Abstract
Malignant triton tumor (MTT) is a rare, highly malignant nerve sheath tumor with rhabdomyoblastic differentiation. Initial debulking surgery followed by adjuvant therapy is the current treatment of choice, but has very limited efficacy when optimal cytoreduction is not achieved by surgical procedure. Neoadjuvant therapy for MTT, to potentially facilitate subsequent surgery, eradicate micrometastatic lesions and, therefore, improve the therapeutical outcome, has never before been presented in literature. Here, we report on the multimodal management of two cases of advanced and metastatic MTT. Treatment modalities involved neoadjuvant and adjuvant chemotherapy, surgical resection, and radiation. In both cases, integrated Positron Emission Tomography/Computed Tomography (PET/CT) emerged as an important diagnostic tool for the reliable assessment of MTT response and metabolic remission.
MeSH terms
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Adult
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Antineoplastic Agents / therapeutic use
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Cecal Neoplasms / drug therapy
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Cecal Neoplasms / radiotherapy
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Cecal Neoplasms / surgery
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Cecal Neoplasms / therapy*
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Chemotherapy, Adjuvant
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Combined Modality Therapy
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Female
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Fluorodeoxyglucose F18
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Humans
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Ileal Neoplasms / drug therapy
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Ileal Neoplasms / radiotherapy
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Ileal Neoplasms / surgery
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Ileal Neoplasms / therapy*
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Liver Neoplasms / drug therapy
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Liver Neoplasms / radiotherapy
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Liver Neoplasms / surgery
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Liver Neoplasms / therapy*
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Male
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Neoadjuvant Therapy
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Neurilemmoma / drug therapy
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Neurilemmoma / radiotherapy
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Neurilemmoma / surgery
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Neurilemmoma / therapy*
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Neurofibromatosis 1 / pathology
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Ovarian Neoplasms / drug therapy*
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Ovarian Neoplasms / pathology
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Positron-Emission Tomography
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Radiopharmaceuticals
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Tomography, X-Ray Computed
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Uterine Neoplasms / drug therapy
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Uterine Neoplasms / radiotherapy
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Uterine Neoplasms / surgery
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Uterine Neoplasms / therapy*
Substances
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Antineoplastic Agents
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Radiopharmaceuticals
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Fluorodeoxyglucose F18