Gastrointestinal neuroectodermal tumor (GNET) : a case.
Tumor neuroectodérmico gastrointestinal maligno (GNET) : a propósito de un caso.
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The gastrointestinal neuroectodermal tumor malignant (GNET) is an extremely rare entity recently described in the literature and is also known as tumor-like clear cell sarcoma of the gastrointestinal tract (CCSLGT) for their morphological and molecular similarity clear cell sarcoma of tendons and aponeuroses (CCS). However, given the presence of rearrangements of EWSR1 gene and neuroectodermal differentiation, some authors has been considered GNET more appropriate term. It is a disease with poor prognosis and high recurrence rate almost always metastatic from diagnosis in contrast to the relatively indolent behavior of CSS. To our knowledge there are fewer than 50 reported cases of this disease and to date this would be the first case located in primary liver and prolonged relapse-free survival after appropriate surgical management and adjuvant chemotherapy
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