Frantz's tumor, a diagnostic challenge: case report
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , , |
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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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 info:eu-repo/semantics/publishedVersion RelCas |
format |
article |
status_str |
publishedVersion |
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 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Editora UFJF |
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 |
instname_str |
Universidade Federal de Juiz de Fora (UFJF) |
instacron_str |
UFJF |
institution |
UFJF |
reponame_str |
HU Revista (Online) |
collection |
HU Revista (Online) |
repository.name.fl_str_mv |
HU Revista (Online) - Universidade Federal de Juiz de Fora (UFJF) |
repository.mail.fl_str_mv |
revista.hurevista@ufjf.edu.br |
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