this were present in one patient on magnetic resonance imaging examination. Distant metastases or local recurrence developed in 13 patients with lung being the most frequent metastatic site (eight patients). Although its radiological features are non-specific, extraskeletal Ewing sarcoma should be included in the differential diagnosis of noncalcified soft-tissue tumours especially in a young age group and where located in an extremity or paravertebral region of the chest.     

Spinal Ewing sarcoma: Misleading appearances

The plain radiographic and computed tomographic (CT) findings in two unusual cases of spinal Ewing sarcoma are reported. Radiographic features resembling neuroblastoma in one case and aneurysmal bone cyst in the other were present. These findings may be misleading and distinguishing characteristics in each case are discussed.     

肾尤文肉瘤一例

患者男,26岁.右侧腰背部间隙性胀痛2周,于2009年11月10日入我院就诊.B超示右肾实质性占位性病变. CT检查:平扫右肾于肾门上方体积增大,见混杂密度影,高密度血块CT值68 HU,等密度肿瘤实质CT值35 HU,肾包膜下见半月形低密度影,未凝固的血液CT值14 HU.     

MR of primary extraosseous Ewing sarcoma.

We present MR imaging findings of a 15-year-old girl with a history of chronic back pain, proved by biopsy to be caused by primary extraosseous Ewing sarcoma, extending into the spinal epidural space. We also demonstrate the involvement of the adjacent structures and encroachment on the intervertebral foramina.     

Radiological findings of primary retroperitoneal Ewing sarcoma

Ewing sarcomas are most commonly located in bone, while extraskeletal involvement of the retroperitoneum is extremely rare. We describe the radiologic and pathological findings in an adult patient with retroperitoneal extraskeletal Ewing sarcoma.     

Extraskeletal Ewing sarcoma with computed tomography correlation

Extraosseous Ewing sarcoma — a rare clinical entity — is described and the radiographic and histologic features discussed. Histologically this type of tumor may be differentiated from other soft tissue malignancies, but the ultrastructural glycogen content of the cytoplasm makes it frequently inseparable from the conventional osseous form of Ewing sarcoma. In spite of the presence of a huge paraosseal mass with bone erosion or periosteal new bone, computed tomography was the only preoperative modality able to provide evidence of non-involvement of the marrow cavity. However, histologic documentation is necessary to prove that one is dealing with a totally extraosseous Ewing sarcoma.     

Imaging of primary Ewing sarcoma with 13N-L-glutamate

Eleven patients with untreated primary Ewing sarcoma were studied with intravenously administered 13N-labeled L-glutamate. Seven were repeatedly scanned during chemotherapy using this agent and 99mTc-methylene diphosphonate (99mTc-MDP). The untreated primary tumor was distinctly visualized with 13N-L-glutamate in all cases; the distribution of 13N label in the tumor sometimes differed from that of 99mTc. A kinetic study showed rapid uptake of 13N by tumor tissue. Repeat scans following therapy indicated that 13N-L-glutamate and 99mTc-MDP uptake showed changes consistent with histological findings following subsequent surgery. 13N uptake often decreased more markedly than 99mTc uptake during chemotherapy, but metastatic lesions were not visualized with 13N-L-glutamate. Tumor imaging with this labeled amino acid may be of value in assessing the response of primary Ewing sarcoma to chemotherapy.     

颈椎尤文肉瘤一例

患者男,15岁.2007年3月10日因颈肩部疼痛伴右上肢麻木5个月就诊.体检:颈肩部压痛,颈椎活动受限,右手握持力减弱.     

Radiographic appearance of Ewing sarcoma of the hands and feet: report from the Intergroup Ewing Sarcoma Study

Review of current data from the Intergroup Ewing Sarcoma Study (IESS) shows that Ewing sarcoma is rare in bones of the hands and feet. The 12 patients from the IESS protocols with hand or foot Ewing sarcoma are comparable to those already reported in the literature. With the exception of lesions in the calcaneus, the prognosis for disease-free survival is excellent. The radiographic features of hand and foot Ewing sarcoma are generally those of classic Ewing sarcoma: permeation, soft-tissue mass, and, often, associated sclerotic reaction. However, with the exception of sclerosis, features suggesting bone reaction and slow tumor growth in these patients were distinctly uncommon compared with Ewing sarcoma in general. Those radiographic features suggesting aggressive behavior were slightly more common than in the general Ewing population. Even so, neither the radiographic appearance nor the greatest dimension of the radiographic lesion can be shown to contribute significantly to prognostication in these patients. Apparently location of the lesion is important, since in the reported cases in the literature and in this series, lesions of the calcaneus fared poorly. The importance of this set of patients therefore relates to awareness and early recognition of an unusual appearance and location of Ewing sarcoma.     

Ewing sarcoma: changes after radiation and chemotherapy

We retrospectively studied 52 cases of Ewing sarcoma to elucidate the pattern of healing and/or recurrence after radiation and chemotherapy. Persistent soft tissue mass was a sign of failure to respond to therapy. Repair or healing of lytic or blastic bone changes were slow and often minimal, and usually bone changes became gradually stable over 3 years. After bone changes became stable, localized new bone changes represented recurrence or secondary malignant neoplasms.