Primary bone tumours of the pelvis in childhood--Ewing's sarcoma of the ilium, pubis and ischium (report of 30 cases). (Part I)

30 children with Ewing's sarcoma, the most common malignant pelvic tumour in childhood, were analysed. The diagnosis of Ewing's sarcoma is relatively easy and can be established in most of the cases on plain radiography. The diagnostic radiographic features of the tumour are discussed. The two most important conditions in differential diagnosis are eosinophilic granuloma and the rare primary bone lymphoma. Osteomyelitis should rarely cause confusion unless the clinico-radiographic findings are not properly evaluated.     

Spinal cord Ewing’s sarcoma metastasis: presentation of one case

Cervical spinal Ewing's sarcomas are rare and cause problems in diagnosis. We present an unusual case of a primary extraosseous Ewing's sarcoma arising from the spinal cord. An 18-year-old woman with fever, headache and back pain lasting one month was admitted to the hospital. Whole body bone scintigraphy was performed with 1110 MBq technetium-99m methylenediphosphonate. Scintigraphy clearly showed abnormal technetium-99m methylenediphosphonate accumulation in the level of the 5th and 6th cervical vertebrae. Magnetic resonance imaging could also confirm this examination finding. After the scintigraphic study, the patient underwent surgery. Pathological diagnosis of the operation specimen was Ewing's sarcoma.     

Intracranial dural metastasis of Ewing's sarcoma: a case report.

Although intracranial dural metastasis of Ewing's sarcoma is a very rare finding, its imaging characteristics are similar to those of its primary form in the central nervous system. Thus, this tumor must be considered in the differential diagnosis of extra-axial dural masses.     

Ewing's sarcoma of the sacrum

Radiological findings in a case of spinal Ewing' s sarcoma are reported. A lytic lesion with soft tissue component in the sacrum was identified. Ewing's sarcoma should be included in the differential diagnosis, especially when a child has a lytic lesion with soft tissue extension in the spine.     

Extramedullary Ewing's sarcoma

The authors refer to six observations of periostal Ewing's sarcoma and describe its X-ray symptomatology. It differs considerably from the commonly known picture of centrally, intramedullar-originated Ewing sarcomas and tumours, diagnosed at considerable advanced stage. The possibility of periosteal origin of Ewing's sarcoma must be considered in differential diagnosis of periostoses and the patients must be examined in a complex way, particularly by computer tomography (and in the future also by MR tomography) in order to demonstrate that the medullar space of the bone is not affected by the tumour. This enables a more considerate resection during the operation. Histological verification of the nature of the tumour and excluding presence of the tumour cells in bone marrow dissection material is necessary. It may be expected that prognosis of this form of Ewing's sarcoma while treated in a modern combined way is more favourable than in the central forms.     

Meningeal hemangiopericytoma in childhood

Meningeal hemangiopericytoma (MHP) is extremely rare in childhood. Mean age at diagnosis is between 38 and 43 years. We present an 8-year-old boy with MHP of the middle cranial fossa. Imaging findings were indistinguishable from an aggressive bone tumor such as Ewing's sarcoma. Imaging findings are presented and discussed. Our case indicates that MHP should be considered in the differential diagnosis of skull-base tumors despite the fact that MHP is extremely rare in childhood. Received: 8 July 1999; Revised: 28 September 1999; Accepted: 29 September 1999     

尤文氏肉瘤的X线及MRI诊断

目的：探讨尤文氏肉瘤的影像诊断及提高其影像诊断水平。方法：回顾性分析18例经病理证实的尤文氏肉瘤的X线、MRI表现。结果：18例尤文氏肉瘤中,11例表现为斑片状、虫蚀样的溶骨性骨质破坏,3例可见斑片状骨质硬化影,10例可见葱皮状或Codman三角状骨膜反应,14例可见明显软组织肿块,2例为骨外尤文氏肉瘤,表现为单纯软组织肿块,未见明确骨质破坏。结论：X线平片结合MRI能提高其诊断准确性。     

