Primary vertebral tumours in children

20 cases of primary benign and malignant bone tumours in children were reported. The most common tumours were Ewing's sarcoma, aneurysmal bone cyst, benign osteoblastoma and osteoid osteoma. Some rare primary bone tumours in children (osteochondroma, chondroblastoma?, primary lymphoma of bone and neurofibromatosis with unusual cervical spinal changes) were also reported. The authors believe that radiographic findings together with clinical history and clinical examination may yield a high percentage of accurate diagnoses. Although microscopy is essential in the final diagnosis, the microscopic report should be viewed with caution.     

Primary bone tumours in infants

Ten cases of primary bone tumours in infants (1 osteosarcoma, 3 Ewing's sarcoma, 1 chondroblastoma and 5 angiomatosis) are reported. All cases of angiomatosis showed characteristic radiographic findings. In all the other tumours the X-ray appearances were different from those usually seen in older children and adolescents. In the authors' opinion the precise diagnosis of malignant bone tumours in infancy is very difficult as no characteristic X-ray features are present in this age period.     

Rare malignant mid-femoral tumours in the first decade of life

The most common femoral shaft tumours in children are eosinophilic granuloma and Ewing's sarcoma. Three children in the first decade of life with rare femoral shaft tumours are reported. There was one osteosarcoma and two chondrosarcomas. Radiographic differential diagnosis of the femoral shaft tumours and microscopic diagnostic difficulties of chondrosarcoma are discussed.     

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 of the rib

In addition to eosinophilic granuloma, chondroma, osteochondroma, aneurysmatic bone cyst, and osteomyelitis, the differential diagnosis of primary costal tumors in children must also include Ewing's sarcoma. Radiologic diagnostic problems in localizing the tumor, in differential diagnosis, and the importance of imaging procedures are discussed with reference to a 16-year-old female patient.     

Improved detection of metastatic Ewing's sarcoma with the use of bone marrow scintigraphy

Prognosis in Ewing's sarcoma is inversely related to the extent of the disease at the time of presentation. The most common sites of metastases are the lungs and skeleton. Bone marrow metastases may be present but clinically silent. We report the use of Technetium (Tc)-99m bone marrow scintigraphy to detect sites of marrow involvement by metastatic Ewing's sarcoma. This method of evaluation allowed identification of sites of involvement by Ewing's sarcoma that were not available by any other method of evaluation. In several instances, information provided by this method was pivotal in the management of these patients. Based on this small series of patients, bone marrow scintigraphy appears to be a sensitive modality in the detection of metastatic disease in patients with Ewing's sarcoma. Better understanding of the role of bone marrow scanning and its correlation with other diagnostic procedures in Ewing's sarcoma will require further study.     

尤文肉瘤的诊断和治疗进展

尤文肉瘤是儿童常见的恶性骨肿瘤,发病时临床表现无特异性,早期易出现漏诊.目前的治疗方案包括放疗、手术治疗、化疗以及靶向治疗,其中化疗的出现使局部肿瘤的生存率达到70％,但难治及复发患者的预后不良.靶向治疗可针对肿瘤细胞的特异性位点杀死肿瘤细胞,有望提高复发及难治性肿瘤患者的生存率.该文就尤文肉瘤的诊断及治疗作一综述.     

Unilateral mid-femoral periosteal newbone of varying aetiology in children

Thigh pain is a serious condition in childhood and should be promptly and thoroughly investigated. Twenty-five cases of thigh pain lasting between 1 day and 7 months with mid-femoral periosteal thickening were analysed. There were 10 patients with Ewing's sarcoma, 7 with eosinophilic granuloma, 1 with osteoid osteoma, 1 with leukaemia, 3 with osteomyelitis, 1 with myositis and 2 with post-traumatic periosteal reaction. It was shown that patients with Ewing's sarcoma and eosinophilic granuloma have certain patterns of X-ray changes of high diagnostic value, which allow a proper diagnosis in most cases.     

