24 research outputs found

    Orbital rhabdomyosarcoma: Clinicopathologic correlation, management and follow-up in two newborns. A preliminary report.

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    Rhabdomyosarcoma (RMS) is the most common primary malignant tumor of the orbit in childhood. The average age of onset is around seven years. In the present paper, the authors present the clinical and radiological findings in two newborns with histopathologically confirmed orbital rhabdomyosarcoma. A review of the literature was also carried out. In both children, the first sign leading to a visit was a unilateral proptosis, without symptoms or any other sign. Both underwent a CT-scan and an MRI. Confirmation of the histology was made by an open sky biopsy. The children were first treated by chemotherapy but had incomplete resolution of the tumor after more than 11 months for the first case and 9 months for the second. Since the parents of both children refused exenteration, radiotherapy had to be resorted to because of the incomplete response to chemotherapy. Our therapeutic approach and the outcome of these two cases are discussed

    Prolonged remission induced by cyclosporine in a patient with familial thrombocytopenia and enteropathy

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    A 21-year-old patient, since the age of 16, presented a familial type of chronic autoimmune thrombocytopenia that responded only partially to various types of immunosuppressive treatment. His prolonged survival, compared to his decreased siblings, was complicated by the appearance of a severe enteropathy. High doses of corticosteroids induced a type 1 diabetes as a major side effect. The introduction of cyclosporine resulted in both a continuous complete clinical remission and a partial hematological remission and allowed the discontinuation of all other medication for 18 months. In uncommon complex autoimmune disease, cyclosporine may represent a safe and effective alternate therapy when other immunosuppressive agents have failed

    La thérapie actuelle du rhabdomyosarcome orbitaire de l'enfant [Update of orbital rhabdomyosarcoma therapy in children]

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    INTRODUCTION: Rhabdomyosarcoma is the most frequent primitive orbital malignant tumor in children. If the treatment is started as soon as possible after discovery of the disease, the vital prognosis is considerably better than otherwise. The goal of this paper is to present the new therapeutic protocol and to report our experience in this field. MATERIAL AND METHOD: During the past 35 years, 102 cases of orbital tumors were collected in children under 15 years of age: 5 cases of rhabdomyosarcoma were cared for in our department. At the time of tumor diagnosis, the age of our patients ranged from 3 weeks to 13 years. After a biopsy or excision biopsy, all our cases were treated by chemotherapy with or without radiotherapy. Medication was mostly vincristine, ifosfamide and actinomycine D. When the result of the treatment was not satisfactory, carboplatine and epirubicine, vincristine as well as ifosfamide were given. Radiotherapy was performed only in particular cases or in recurrences. CONCLUSION: Rhabdomyosarcoma is a highly malignant tumor. Although rare, it is the most frequent of malignant tumors in children. It is important to keep it in mind in order to perform a biopsy enabling quick diagnosis and treatment following the modern protocol giving the highest chances of survival to these patients: about 98% in 3 years

    Mon enfant saigne facilement: approche clinique et paraclinique pour le praticien [My child bleeds easily: clinical and paraclinical approach]

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    The aim of this review article is to provide a clinical guideline for the child presenting with easy bruising, distinguishing among the different etiologic groups associated with this symptom what is normal and what is not, allowing then to establish an algorithm for work-up and follow-up. We also precise in which concrete situation it would be necessary to refer the child to a pediatric hematologist

    Evaluating bone marrow metastasis of neuroblastoma with iodine-123-MIBG scintigraphy and MRI.

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    Of 10 patients with neuroblastoma who had both 123I-MIBG scintigraphy and MRI at diagnosis, four presented with bone marrow metastasis that was diagnosed by both imaging modalities and confirmed by bone marrow biopsy and smears. This report focuses on the follow up of the four patients with bone marrow metastasis. MIBG scintigraphy and MRI were concordant in two patients, a case of normalization and a case of relapse in the seventh dorsal vertebra confirmed by surgical biopsy. The last two patients presented a normalized MIBG scan for marrow infiltration after chemotherapy but persistent abnormal MRI signal of several vertebrae, suggesting marrow infiltration, up to 27 mo after the end of chemotherapy in one case. In the second patient, MRI bone marrow aspect returned to normal 4 mo after the end of chemotherapy. Bone marrow biopsy remained negative in these two MIBG-negative patients. These cases suggest that in presence of complete normalization of the MIBG scan after chemotherapy, the persistence of a hypointense signal on bone marrow on T1WI does not necessarily indicate persistence of disease but may be due to delayed normalization. Therefore, attention must be paid to the delay of signal normalization on MRI (which can be as long as more than 2 yr after the end of chemotherapy) in order to avoid false-positive interpretation
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