Abstract
Infantile myofibromatosis is the most common fibrous tumor of infancy. The generalized form of the disease is associated with a high rate of early mortality, especially if visceral structures are involved. Various therapeutic strategies have been used in these patients, including high-dose chemotherapy, with the risk of therapy-related toxicity. The authors present two cases of generalized infantile myofibromatosis, with life-threatening visceral and nonvisceral involvement, in which the patients were cured with a combination of low-dose chemotherapy and intensive care. The authors propose a prospective international trial using a safe low-dose chemotherapy protocol to test the efficacy of this treatment strategy.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
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Combined Modality Therapy
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Critical Care
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Dactinomycin / administration & dosage
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Female
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Humans
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Infant, Newborn
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Male
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Methotrexate / administration & dosage
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Muscle Neoplasms / congenital
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Muscle Neoplasms / drug therapy
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Myofibromatosis / congenital
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Myofibromatosis / drug therapy*
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Osteolysis
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Prednisolone / administration & dosage
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Remission Induction
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Respiration, Artificial
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Skin Neoplasms / congenital
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Skin Neoplasms / drug therapy
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Soft Tissue Neoplasms / congenital
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Soft Tissue Neoplasms / drug therapy*
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Vinblastine / administration & dosage
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Vincristine / administration & dosage
Substances
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Dactinomycin
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Vincristine
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Vinblastine
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Prednisolone
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Methotrexate