Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report

Detalhes bibliográficos
Autor(a) principal: de Azevedo Souza Junior, Walberto
Data de Publicação: 2023
Outros Autores: Kuerten Pamplona da Silva, Laura, Cerveira Cardoso Vilela, Laura, El-Jaick Bonifacio Costa, Tatiana, Grechi Camacho, Johnny
Tipo de documento: Artigo
Idioma: por
Título da fonte: Revista Brasileira de Cancerologia (Online)
Texto Completo: https://rbc.inca.gov.br/index.php/revista/article/view/4350
Resumo: Introduction: The solid pseudopapillary tumor of the pancreas is a rare pathology, accounting for less than 3% of all pancreatic exocrine tumors. This tumor occurs mainly in young women, between 20 and 30 years old, has low malignant potential, causing symptoms only when they arelarge. Case report: Female patient, 14 years of age, presented hematemesis and varicose veins in the gastric fundus. After submitting to computed tomography, a hypodense mass of hypovascular behavior was shown inthe pancreatic tail, raising the suspicion of primary pancreatic neoplasia promoting compression of the splenic vein. The patient underwent bodycaudal pancreatectomy associated with splenectomy in February 2021. The biopsy of the hepatic nodule, evidenced on abdominal ultrasound, confirmed metastasis and the patient underwent subsequent hepatic segmentectomy in July 2021. Conclusion: The case reported is extremely rare because it is a Frantz tumor in childhood associated with left portal hypertension due to tumor compression of the splenic vein, which consequently presented upper digestive hemorrhage as an initial clinical manifestation; similar cases are not easily found in the literature in the pediatric age range.
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spelling Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case ReportAsociación Entre Tumor Pancreático Pseudopapilar Sólido (Tumor de Frantz) e Hipertensión Portal Izquierda en Pediatría: Reporte de CasoAssociação entre Tumor Sólido Pseudopapilar Pancreático (Tumor de Frantz) e Hipertensão Portal Esquerda em Pediatria: Relato de Casopancreaspediatricshypertension, portalpáncreaspediatríahipertensión portalpâncreaspediatriahipertensão portalIntroduction: The solid pseudopapillary tumor of the pancreas is a rare pathology, accounting for less than 3% of all pancreatic exocrine tumors. This tumor occurs mainly in young women, between 20 and 30 years old, has low malignant potential, causing symptoms only when they arelarge. Case report: Female patient, 14 years of age, presented hematemesis and varicose veins in the gastric fundus. After submitting to computed tomography, a hypodense mass of hypovascular behavior was shown inthe pancreatic tail, raising the suspicion of primary pancreatic neoplasia promoting compression of the splenic vein. The patient underwent bodycaudal pancreatectomy associated with splenectomy in February 2021. The biopsy of the hepatic nodule, evidenced on abdominal ultrasound, confirmed metastasis and the patient underwent subsequent hepatic segmentectomy in July 2021. Conclusion: The case reported is extremely rare because it is a Frantz tumor in childhood associated with left portal hypertension due to tumor compression of the splenic vein, which consequently presented upper digestive hemorrhage as an initial clinical manifestation; similar cases are not easily found in the literature in the pediatric age range.Introducción: El tumor sólido pseudopapilar de páncreas es una patología rara, representando menos del 3% de todos los tumores pancreáticos exocrinos. Este tumor se presenta principalmente en mujeres jóvenes, entre 20 y 30 años, generalmente tiene un bajo potencial maligno y causa síntomas sólo cuando son de gran tamaño. Informe del caso: Paciente femenino de 14 años de edad, inició con hematemesis y se identificaron varices en fondo gástrico. Tras realizar tomografía computarizada se evidenció una masa hipodensa de comportamiento hipovascular en la cola pancreática, lo que hizo sospechar de neoplasia pancreática primaria promoviendo la compresión de la vena esplénica. La paciente fue sometida a pancreatectomía cuerpo-caudal asociada a esplenectomía en febrero de 2021. La biopsia del nódulo hepático, evidenciada en ecografía abdominal, confirmó metástasis y la paciente fue sometida a posterior segmentectomía hepática en julio de 2021. Conclusión: El caso reportado es extremadamente raro por tratarse de un tumor de Frantz en la infancia asociado a hipertensión portal izquierda por compresión tumoral de la vena esplénica y que, en consecuencia, presentó como manifestación clínica inicial hemorragia digestiva alta, no encontrándose fácilmente en la literatura casos similares en el rango