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CC BY 4.0 · Brazilian Journal of Oncology 2018; 14(49): 1-5
DOI: 10.5935/1806-6054.20180001
CASE REPORTS

A young man with constitutional symptoms and a small bowel vegetating lesion

Um jovem com sintomas constitucionais e uma lesão vegetante em intestino delgado
Pedro Grachinski Buiar
1   Hospital de Clínicas de Porto Alegre, Department of Medical Oncology, Porto Alegre, Rio Grande do Sul, Brazil
,
Gustavo Cartaxo de Lima Gossling
1   Hospital de Clínicas de Porto Alegre, Department of Medical Oncology, Porto Alegre, Rio Grande do Sul, Brazil
,
Gilberto Schwartsmann
1   Hospital de Clínicas de Porto Alegre, Department of Medical Oncology, Porto Alegre, Rio Grande do Sul, Brazil
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ABSTRACT

Gastrointestinal neuroectodermal tumor is a rare neoplasm that affects young population and is associated with poor prognosis. Previously named Clear cell sarcoma-like tumor of the gastrointestinal tract, due to the similar phenotypic pattern, advances in the molecular characterization of this tumor helped to differentiate it as a separate disease. Despite this, the small number of cases reported only suggests a reserved prognosis and isolated treatment recommendations based on local experiences. This case report illustrates a 37-year-old patient with constitutional symptoms diagnosed with metastatic disease treated with an aggressive approach involving preoperative chemotherapy followed by metastasis resection, with no evidence of disease recurrence after two years of follow-up.

RESUMO

O Tumor neuroectodérmico gastrintestinal é uma neoplasia rara, caracterizada por acometer pacientes jovens e possuir comportamento agressivo. Antigamente classificado como tumor gastrintestinal similar ao sarcoma de células claras, devido ao padrão fenotípico, os avanços na caraterização molecular deste tumor ajudaram a diferenciá-lo como uma entidade própria. Apesar disso, a pequena quantidade de casos relatados até hoje apenas sugerem uma prognostico reservado e trazem esquemas de tratamento baseados em poucos casos/experiências locais. Este relato de caso ilustra um paciente de 37 anos com sintomas sistêmicos, diagnosticado com doença metastática tratado com abordagem agressiva envolvendo ressecção das metástases e quimioterapia citotóxica pré-operatória, sem evidência de progressão de doença após dois anos de follow-up.

Keywords:

Neuroectodermal - Gastrointestinal - Rare - Young - Clear cell sarcoma

Descritores:

Neuroectodérmico - Gastrointestinal - Raro - Jovem - Sarcoma de células claras


Publication History

Received: 24 August 2018

Accepted: 19 December 2018

Article published online:
07 March 2025

© 2022. This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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