Late effects of therapy in orbital rhabdomyosarcoma in children. A report from the intergroup rhabdomyosarcoma study
Ruth HeynAbdel RagabR. Beverly RaneyFred RuymannMelvin TefftWalter LawrenceEdward H. SouleHarold M. Maurer
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Abstract:
Late effects of therapy were evaluated in 50 children surviving orbital rhabdomyosarcoma following treatment on Intergroup Rhabdomyosarcoma Study I. All patients had microscopic or gross tumor present following surgery and subsequently received radiotherapy and various combinations of chemotherapeutic agents. Problems in the eye included infections and functional and structural changes. Decreased vision in the treated eye was the most common functional problem and in most patients related to cataract formation, which occurred in 90% of eyes. Changes in the cornea and retina were also seen. Bony hypoplasia of the orbit and facial asymmetry were present in one half of the children. Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. The excellent survival in patients with orbital rhabdomyosarcoma provides support for treatment programs that use multiple-agent chemotherapy and radiotherapy and do not include orbital exenteration initially.Keywords:
Retinoblastoma
Acute myeloblastic leukemia
Orbit (dynamics)
Retinoblastoma
Capillary hemangioma
Orbit (dynamics)
Choroid
Exophthalmos
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Late effects of therapy were evaluated in 50 children surviving orbital rhabdomyosarcoma following treatment on Intergroup Rhabdomyosarcoma Study I. All patients had microscopic or gross tumor present following surgery and subsequently received radiotherapy and various combinations of chemotherapeutic agents. Problems in the eye included infections and functional and structural changes. Decreased vision in the treated eye was the most common functional problem and in most patients related to cataract formation, which occurred in 90% of eyes. Changes in the cornea and retina were also seen. Bony hypoplasia of the orbit and facial asymmetry were present in one half of the children. Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. The excellent survival in patients with orbital rhabdomyosarcoma provides support for treatment programs that use multiple-agent chemotherapy and radiotherapy and do not include orbital exenteration initially.
Retinoblastoma
Acute myeloblastic leukemia
Orbit (dynamics)
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Retinoblastoma is the most common intraocular tumor in childhood. There is an increased risk of secondary malignancy in these cases, especially in hereditary retinoblastoma. Here, we report a case with bilateral retinoblastoma who was treated with chemotherapy and radiotherapy, and developed soft tissue sarcoma 14 years later within the radiation field. A 16-year-old girl who had enucleation of the right eye and radiotherapy of the left eye when she was 1 year old, presented with a mass in the maxillary sinus. Biopsy was consistent with low-grade non-rhabdomyosarcoma soft tissue sarcoma. The patient was treated with surgery and chemotherapy, but had multiple recurrences. She received various salvage chemotherapy regimens and multiple surgical procedures, and is alive with disease. There is an increased risk of secondary malignancy in retinoblastoma, especially in the hereditary form. This risk is increased in patients receiving radiotherapy. Tumors may appear within the radiation field even years later.
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The paper is a retrospective study about complex treatment (surgery, chemotherapy and radiotherapy) in a group of 25 patients with retinoblastoma. The best results were in patients with early diagnosis, who came soon after surgery for chemotherapy and radiotherapy. Non presentation for chemotherapy and radiotherapy was the main cause of therapeutical fail.
Retinoblastoma
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Cosmesis
Eye Enucleation
Orbit (dynamics)
Retinoblastoma
Enophthalmos
Ocular prosthesis
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Abstract The case of a 6‐year‐old boy with rhabdo‐myosarcoma of the larynx, treated by laser surgery combined with external radiotherapy and chemotherapy, is presented. Using this treatment modality, cure was affected without comprising laryngeal function.
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Rhabdomyosarcoma (RMS) is a relatively frequent tumor in children. Judicious combinations have markedly improved treatment results in recent years. Orbital localizations are treated with chemotherapy and radiotherapy. It has been advocated that radiotherapy be limited to the original tumor bed. This case report illustrates the danger of shielding part of the orbit: in a clinically tumor-free region before chemotherapy, which was shielded during radiotherapy, a local recurrence was seen while the original tumor bed remained controlled. Therefore, the whole content of the orbit should be irradiated in orbital RMS.
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Prior to the use of chemotherapy, survival for rhabdomyosarcoma which had been completely resected was 50-60%. A controled study done by Children's Cancer Study Group showed the effectiveness of combined chemotherapy used as part of primary therapy in similar patients. Children who received actinomycin D (Act D) and vincristine (Vcr) for 1 year after surgery and radiotherapy had a lower metastatic rate than those who received none. This combination resulted in an 89% survival in patients with localized disease which was surgically resectable. Patients with microscopic residual disease had a 91% survival. With more aggressive use of combined chemotherapy, experience has accumulated demonstrating improved response and survival in both localized and gross residual disease. Combinations of Act D, Vcr, and cyclophosphamide can reduce initial tumor size, making large tumor masses more amenable to surgery and radiotherapy. Currently, an intergroup study is testing the response to four different drug combinations and the duration of therapy needed for various stages of the disease.
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Objective To investigate the classification and incidence,the clinical features of orbital space-occupying lesions in children.Methods Retrospective survey of 214 padiatric patients(from January 1997 to December 2003)with orbital space-occupying lesions diagnosed by clinical and pathological examinations.Results Thirteen different kinds of pediatric orbital space-occupying lesions were categorized.The incidence of benign diseases was 80.8%,and of malignant tumors was 19.2%.Vasculogenic,cystic and peripheral nerve diseases were the most common pediatric orbital benign diseases.Orbital extension of retinoblastoma,rhabdomyosarcoma,lymphoma were the most common malignant tumor in children.It was characterstic of the clinical and imaging features of pediatric orbital space-occupying lesions.Conclusions Vasculogenic,cystic and peripheral nerve diseases were the most common pediatric orbital diseases.Characteristic clinical and imaging features are helpful for the diagnosis of pediatric orbital space-occupying lesions.The treatment may be considered according to the clinical features of pediatric orbital space-occupying lesions.
Key words:
Cild; Space-occuping orbital diseases; Diagnosis; Treatment.
Orbit (dynamics)
Retinoblastoma
Orbital Diseases
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Objective To analyze and discuss the clinical features, pathology, diagnosis, treatment and prognosis of head and neck rhabdomyosarcoma. Methods Eight cases of head and neck rhabdomyosarcoma were studied retrospectively and the related literature was reviewed. Results Of the 8 cases, 5 were operated in addition to radio-therapy and chemotherapy, 1 was operated together with chemotherapy, 1 was operated by exploration of mastoid; and the other one was treated by radiotherapy and chemotherapy. The 5-year survival rate was 62.5%(5/8). Conclucion It can apparently increase the survival rate through the combined therapy which involves operation, radiotherapy and chemotherapy.
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