Bone tumours in childhood and adolescence

Primary bone tumours are rare diseases in children and adolescence. Osteosarcoma and Ewing sarcoma are the commonest types of bone tumour in this age group. Whilst other rarer tumours occur their presentation and investigation are similar and their treatment will be individualised by specialist teams. We will therefore focus in on the challenges faced in the presentation, timely diagnosis, treatment and rehabilitation in young growing individuals with osteosarcoma and Ewing sarcoma.     

High-dose melphalan therapy for the treatment of children with refractory neuroblastoma and Ewing's sarcoma

Neuroblastoma and Ewing's sarcoma are examples of pediatric cancers in which disseminated disease is often present at diagnosis or develops later in spite of combination therapy. The demonstration that marrow-ablative doses of chemotherapy can increase tumor cell kill, and that autologous bone marrow can be cryopreserved and reinfused into the patient to reverse such marrow ablation, has stimulated interest in this approach to refractory childhood cancers. We present results of treating eighteen patients with recurrent neuroblastoma and Ewing's sarcoma resistant to conventional therapy. We used supralethal doses of melphalan, supported by reinfusion of previously cryopreserved autologous bone marrow. Seven of 10 neuroblastoma and six of eight Ewing's sarcoma patients had complete or partial responses, lasting for a median of 6 months (neuroblastoma) and 3 months (Ewing's sarcoma). Prolonged hospitalization, pancytopenia complicated by sepsis, and reversible gastrointestinal toxicity were the major side effects. These results suggest this approach should be tested in therapeutic trials at an earlier disease stage in children who have cancers with a predictably bad prognosis.     

Paraplegia in Childhood Malignant Disease

1. Five cases of paraplegia due to secondary malignant disease in children are described.
2. There was one case of generalized lymphosarcoma; one case of acute leukaemia who had recurrent neurological complications, paraplegia developing terminally; one case of metastatic Wilm's tumour; one case of Ewing's sarcoma; and one case in which the histological diagnosis was not confirmed, but was probably lymphosarcoma.
3. Tumours of the spinal cord and appendages are uncommon in children and are more often primary than secondary.
4. Spread to the spinal canal may be by direct extension from the abdomen or vertebrae or by blood-borne metastases.
5. The management of paraplegia includes care of the bladder, the bowels and of the skin.     

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.     

Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma

Twenty-one children with high-risk Ewing's tumor received high-dose chemotherapy with a PBSCT. Aim of the study was evaluation of efficiency and safety of this procedure. All but three patients have meta-static disease at presentation. There were 11 females and the median age at diagnosis was 12 yr (range 4.5-18 yr). Megachemotherapy consisted of melphalan 140 mg/m2/busulfan 16 mg/kg in 12 patients, melphalan 140 mg2/treosulfan 10.0 g/m2 in two patients and melphalan with other drugs in seven patients. Eight of 11 patients transplanted in CR survived with a median follow-up 24 month (range 14-60) and probability of 2-year OS is 0.68 and DFS is 0.63. There was no severe regimen-related toxicity in this group. Children transplanted without remission died: Two of them due to transplant related causes and eight had progression of disease in a median time 7 month after PBSCT. Megachemotherapy with PBSCT is a safe procedure in children with Ewing's sarcoma in remission. Autologos transplantation in children with metastatic Ewing's sarcoma seems to improve their outcome. Patients with Ewing's sarcoma, resistant to conventional therapy and with recurrent disease did not benefit from megachemotherapy. New approaches such as anti-tumor vaccination or using of imatinib are reasonable to introduce in patients with relapsed or resistant to therapy Ewing's tumor.     

Bone tumours in childhood and adolescence

Primary bone tumours are rare diseases in children and young people. Osteosarcoma and Ewing sarcoma are the commonest types of bone tumour in this age group. Whilst other rarer tumours occur their presentation and investigation are similar and their treatment will be individualised by specialist teams. We will therefore focus in on the challenges faced in the presentation, timely diagnosis, treatment and rehabilitation in young growing individuals with osteosarcoma and Ewing Sarcoma.     

