Malignant bone tumors in children: Ewing's sarcoma

Ewing's sarcoma is a rare, malignant, small, round-cell tumor of bone. Most tumors arise in the diaphyses of long bones, but any bone can be involved. Chemotherapy is essential for long-term survival. Multiagent protocols are superior to single-agent therapies, but the ideal regimen has not been established. Both radiation therapy and surgery have been employed for local control of primary tumors. Control with either modality is significantly better when used in conjunction with effective chemotherapy. Patients who present with metastatic disease or pelvic primaries have a worse prognosis. Autologous bone marrow transplantation may improve disease-free survival for this group.     

Malignant humeral bone tumors in children: Excision and reconstruction with the use of rotated clavicle

The application of intensive multimodality therapy has made possible salvage surgery in bone tumors. Reconstruction of the removed part of bone is the great problem, especially in fast-growing children. In three patients (two osteosarcomas and one Ewing's sarcoma), the tumor was confined to the proximal half part of humerus, without invasion of shoulder joint. After induction chemotherapy, reduction of tumor size was observed both clinically and radiologically. During the operation, wide resection of the tumor together with a 12- to 14-cm-long fragment of humerus, was performed. Afterward, the clavicle was rotated in the place of the removed bone, with preservation of the coracoacromial ligament. The humeral stump and clavicle were fixed with the use of metal plate. Adjuvant chemotherapy was used a few days following surgery. After 3 months, the osteosynthesis had healed. The movements in shoulder joint are limited, but functions of elbow joint remained normal. All children are alive and disease free. Reconstruction of humerus with clavicle rotation is possible when the proximal bone loss is not longer than 10–14 cm. This method seems to be an alternative to allogeneic grafts and endoprostheses. © 1996 Wiley-Liss, Inc.     

Malignant lymphoma of bone in children

From 1960 to 1985 at the Royal Children's Hospital, Melbourne, seven cases of malignant lymphoma of bone (MLB) were identified in children younger than 16 years of age. Over the same period there were 88 cases of Ewing's sarcoma (ES), which illustrates the rarity of MLB. All cases were staged and treated according to protocols current at the time, namely, systemic chemotherapy with radiotherapy to the primary lesions. One child received radiotherapy alone as chemotherapy was not then available (1963). The disease-free five-year survival rate in our small series is 57%, which compares favorably with the 62% that we have calculated from survival data available from 26 previously reported cases of MLB in children and better than the 35% to 50% quoted in adult series of MLB. The main problem in differential diagnosis is distinguishing MLB from ES. A positive diagnosis of MLB rather than ES can be made, however, by observation of the characteristic reticulin staining pattern, the absence of cytoplasmic glycogen, and the ultrastructure.     

Malignant ovarian tumors in children and adolescents

Eighteen patients aged 4 to 18 years (median, 10 years) were treated at the Children's Cancer Research Center of The Children's Hospital of Philadelphia (CHP) from September 1973 to September 1983 for malignant ovarian tumors. The pathologic categories were endodermal sinus tumor (eight patients), embryonal carcinoma (three patients), pure dysgerminoma (three patients), adenocarcinoma (three patients), and malignant granulosa-cell tumor (one patient). In 1973 and 1975, respectively, two patients with endodermal sinus tumors were treated with surgical removal with or without radiation therapy (RT) and chemotherapy with vincristine (Vc) and actinomycin D+ cyclophosphamide + Adriamycin (Adria Laboratories, Columbus, OH) (ACAdr); both developed local recurrence within 12 months and died of tumor. Since 1977, four of six patients with endodermal sinus tumors have been managed successfully with surgery followed by chemotherapy with cisplatin (P), bleomycin (B), vinblastine (Vb), and ACAdr; none received RT. One died of recurrent tumor and the other died of congestive heart failure attributed to Adriamycin (Adr). All three patients with embryonal carcinoma were well after surgical excision alone (one patient) or surgery and chemotherapy with cisplatin + bleomycin + vinblastine (PBVb) + ACAdr (two patients). Treatment of the remaining seven patients included excision alone (two patients) or excision and chemotherapy (five patients) with various combinations of Vc, ACAdr, and P. One patient with granulosa-theca cell tumor also received whole abdominal RT (3000 rad by external beam), and died of congestive heart failure attributed to Adr. The other six children were free of detectable tumor 16 months to 8 years from diagnosis. Chemotherapy with PBVb and actinomycin D + cyclophosphamide (AC) should be used in the postoperative management of young persons with malignant ovarian tumors. Adr is of doubtful value and may be considerably toxic. RT is of limited use and usually is not indicated.     

Malignant tumors in American black and Nigerian children: a comparative study.

Results of a study on the relative frequencies of tumors in American black and Nigerian children were compared with data from the Childhood Cancer Registries in Manchester, United Kingdom, and Kampala, Uganda. The American black child living in Washington, D.C. and the Caucasian child living in Manchester had similar high frequencies for leukemia and glioma, whereas the incidence of lymphoma and retinoblastoma was low. African children living in Nigeria or Uganda had the opposite frequency patterns. These differences in frequencies of tumors between two ethnologically related population groups, American black and Nigerian, suggested the influence of environmental factors in the etiology of these tumors, even though exposure to environmental carcinogens was short. The rarity of Ewing's sarcoma and testicular tumors in American black and Nigerian children suggested a genetic influence.     

