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.     

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.     

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.     

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.     

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.     

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.     

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.     

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 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.     

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.     

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.     

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.     

Primary Ewing sarcoma: follow-up with Ga-67 scintigraphy

While avid accumulation of gallium-67 citrate and technetium-99m methylene diphosphonate (MDP) occurs initially in most cases of primary Ewing sarcoma, uptake after therapy is less well defined. Thirty patients with Ewing sarcoma who underwent Ga-67 and bone scintigraphy at diagnosis, at completion of therapy, and at relapse from 1978 to 1988 were evaluated. All 30 patients showed less primary site Ga-67 activity following therapy. Twenty-three of 28 patients who underwent corresponding bone scintigraphy showed less uptake, but residual activity was usually more intense than with Ga-67. Avid reaccumulation of Ga-67 occurred in four of five patients with primary site relapse, while patients who underwent bone scintigraphy showed less change. It was concluded that a greater decrease in Ga-67 than in Tc-99m MDP uptake often occurs in patients successfully treated for primary Ewing sarcoma. Information obtained at Ga-67 scintigraphy is most likely to be helpful if results of bone scintigraphy remain abnormal or if occult relapse is suspected.     

股骨骨骺Ewing瘤一例

患者男,15岁.右腿疼痛半年,肿胀1个月余.体检发现右膝关节软组织肿胀,局部皮肤发红,皮温升高,有压痛.实验室检验血白细胞增高,关节镜检查诊断为化脓性关节炎.X线检查:右股骨内髁密度减低,边缘非承重面见溶骨性骨质破坏,膝关节面光整,关节间隙未见异常(图1).CT检盎:右股骨骨骺髓腔密度减低,边缘骨皮质模糊中断,可见溶骨性骨质破坏.无骨膜反应,周围软组织肿胀明显(图2,3).影像诊断:右膝关节结核可能大.手术后病理切片见大小形态一致的小圆形细胞(图4),免疫组织化学染色:CD99阳性,突触素(Syn)阳性.诊断为Ewing瘤.     

Limited remodelling of expansile Ewing sarcoma after treatment.

Radiological findings of Ewing sarcoma have been well documented, and bone expansion or cystic change is known to be a relatively uncommon finding. On the other hand, expected changes in Ewing sarcoma after treatment have also been reported, e.g. regression of extraosseous soft tissue mass, regression and organization of the periosteal reaction, and remodelling of the lytic or sclerotic bone changes. However, remodelling of bone is often limited or incomplete. We saw an atypical change in Ewing sarcoma after treatment due to lack of significant remodelling of the expanding bone. Because such changes might be a cause of misdiagnosis, knowledge of it will help the radiologist in the follow up evaluation.