VM-26 and dimethyl triazeno imidazole carboxamide in Ewing's sarcoma

Twenty-seven patients with biopsy proven Ewing's sarcoma were randomised to receive one of two adjuvant chemotherapy regimens for two years. Group A were given monthly courses of vincristine, adriamycin and dimethyl triazeno imidazole carboxamide (DIC); Group B received monthly courses of VM-26, adriamycin and DIC. Chemotherapy was commenced after biopsy confirmation of disease. The primary tumour was treated with irradiation to 3,000-5,000 rad and by resection in three patients. With a median follow-up of 26 months, 52% of patients are disease free. Ten patients died, seven from recurrent disease and three from complications of treatment. There was a significant difference in disease free survival rate of patients with long bone lesions (11/12) compared with patients with flat bone lesions (6/15). The disease free survival rate of Groups A and B was similar. Our results suggest that in combination chemotherapy for Ewing's sarcoma, vincristine and VM-26 have similar anti-tumour activity.     

Unusual presentation of osteoid osteoma mimicking osteomyelitis in a 27-month-old infant

Osteoid osteoma is an uncommon, benign disorder of bone that is most often encountered in school-age children and in adolescents. It is quite rare in children under the age of 3 years. We report a case of osteoid osteoma with unusual clinical and imaging features in a 27-month-old toddler who presented with a limp and swelling of the right knee. Initial clinical evaluation, plain films, and computed tomography (CT) were strongly suggestive of chronic osteomyelitis involving the distal right femoral metadiaphysis. The appearance on nuclear medicine bone scan, however, was typical of osteoid osteoma. A biopsy of the lesion was taken and histological examination confirmed the diagnosis of osteoid osteoma. The misleading clinical and imaging features in this case are discussed.     

Imaging of pediatric bone tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

Malignant primary bone tumors are uncommon in the pediatric population, accounting for 3%–5% of all pediatric malignancies. Osteosarcoma and Ewing sarcoma comprise 90% of malignant primary bone tumors in children and adolescents. This paper provides consensus-based recommendations for imaging in children with osteosarcoma and Ewing sarcoma at diagnosis, during therapy, and after therapy.     

Primary spinal epidural extraosseous Ewing's sarcoma mimicking a spinal abscess

Ewing's sarcoma is one of the most common malignant tumors of the skeletal system in children and young adults. It most frequently stems from the long bones of the extremities. However, though uncommon, extraosseous localization can be seen. Epidural extraosseous presentations are extremely rare. In this case, the authors report on the long-term follow-up of a patient with this well-demonstrated, but uncommon localization, and the initial unique presentation of the patient, which clinically and radiologically mimicked a cervical abscess.     

Bone mineral density and bone metabolism in children treated for bone sarcomas

In adolescent bone sarcoma patients, bone mass acquisition is potentially compromised at a time in which it should be at a maximum. To evaluate the problem we measured bone mineral density (BMD) and serum markers of bone formation and resorption in a series of pediatric patients with bone tumors. BMD was measured by dual-energy x-ray absorptiometry, at clinical remission, for lumbar spine and the neck of the femur in 38 osteosarcoma and 25 Ewing's sarcoma patients. Mean age was 20.65 and 19.13 y respectively. Serum markers of bone metabolism were: OC, PICP, ICTP, 25-OH vit D and 1,25-(OH)(2) vit D, IGF-I, IGFBP-3 and intact PTH. Serum was sampled throughout anti-tumoral treatments and follow-up. We analyzed 85 samples from 59 osteosarcoma patients and 54 samples from 36 Ewing's sarcoma patients. Patients had decreased lumbar and femoral BMD. The decrease was more pronounced in pubertal patients compared with those who had completed pubertal development at the time of disease diagnosis. Multivariate analysis indicated that sex, age, weight and BMI were significant in lumbar BMD depletion. Weight and BMI were significant in femoral BMD depletion. Serum markers of bone formation (PICP and OC) and resorption (ICTP) were, throughout, lower than reference values. Significant alterations in other markers were also observed. Up to a third of osteosarcoma and Ewing's sarcoma patients in clinical remission had some degree of BMD deficit. The corresponding increased risk of pathologic bone fractures constitutes a reduction in future quality of life.     

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.