Imaging of bone tumors using a monoclonal antibody raised against human osteosarcoma

The radiolabeled monoclonal antibody 791T/36 raised against a human osteosarcoma was injected into 20 patients with known or suspected bone tumors. Gamma camera images were acquired at 48 or 72 hours after injection, and assessed for antibody localization. Positive images were obtained in all five osteosarcomas and four other primary malignant sarcomas. Two of the four other primary bone tumors gave positive images. Three patients with trauma had negative images as did one patient with Paget's disease. Two patients with suppurative disease gave positive images. The antibody localized in the majority of malignant sarcomas tested. In one tumor where tissue was available, a tumor:non-tumor ratio of 2.8:1 was measured. Repeat imaging was performed in five patients. Immunoscintigraphy using the monoclonal antibody 791T/36 has shown tumor localization in patients with bone and soft tissue sarcomas.     

Autologous bone marrow transplantation in solid tumors in children

Seven children with advanced solid tumors (2 rhabdomyosarcomas, 2 Ewing's sarcomas, 1 astrocytoma, 1 T cell lymphoma and 1 neuroblastoma) received high dose chemotherapy and/or radiotherapy followed by autologous bone marrow transplantation. Four patients achieved complete remissions, two had partial remissions, and one was no response. Side effect of autologous bone marrow transplantation was few compared with that of allogeneic bone marrow transplantation in which graft versus host reaction, profound posttransplantation immunodeficiency and interstitial pneumonitis were unavoidable. In this report methods of bone marrow cryopreservation and elimination of tumor cells from harvested bone marrow were also discussed.     

Blood serum somatomedin activity in children with bone tumors

Blood somatomedin levels were assayed biologically in 10 children with Ewing's sarcoma and osteosarcoma and 11 children with other benign tumors. The patients were of the same age. The basal level of somatomedin in malignant bone sarcoma was similar to that in controls. However, after glucose loading, the patterns of somatomedin level curve in children and adults with normal lipo-carbohydrate metabolism were different. The difference may be due either to the specific features of child's organism or a rise in the level of blood somatomedin inhibitors.     

Malignant cardiac tumors

Cardiac neoplasms are a rare occurrence in clinical practice. The various frequencies of primary and secondary malignant tumors vary from report to report, approximately 1% in most autopsy series and 4% in cancer patient's autopsies. Cardiac malignancies account for less 1% of cardiac surgery and about for 0.1% of cardiac echographic studies. The presence of metastatic tumor to the heart usually indicates widespread metastases. Lung carcinomas are the most commonly encountered tumor followed by breast and pancreas cancer and melanoma. Apart from primary pericardial mesothelioma, primary cardiac tumors are high-grade sarcomas with a high metastatic potency that often becomes evident early after surgery. Symptoms are non specific, occur late in the disease and affect few patients; especially secondary neoplasms of the heart take their course so fast that they cannot become symptomatic. The signs of cardiac neoplasms are divided into systemic symptoms (fever, arthralgias and myalgias), cardiac symptoms (congestive heart failure, arrhythmia, chest pain) and uncommon embolisms. Diagnosis is actually made easier with cardiac echography. Cardiac RMI is helpful to estimate vessels and pericardium involvement. Due to its poor prognosis, treatment of cardiac metastases is restricted to best supportive care. For primary cardiac neoplasms, surgery must be carefully discussed because operative intervention is often followed by rapid widespread metastases that adjuvant chemotherapy cannot avoid in most cases.     

Malignant ovarian tumors in childhood

Malignant ovarian tumors only account for 3 per cent of all malignancies under 15 years of age, and more than two third of them are of germ cell origin. Abdominal mass and/or pains are the usual revealing symptoms and abnormal sexual prematurity is infrequently encountered. Diagnostic ultrasonography is currently the most useful imaging investigation. Alpha-foeto-protein (alpha-FP) serum determination is mandatory before surgery to indicate the presence of endodermal sinus tumor cells. Surgical procedure is in childhood usually restricted to unilateral salpingo-oophorectomy, if allowed by tumoral spreading which is carefully searched in the abdominal cavity. Thus will be defined, along with the post-operative lymphangiography, the extension of the tumor, according to the staging system of Wollner (Memorial Hospital NY) more than the FIGO. Malignant teratomas are the most frequent malignant germ cell ovarian tumors in young females. They may realize pure or mixed pathological types of immature teratoma, embryonal carcinoma, endodermal sinus tumor and very seldom choriocarcinoma. Serial assays of serum alpha-FP are of utmost value to follow therapeutic progress or detect recurrences. Since new sequential multidrug protocol including, vincristine, actinomycin D, cyclophosphamide, adriamycin, méthotrexate and cis-platinum, protracted survivals are to be expected in a higher number of patients with localized stages and even in some with advanced disease. Value of extended pelvi-abdominal and/or lymph node radiotherapy is still under evaluation. Ovarian dysgerminomas are radiosensitive tumors in which chemotherapy is as a rule not indicated. Relapses may be most often prevented by a prophylactic irradiation of iliac ipsilateral, lumboaortic, mediastinal and supraclavicular nodes. Among functional ovarian tumors, mainly granulosa cell types with isosexual precocity, malignant tumors are so rarely encountered that no complementary treatment has to be performed after a complete resection. Cystadenocarcinoma or borderline ovarian epithelial tumors occur after puberty and require the same treatment as in adult though keeping a conservative view. Ovarian deposits of Burkitt's tumor may account in some exposed african areas for most ovarians malignancies in childhood. Gonadoblastomas with both stromal and germ cell elements are exclusively to be found in dysgenetic gonads.     

Malignant neuroblastic tumors in adolescents

Malignant neuroblastic tumors (MNT) commonly affect children in the early years of life and become rare afterwards. The clinical characteristics and outcome of these tumors occurring in adolescents are still unclear. In this review, the main clinical reports available in the literature are summarized together with some original data. In general, MNT occurring in adolescents, if compared to those occurring in children, are more frequently diagnosed in advanced stages, have uncommon sites of metastases, but have less frequently unfavorable biological markers. If treated with standard protocols as those used for children, their survival is poor; in particular, patients with localized disease seem to have a more aggressive course than children.