Therapeutic approach of a pancreatic Ewing Sarcoma/PNET: case report and literature review

ABSTRACT

Peripheral primitive neuroectodermal tumors (pPNETs) are part of the Ewing sarcoma family. A few more than 30 cases of pPNETs originating on the pancreas have been reported in the consulted literature. We report a locally advanced pancreatic pPNET, with the longest follow-up and survival in the consulted literature, considering quality of life outcomes. 22-year-old male patient, with a pancreatic pPNET comprising pancreatic body and tail with gastric invasion. He underwent multi organic resection and adjuvant chemotherapy and radiotherapy. After 8 years of follow-up, he was free of disease and with a good quality of life self-evaluation. The aggressive surgical approach associated with multimodal regimens provided improvement in disease-free survival and a positive impact on quality of life.

RESUMO

Os tumores neuroectodérmicos primitivos periféricos (pPNETs) fazem parte da família dos sarcomas de Ewing. Pouco mais de 30 casos de pPNETs originários do pâncreas foram relatados na literatura consultada. Relatamos um pPNET pancreático localmente avançado, com o acompanhamento mais longo e sobrevida na literatura consultada, considerando os resultados de qualidade de vida. Paciente do sexo masculino, 22 anos, com pPNET pancreático que compreende corpo e cauda pancreáticos com invasão gástrica. Foi submetido à ressecção multiorgânica e quimioterapia adjuvante e radioterapia. Após 8 anos de acompanhamento, ele estava livre de doenças e com uma autoavaliação de boa qualidade de vida. A abordagem cirúrgica agressiva associada aos esquemas multimodais proporcionou melhora na sobrevida livre de doença e impacto positivo na qualidade de vida.

Keywords:

Neuroectodermal tumors - Primitive - Peripheral - Sarcoma - Ewing - Pancreatic neoplasms - Combined modality therapy - Quality of life

Descritores:

Tumores neuroectodérmicos primitivos periféricos - Sarcoma - Ewing - Neoplasias pancreáticas - Terapia de modalidade combinada - Qualidade de vida

AUTHOR'S CONTRIBUTION

Rodrigo Nascimento Pinheiro: Collection and assembly of data, Data analysis and interpretation, Final approval of manuscript, Manuscript writing, Provision of study materials or patient.

Fabiane Kellem Oliveira dos Santos Cesário: Collection and assembly of data, Final approval of manuscript, Provision of study materials or patient.

José Pablo Mata Mondragon: Collection and assembly of data, Provision of study materials or patient.

Fábio Daniel Barbosa da-Silva: Collection and assembly of data, Data analysis and interpretation, Manuscript writing.

Renata Pereira Fontoura: Conception and design, Data analysis and interpretation, Manuscript writing.

José Donato de Sousa Netto: Data analysis and interpretation, Manuscript writing.

Carlos Henrique de Aguiar Botelho: Collection and assembly of data, Provision of study materials or patient.

Publication History

Received: 01 June 2019

Accepted: 21 July 2019

Article published online:
02 July 2020

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