Radiotherapy and High-Dose Chemotherapy in Advanced Ewing's Tumors

BACKGROUND: Ewing's tumors are sensitive to radio- and chemotherapy. Patients with multifocal disease suffer a poor prognosis. Patients presenting primary bone marrow involvement or bone metastases at diagnosis herald a 3-year disease-free survival below 15%. The European Intergroup Cooperative Ewing's Sarcoma Study (EICESS) has established the following indications for high-dose therapy in advanced Ewing's tumors: Patients with primary multifocal bone disease, patients with early (< 2 years after diagnosis) or multifocal relapse. PATIENTS AND METHOD: As of 1987, 83 patients have been treated in the EICESS group, 39 of them at the transplant center in Düsseldorf, who have been analyzed here. All individuals received 4 courses of induction chemotherapy with EVAJA and stem cell collection after course 3 and 4. Consolidation radiotherapy of the involved bone compartments was administered in a hyperfractionated regimen 2 times 1.6 Gy per day, up to 22.4 Gy simultaneously to course 5 and 22.4 Gy to course 6 of chemotherapy. The myeloablative chemotherapy consisted of melphalan and etoposide (ME) in combination with 12 Gy TBI (Hyper-ME) or Double-ME with whole lung irradiation up to 18 Gy (without TBI). RESULTS: The survival probability at 40 months was 31% (44% DOD; 15% DOC). Pelvic infiltration did not reach prognostic relevance in this cohort. Radiotherapy encompassed 75% of the bone marrow at maximum (average 20%). Engraftment was not affected by radiotherapy. CONCLUSION: High-dose chemotherapy can improve outcome in poor prognostic advanced Ewing's tumors. The disease itself remains the main problem. The expected engraftment problems after intensive radiotherapy in large volumes of bone marrow can be overcome by stem cell reinfusion.     

Radiographic features of Ewing’s sarcoma of the bones of the hands and feet

The radiographic features of Ewing's sarcoma of the bones of the hands and feet are reviewed utilizing cases obtained from the Mayo Clinic patient files and the consultation files of Drs. D.C. Dahlin and K.K. Unni. This series consists of a total of 43 cases of pathologically proven Ewing's sarcoma involving the small bones of the hands and feet. The classic radiographic features of Ewing's sarcoma in the long bones, including lytic, permeative destruction, aggressive periosteal reaction, cortical violation, and a soft tissue mass, are also seen in the bones of the hands and feet, with similar frequency. These classic features are most commonly present in lesions affecting the short tubular bones. Lesions affecting the tarsal bones more often demonstrate atypical radiographic features. These atypical radiographic appearances may play a role in the reported delay in diagnosis of Ewing's sarcoma within the tarsal bones.     

Roentgen diagnosis of structural changes in the pubic area in children and adolescents. Personal case reports, differential diagnosis and literature review

Structural changes in the pubic bone often are difficult to interpret. The radiologic appearance is very different. The differentiation between inflammatory and malignant processes is hardly to distinguish. We are reporting ten patients with osseous changes in the os pubic (4 Ewing's sarcoma, 1 osteosarcoma, 1 Morbus Hodgkin, 1 osteochondronecrosis, 2 osteochondritis, 1 subchronic osteomyelitis). Two patients with Ewing's sarcoma who were initially treated for osteomyelitis are described in detail. Differential diagnosis and previous literature are reviewed.     

Computed tomography in the evaluation of 41 cases of Ewing's sarcoma

Computed tomography (CT) has already proved extremely effective both in cerebral and abdominal pathology. Several recent publications describing first heterogeneous series [1, 2, 7, 11–17], then studies of a single type of lesion [3–6, 8] have illustrated its usefulness in the study of bone lesions. This report deals with 41 cases of Ewing's sarcoma studied by CT at the Institut Gustave Roussy from October 1977 to July 1981, and tries to show both the limitations and indications of this technique for the diagnosis, treatment, and follow-up of Ewing's sarcoma as well as in the diagnosis of any eventual recurrence.     

