Frantz's tumor, a diagnostic challenge: case report

Detalhes bibliográficos
Autor(a) principal: de Moraes Coimbra, Bianca
Data de Publicação: 2020
Outros Autores: Leite da Silva Júnior, Cilmário, Neves Marcos, Vinícius, Sabbagh de Hollanda, Erick
Tipo de documento: Artigo
Idioma: por
Título da fonte: HU Revista (Online)
Texto Completo: https://periodicos.ufjf.br/index.php/hurevista/article/view/29025
Resumo: Introduction: Pseudopapillary solid pancreatic tumor (Frantz's tumor) is a solid-cystic exocrine cell neoplasm that accounts for about 0.2-2.7% of all pancreatic tumors. It is a rare disease with low potential for malignancy, preferentially affecting young women, with an average age of 25 years. Complete surgical resection of the lesion is the treatment of choice, and the prognosis is excellent. Objective: To report a challenging case of Frantz's tumor with atypical clinical-radiological presentation, in which imaging exams were fundamental for the diagnostic elucidation and therapeutic management. Case Report: 31-year-old female patient with chronic anemia, hematemesis, melena and hematochezia. Upper gastrointestinal endoscopy showed an active ulcer in the duodenal bulb. Tomography revealed an expansive mass located between the pancreatic head and the duodenum, with signs of ulceration. At this time, the main diagnostic hypotheses were gastrointestinal stromal tumor (GIST) or Frantz tumor. Magnetic resonance imaging characterized an intimate relationship of the lesion with pancreatic tissue, making the diagnosis of Frantz tumor as the best hypothesis. Then, the patient was submitted to total duodenopancreatectomy for resection and definitive treatment of the lesion. Immunohistochemistry revealed cells with indistinct eosinophilic cytoplasm and hyperchromatic nuclei, sometimes with pseudopapillary arrays, as well as expression for beta-catenin in nuclear pattern and positivity for CD10 and CD99, confirming that it is a solid pseudopapillary tumor of the pancreas. Conclusion: The importance of imaging tests for characterization and assertive diagnosis is observed of this type of tumor, allowing the complete lesion resection and perspective of cure.
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spelling Frantz's tumor, a diagnostic challenge: case reportTumor de Frantz, um desafio diagnóstico: relato de casoPancreatic NeoplasmsDuodenal UlcerAnemiaNeoplasias PancreáticasÚlcera DuodenalAnemiaIntroduction: Pseudopapillary solid pancreatic tumor (Frantz's tumor) is a solid-cystic exocrine cell neoplasm that accounts for about 0.2-2.7% of all pancreatic tumors. It is a rare disease with low potential for malignancy, preferentially affecting young women, with an average age of 25 years. Complete surgical resection of the lesion is the treatment of choice, and the prognosis is excellent. Objective: To report a challenging case of Frantz's tumor with atypical clinical-radiological presentation, in which imaging exams were fundamental for the diagnostic elucidation and therapeutic management. Case Report: 31-year-old female patient with chronic anemia, hematemesis, melena and hematochezia. Upper gastrointestinal endoscopy showed an active ulcer in the duodenal bulb. Tomography revealed an expansive mass located between the pancreatic head and the duodenum, with signs of ulceration. At this time, the main diagnostic hypotheses were gastrointestinal stromal tumor (GIST) or Frantz tumor. Magnetic resonance imaging characterized an intimate relationship of the lesion with pancreatic tissue, making the diagnosis of Frantz tumor as the best hypothesis. Then, the patient was submitted to total duodenopancreatectomy for resection and definitive treatment of the lesion. Immunohistochemistry revealed cells with indistinct eosinophilic cytoplasm and hyperchromatic nuclei, sometimes with pseudopapillary arrays, as well as expression for beta-catenin in nuclear pattern and positivity for CD10 and CD99, confirming that it is a solid pseudopapillary tumor of the pancreas. Conclusion: The importance of imaging tests for characterization and assertive diagnosis is observed of this type of tumor, allowing the complete lesion resection and perspective of cure.Introdução: O tumor sólido pseudopapilar do pâncreas (tumor de Frantz) é uma neoplasia sólido-cística de células exócrinas que corresponde a cerca 0,2-2,7% de todos os tumores pancreáticos. Trata-se de doença rara, com baixo potencial de malignidade, que acomete preferencialmente em mulheres jovens, com média de idade de 25 anos. A ressecção cirúrgica completa da lesão é o tratamento de escolha, e o prognóstico é excelente. Objetivo: Relatar um caso desafiador de tumor de Frantz com apresentação clínico-radiológica atípica, em que os exames de imagem tiveram importância fundamental para elucidação diagnóstica e no manejo terapêutico. Relato de Caso: Paciente do gênero feminino, 31 anos, apresentando anemia crônica, hematêmese, melena e hematoquezia. A endoscopia digestiva alta evidenciou úlcera em atividade no bulbo duodenal. A tomografia revelou massa expansiva localizada entre a cabeça pancreática e o duodeno, com sinais de ulceração. As principais hipóteses diagnósticas eram tumor estromal gastrointestinal (GIST) ou tumor de Frantz. A ressonância magnética caracterizou íntima relação da lesão com o tecido pancreático, deixando o diagnóstico de tumor de Frantz como a hipótese mais provável. A paciente foi submetida a duodenopancreatectomia total para ressecção e tratamento definitivo da lesão. A imunohistoquímica revelou células com citoplasma eosinofílico