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Daniel Azorín
Public Documents
2
Retroperitoneal malignant triton tumor in an adolescent with Neurofibromatosis type 1
Maitane Andion
and 5 more
August 25, 2020
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.
Primary osseous composite sarcoma with focal rhabdomyosarcoma and lymph node metastas...
Maitane Andion
and 5 more
May 28, 2020
Primary bone composite sarcoma is a very rare entity that primarily affects adolescent and young adult patients. It usually combines areas of liposarcoma and osteosarcoma and up to 60% of cases have metastatic disease at diagnosis. It is a highly aggressive pathology with intrinsic resistance to conventional treatment schemes for bone sarcomas. The prognosis is poor, with long term survival rates that do not exceed 30%. We present the case of an adolescent female diagnosed with a primary agressive bone composite sarcoma with rhabdomyosarcoma foci and loco-regional lymph node involvement.