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Systemic treatment of malignant gastrointestinal neuroectodermal tumour after childhood neuroblastoma

chemotherapy in malignant gastrointestinal neuroectodermal tumour

Ottaviano, Margareta,,b; Maddalena, Chiaraa; D’Armiento, Mariac; Lauria, Rossellaa; D’Alessandro, Vincenzod; Tortora, Mariannab; Matano, Elidea; Di Lauro, Vincenzoa; Mucci, Brigittaa; Ferraro, Gabriellae; De Placido, Sabinoa,,b; Giuliano, Marioa,,b; Palmieri, Giovannellab

doi: 10.1097/CAD.0000000000000806
Case Reports

Malignant gastrointestinal neuroectodermal tumour is an extremely rare neoplasm that arises in the wall of the small bowel, stomach or large bowel in young-aged 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-carmustine-teniposide), 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.

aOncology Unit, Department of Clinical Medicine and Surgery, University Federico II of Naples

bOncology Unit, CRTR Rare Tumors Reference Center, AOU Federico II of Naples

cDepartment of Biomorphological and Functional Sciences, Section of Pathology, University of Naples ‘Federico II’

dOperative Unite of Kidney Transplantation and retroperitoneal pathologies, AOU Federico II of Naples

eUFA Operative Unit, AOU Federico II of Naples, Naples, Italy

Received 18 March 2019 Revised form accepted 25 April 2019

Correspondence to Margaret Ottaviano, Oncology Unit, Department of Clinical Medicine and Surgery, Università degli Studi di Napoli Federico II, Via Sergio Pansini 5, 80131 Naples, Italy, Tel: +39 3333759668, fax: +39 0817462114; e-mail:

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