Musculoskeletal lymphomas

Primary lymphomas of bone or skeletal muscle are rare entities. The most frequent among these diseases are primary non-Hodgkin's lymphomas of bone. They account for 3-5% of all bone tumors and 5% of all primary extranodal non-Hodgkin's lymphomas. Primary manifestations of Hodgkin's disease in bone or skeletal muscle are rarities. Primary non-Hodgkin's lymphomas of skeletal muscle are rarities as well.Primary non-Hodgkin's lymphomas of bone can be found in any patient age. A preference exists for the 3.-6. decade of life. The radiographic appearance of these entities resembles other aggressive bone tumors.Their differential diagnosis includes -- depending on the patient's age -- Ewing's sarcoma,malignant fibrous histiocytoma,metastases of small cell tumors and osteomyelitis.Further differential diagnoses are the peripheral primitive neuroectodermal tumor (PNET), osteosarcoma, eosinophilic granuloma and fibrosarcoma.Treatment of primary non-Hodgkin's lymphomas uses combinations of chemotherapy and radiation therapy.Operative treatment is reserved for the treatment of complications.The prognosis of primary non-Hodgkin's lymphomas is reflected by 10-year-survival-rates without recurrence of more than 80% in unifocal manifestations.     

Bone marrow oedema associated with benign and malignant bone tumours

Bone marrow oedema is associated with a wide variety of pathological processes including both benign and malignant bone tumours. This imaging finding in relation to intraosseous tumours can aid in providing a more focused differential diagnosis. In this review, we will discuss the MR imaging of bone marrow oedema surrounding intraosseous neoplasms. The different pulse sequences used in differentiating underlying tumour from surrounding oedema are discussed along with the role of dynamic contrast enhanced MRI. Benign lesions commonly associated with bone marrow oedema include osteoid osteoma, osteoblastoma, chondroblastoma and Langerhan's cell histiocytosis. Metastases and malignant primary bone tumours such as osteosarcoma, Ewing's sarcoma and chondrosarcoma may also be surrounded by bone marrow oedema. The imaging findings of these conditions are reviewed and illustrated. Finally, the importance of bone marrow oedema in assessment of post chemotherapeutic response is addressed.     

Primary sacral bone tumours in children (report of 16 cases with a short literature review)

16 cases of primary sacral bone tumours in children are reported. These include 13 patients with Ewing's sarcoma and 3 with very rare primary sacral bone tumours in childhood--chordoma, haemangiopericytoma and osteoblastoma. All sacral bone tumours, with the exception of Ewing's sarcoma are very rare in childhood. The possibility of a sacral tumour should be considered in a child with radiculopathy. CT and MR make the diagnosis of primary sacral bone tumours much easier with the added possibility of recognition of the true nature of the lesion in many instances. Reports of primary sacral bone tumours in children are scarce. Most of the patients are incorporated in adult series which do not specify the age of the child and the site of the tumour. The purpose of this paper is to describe 16 children with primary sacral bone tumours.     

儿童骨骼尤文肉瘤的影像学诊断和鉴别诊断

目的 探讨儿童骨骼尤文肉瘤在X线平片、CT和MRI上的影像学征象，以提高对其诊断和鉴别诊断能力。资料与方法 回顾性分析经临床病理证实的儿童骨骼尤文肉瘤11例，其中X线平片检查7例，单行MRI扫描2例，单行CT扫描4例，同时行MRI及CT扫描者4例。结果 11例尤文肉瘤中发生于骨盆4例，股骨3例，胫骨、肋骨、趾骨和脊柱各1例，其中胫骨和髂骨各1例术后出现颅骨转移。X线平片上6例表现为虫蚀状或溶骨性破坏，2例病灶略呈膨胀性改变，骨皮质破坏伴单层、多层或放射状骨膜反应，邻近软组织层次欠清；发生于肋骨的1例以硬化为主，CT像上髓腔密度增高，骨质鼠咬状或斑片状破坏伴骨皮质裂隙状或不规则破坏；2例骨皮质呈膨胀、变薄、中断，周围见不完整的层状骨膜反应及较大软组织肿块；MRI上表现为大片长T1长T2信号的骨质破坏和巨大的软组织肿块；1例肿块呈跨关节生长。结论 X线平片、CT和MPI在骨尤文肉瘤的评估方面有重要作用。X线平片可有助于肿瘤的定性和定位；MRI平扫T1WI像及短反转时间反转恢复技术(STIR)能清晰显示肿瘤的范围，在显示肿瘤对骨骺及邻近软组织的侵犯方面尤为敏感；CT在显示骨及骨皮质破坏等方面优于MRI。     