indistinto e núcleos hipercromáticos, por vezes com arranjos pseudopapilíferos, bem como expressão para betacatenina em padrão nuclear e positividade para CD10 e CD99, confirmando se tratar de um tumor sólido pseudopapilar do pâncreas. Conclusão: Observa-se a importância dos exames de imagem para caracterização e diagnóstico assertivo desse tipo de tumor, possibilitando a ressecção completa da lesão e perspectiva de cura da doença.Editora UFJF2020-06-08info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelCasapplication/pdfhttps://periodicos.ufjf.br/index.php/hurevista/article/view/2902510.34019/1982-8047.2020.v46.29025HU Revista; v. 46 (2020); 1 - 71982-80470103-3123reponame:HU Revista (Online)instname:Universidade Federal de Juiz de Fora (UFJF)instacron:UFJFporhttps://periodicos.ufjf.br/index.php/hurevista/article/view/29025/20662Copyright (c) 2020 Bianca de Moraes Coimbra, Cilmário Leite da Silva Júnior, Vinícius Neves Marcos, Erick Sabbagh de Hollandainfo:eu-repo/semantics/openAccessde Moraes Coimbra, BiancaLeite da Silva Júnior, Cilmário Neves Marcos, ViníciusSabbagh de Hollanda, Erick2020-11-20T19:41:33Zoai:periodicos.ufjf.br:article/29025Revistahttps://periodicos.ufjf.br/index.php/hurevistaPUBhttps://periodicos.ufjf.br/index.php/hurevista/oairevista.hurevista@ufjf.edu.br1982-80470103-3123opendoar:2020-11-20T19:41:33HU Revista (Online) - Universidade Federal de Juiz de Fora (UFJF)false
dc.title.none.fl_str_mv Frantz's tumor, a diagnostic challenge: case report
Tumor de Frantz, um desafio diagnóstico: relato de caso
title Frantz's tumor, a diagnostic challenge: case report
spellingShingle Frantz's tumor, a diagnostic challenge: case report
de Moraes Coimbra, Bianca
Pancreatic Neoplasms
Duodenal Ulcer
Anemia
Neoplasias Pancreáticas
Úlcera Duodenal
Anemia
title_short Frantz's tumor, a diagnostic challenge: case report
title_full Frantz's tumor, a diagnostic challenge: case report
title_fullStr Frantz's tumor, a diagnostic challenge: case report
title_full_unstemmed Frantz's tumor, a diagnostic challenge: case report
title_sort Frantz's tumor, a diagnostic challenge: case report
author de Moraes Coimbra, Bianca
author_facet de Moraes Coimbra, Bianca
Leite da Silva Júnior, Cilmário
Neves Marcos, Vinícius
Sabbagh de Hollanda, Erick
author_role author
author2 Leite da Silva Júnior, Cilmário
Neves Marcos, Vinícius
Sabbagh de Hollanda, Erick
author2_role author
author
author
dc.contributor.author.fl_str_mv de Moraes Coimbra, Bianca
Leite da Silva Júnior, Cilmário
Neves Marcos, Vinícius
Sabbagh de Hollanda, Erick
dc.subject.por.fl_str_mv Pancreatic Neoplasms
Duodenal Ulcer
Anemia
Neoplasias Pancreáticas
Úlcera Duodenal
Anemia
topic Pancreatic Neoplasms
Duodenal Ulcer
Anemia
Neoplasias Pancreáticas
Úlcera Duodenal
Anemia
description Introduction: Pseudopapillary solid pancreatic tumor (Frantz's tumor) is a solid-cystic exocrine cell neoplasm that accounts for about 0.2-2.7% of all pancreatic tumors. It is a rare disease with low potential for malignancy, preferentially affecting young women, with an average age of 25 years. Complete surgical resection of the lesion is the treatment of choice, and the prognosis is excellent. Objective: To report a challenging case of Frantz's tumor with atypical clinical-radiological presentation, in which imaging exams were fundamental for the diagnostic elucidation and therapeutic management. Case Report: 31-year-old female patient with chronic anemia, hematemesis, melena and hematochezia. Upper gastrointestinal endoscopy showed an active ulcer in the duodenal bulb. Tomography revealed an expansive mass located between the pancreatic head and the duodenum, with signs of ulceration. At this time, the main diagnostic hypotheses were gastrointestinal stromal tumor (GIST) or Frantz tumor. Magnetic resonance imaging characterized an intimate relationship of the lesion with pancreatic tissue, making the diagnosis of Frantz tumor as the best hypothesis. Then, the patient was submitted to total duodenopancreatectomy for resection and definitive treatment of the lesion. Immunohistochemistry revealed cells with indistinct eosinophilic cytoplasm and hyperchromatic nuclei, sometimes with pseudopapillary arrays, as well as expression for beta-catenin in nuclear pattern and positivity for CD10 and CD99, confirming that it is a solid pseudopapillary tumor of the pancreas. Conclusion: The importance of imaging tests for characterization and assertive diagnosis is observed of this type of tumor, allowing the complete lesion resection and perspective of cure.
publishDate 2020
dc.date.none.fl_str_mv 2020-06-08
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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dc.identifier.uri.fl_str_mv https://periodicos.ufjf.br/index.php/hurevista/article/view/29025
10.34019/1982-8047.2020.v46.29025
url https://periodicos.ufjf.br/index.php/hurevista/article/view/29025
identifier_str_mv 10.34019/1982-8047.2020.v46.29025
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://periodicos.ufjf.br/index.php/hurevista/article/view/29025/20662
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publisher.none.fl_str_mv Editora UFJF
dc.source.none.fl_str_mv HU Revista; v. 46 (2020); 1 - 7
1982-8047
0103-3123
reponame:HU Revista (Online)
instname:Universidade Federal de Juiz de Fora (UFJF)
instacron:UFJF
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instacron_str UFJF
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reponame_str HU Revista (Online)
collection HU Revista (Online)
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