1. Systemic treatment of malignant gastrointestinal neuroectodermal tumour after childhood neuroblastoma
- Author
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Mario Giuliano, Chiara Maddalena, Maria D'Armiento, Brigitta Mucci, Gabriella Ferraro, Giovannella Palmieri, Vincenzo d'Alessandro, Marianna Tortora, Rossella Lauria, Elide Matano, Sabino De Placido, Margaret Ottaviano, Vincenzo Di Lauro, Ottaviano, M., Maddalena, Chiara, D'Armiento, M., Lauria, R., D'Alessandro, Vincenzo, Tortora, Martina, Matano, E., DI LAURO, Vincenzo, Mucci, B., Ferraro, G., De Placido, S., Giuliano, M., and Palmieri, G.
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Adult ,0301 basic medicine ,Cancer Research ,medicine.medical_specialty ,Vincristine ,Cyclophosphamide ,medicine.medical_treatment ,Digestive System Neoplasms ,Gastroenterology ,Metastasis ,Neuroblastoma ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Antineoplastic Combined Chemotherapy Protocols ,Humans ,Medicine ,Autologous transplantation ,Pharmacology (medical) ,anthracyclines, cisplatinum, clear cell sarcoma-like of the gastrointestinal tract, gene translocation, malignant gastrointestinal neuroectodermal tumour, neuroblastoma, radiotherapy ,Etoposide ,Pharmacology ,Chemotherapy ,business.industry ,medicine.disease ,030104 developmental biology ,Oncology ,030220 oncology & carcinogenesis ,Female ,business ,Childhood Neuroblastoma ,Progressive disease ,medicine.drug - Abstract
Malignant gastrointestinal neuroectodermal tumour is an extremely rare neoplasm that arises in the wall of the small bowel, stomach or large bowel in youngaged and middle-aged adults. Histologically, it is generally characterized by monomorphic cells with clear cytoplasma, S-100 protein expression, and EWSR1 gene translocation. To the best of our knowledge, we describe for the first time, the case of a young woman with a diagnosis of metastatic gastrointestinal neuroectodermal tumour arising from ileum, who had a childhood adrenal neuroblastoma with liver, bone and lymph nodes metastasis, treated with four cycles of chemotherapy with the schedule CADO-CVP (CADO: cyclophosphamide 300 mg/m2/day on days 1–5, vincristine 1,5 mg/m2/ day on days 1 and 5, and doxorubicin 60 mg/m2/day on day 5; CVP: cisplatin 40 mg/m2/day on days 1–5 and etoposide 100 mg/m2/day on days 1–5) followed by right adrenal, kidney, lymph nodes and liver lesion resection, conditioning chemotherapy (melphalan-carmustineteniposide), stem cells autologous transplantation and consecutively radiotherapy on the spine (T9 to L3) for a total of 30 Gy. For the second diagnosis of gastrointestinal neuroectodermal tumour with liver metastasis, she underwent ileal tumour resection and platinum-anthracycline based chemotherapy with initial shrinkage of liver metastasis. Unfortunately, despite the initial response and the following delivered therapies, she died for rapid progressive disease. Taking into account the late effects of past therapeutic modalities, a long-term surveillance of young child treated for neuroblastoma, is required to appreciate their overall risks of second malignancies.
- Published
- 2019
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