Computerized tomography: problems and errors in the diagnosis of renal masses

The diagnostic imaging of renal masses has greatly benefitted from US and CT. Nevertheless, a certain number of problems and errors are still present, and they must be discussed once more. In the authors' experience, problems and errors have 3 fundamental causes: operator, examination technique and complex patterns of renal masses. The latter is the most important cause of error in CT diagnosis. Complex patterns are seen in a limited number of cystic masses--i.e., hemorrhagic, inflammatory, and septated cysts, and cystic tumors. Hemorrhagic cysts, if hemorrhage is not recent, can cause some diagnostic difficulties since the typical high density of the recent hemorrhage is no longer present. Other renal masses may appear hyperdense on unenhanced CT scans, and therefore they must be considered in the differential diagnosis. Inflammatory cysts and abscesses are sometimes difficult to differentiate from other masses due to their aspecific appearance; clinical correlation is important in these instances to support CT diagnosis. Multiseptated cysts and cystic tumors cause huge problems of differential diagnosis. A good knowledge of differential CT findings is sure to reduce the problems in differentiating benign from malignant tumors and, among the latter, the different histotypes and metastases, when present as solitary masses. Also in this case, the correlation between CT, clinical history and other modalities can reduce the number of questionable cases.     

Primary spinal epidural extraosseous Ewing's sarcoma: report of five cases and literature review

Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinicopathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit--both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy.     

Tumoral thrombosis of inferior vena cava and iliac veins resulting in unilateral lower limb edema in a young adult: A rare presentation of skeletal Ewing's sarcoma

Association of venous thromboembolic events (TEs) with malignancy is well known. Ewing's sarcoma usually presents as swelling at the primary site, however presenting as unilateral lower limb edema due to tumoral thrombosis of right iliac vein and IVC is a rare event. A 30-year-old male patient with extensive right lower limb swelling was admitted and imaging studies (plain film, ultrasonography, Doppler, computed tomography) revealed a mass arising from right iliac bone, adjoining sacrum and tumoral thrombosis of right iliac veins extending into inferior vena cava. Histopathological diagnosis was Ewing's sarcoma. Patient was treated successfully with chemotherapy and surgical embolectomy. So a young patient presenting with deep vein thrombosis should be investigated not only to establish any thrombophilic pre-disposition, but also let the clinician to suspect for any local malignancy. Treatment depends upon the proximal extent of tumoral thrombus. With advances in imaging and chemotherapeutic regimens early diagnosis is possible and appropriate treatment planning improves the prognosis. To our knowledge skeletal Ewing's sarcoma with bland thrombosis is a known entity however no such case with direct invasion of tumor into veins leading to tumoral venous thrombosis has been reported in medical literature.     

Sonographic evaluation of chest masses in children.

The sonographic appearances of 12 intrathoracic masses in children are presented. Seven out of 12 masses were malignant and presented with opaque hemithorax on chest X-ray. Different types of masses encountered were: Lymphoma, Neuroblastoma, Ewing's sarcoma, metastatic Ewing's sarcoma, Teratocarcinoma, Pseudotumor of the lung, Neuroenteric duplication cyst, Bronchogenic cyst and tubercular mediastinal lymph nodes.     

Unilateral pulmonary metastases from Ewing's sarcoma shown in a technetium-99m-methylene-diphosphonate bone scan

A 32-year-old man with a history of painful swelling of the right ankle underwent bone scintigraphy, which showed increased uptake in the right ankle and also unexpected diffuse uptake throughout the right hemithorax. A single photon emission tomography scan performed after the intravenous injection of 740 MBq of technetium-99m methylene-diphosphonate ((99m)Tc-MDP) showed abnormal uptake throughout the right lung. Computed tomography (CT) revealed a large mass in the right lower lobe. CT-guided biopsy of this mass led to a diagnosis of metastatic Ewing's sarcoma. Although lung uptake on bone scans has been noted in various occasions (such as: pulmonary alveolar microlithiasis, Pneumocystis carinii pneumonia, and various tumoral lesions), increased uptake of (99m)Tc-MDP in lung metastases of Ewing's sarcoma has not been reported according to our knowledge until now. We report such a case.