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Retroperitoneal malignant triton tumor in an adolescent with Neurofibromatosis type 1
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  • Maitane Andion,
  • Susana Buendía,
  • Natalia Camarena,
  • Daniel Azorín,
  • Sara Sirvent Cerdá,
  • Pablo Morató
Maitane Andion
Hospital Infantil Universitario Nino Jesus
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Susana Buendía
Hospital Infantil Universitario Nino Jesus
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Natalia Camarena
Hospital Infantil Universitario Nino Jesus
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Daniel Azorín
Hospital Infantil Universitario Nino Jesus
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Sara Sirvent Cerdá
Hospital Infantil Universitario Nino Jesus
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Pablo Morató
Hospital Infantil Universitario Nino Jesus
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Abstract

Malignant triton tumor (MTT) is a very infrequent variant of the malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. Up to 70% of cases are diagnosed in patients with neurofibromatosis type 1 (NF1). It is a highly aggressive pathology with early relapses occurring in up to 50% of patients. Despite multimodal treatment the prognosis is poor, with long term survival rates not exceeding 15%. We present the case of an adolescent male with known NF1 diagnosed with an aggressive retroperitoneal MTT and disseminated pulmonary disease.