de edad pediátrica.Introdução: O tumor sólido pseudopapilar do pâncreas é uma patologia rara responsável por menos de 3% de todos os tumoresexócrinos pancreáticos. Tal tumor ocorre principalmente em mulheres jovens, entre 20 e 30 anos, e geralmente possui baixo potencialde malignidade e causa sintomas apenas quando são de grandes volumes. Relato do caso: Paciente feminina, 14 anos, iniciou quadro de hematêmese, sendo identificadas varizes de fundo gástrico. Após realizar tomografia computadorizada, evidenciou-se massa hipodensa de comportamento hipovascular na cauda pancreática, levantando-se a suspeita de neoplasia pancreática primária, promovendo compressão da veia esplênica. A paciente foi submetida à pancreatectomia corpo-caudal associada à esplenectomia em fevereiro de 2021. A biópsia do nódulo hepático, evidenciado em ultrassonografia abdominal, confirmou metástase, e a paciente foi submetida à posterior segmentectomia hepática em julho de 2021. Conclusão: O caso relatado é extremamente raro por tratar-se de um tumor de Frantz na infância associado à hipertensão portal esquerda por compressão tumoral da veia esplênica e que, por consequência, apresentou hemorragia digestiva alta como manifestação clínica inicial, não sendo encontrados facilmente na literatura casos similares na faixa pediátrica.INCA2023-12-14info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelato de Casoapplication/pdftext/htmlhttps://rbc.inca.gov.br/index.php/revista/article/view/435010.32635/2176-9745.RBC.2023v69n4.4350Revista Brasileira de Cancerologia; Vol. 69 No. 4 (2023): oct./nov./dec.; e-144350Revista Brasileira de Cancerologia; Vol. 69 Núm. 4 (2023): oct./nov./dic.; e-144350Revista Brasileira de Cancerologia; v. 69 n. 4 (2023): out./nov./dez.; e-1443502176-9745reponame:Revista Brasileira de Cancerologia (Online)instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)instacron:INCAporhttps://rbc.inca.gov.br/index.php/revista/article/view/4350/3275https://rbc.inca.gov.br/index.php/revista/article/view/4350/3290Copyright (c) 2023 Os direitos morais, intelectuais e de publicação dos artigos pertencem aos respectivos autores de forma irrestrita, não sendo propriedade da revistahttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessde Azevedo Souza Junior, WalbertoKuerten Pamplona da Silva, LauraCerveira Cardoso Vilela, LauraEl-Jaick Bonifacio Costa, TatianaGrechi Camacho, Johnny2023-12-26T16:14:07Zoai:rbc.inca.gov.br:article/4350Revistahttps://rbc.inca.gov.br/index.php/revistaPUBhttps://rbc.inca.gov.br/index.php/revista/oairbc@inca.gov.br0034-71162176-9745opendoar:2023-12-26T16:14:07Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)false
dc.title.none.fl_str_mv Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
Asociación Entre Tumor Pancreático Pseudopapilar Sólido (Tumor de Frantz) e Hipertensión Portal Izquierda en Pediatría: Reporte de Caso
Associação entre Tumor Sólido Pseudopapilar Pancreático (Tumor de Frantz) e Hipertensão Portal Esquerda em Pediatria: Relato de Caso
title Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
spellingShingle Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
de Azevedo Souza Junior, Walberto
pancreas
pediatrics
hypertension, portal
páncreas
pediatría
hipertensión portal
pâncreas
pediatria
hipertensão portal
title_short Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
title_full Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
title_fullStr Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
title_full_unstemmed Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
title_sort Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
author de Azevedo Souza Junior, Walberto
author_facet de Azevedo Souza Junior, Walberto
Kuerten Pamplona da Silva, Laura
Cerveira Cardoso Vilela, Laura
El-Jaick Bonifacio Costa, Tatiana
Grechi Camacho, Johnny
author_role author
author2 Kuerten Pamplona da Silva, Laura
Cerveira Cardoso Vilela, Laura
El-Jaick Bonifacio Costa, Tatiana
Grechi Camacho, Johnny
author2_role author
author
author
author
dc.contributor.author.fl_str_mv de Azevedo Souza Junior, Walberto
Kuerten Pamplona da Silva, Laura
Cerveira Cardoso Vilela, Laura
El-Jaick Bonifacio Costa, Tatiana
Grechi Camacho, Johnny
dc.subject.por.fl_str_mv pancreas
pediatrics
hypertension, portal
páncreas
pediatría
hipertensión portal
pâncreas
pediatria
hipertensão portal
topic pancreas
pediatrics
hypertension, portal
páncreas
pediatría
hipertensión portal
pâncreas
pediatria
hipertensão portal
description Introduction: The solid pseudopapillary tumor of the pancreas is a rare pathology, accounting for less than 3% of all pancreatic exocrine tumors. This tumor occurs mainly in young women, between 20 and 30 years old, has low malignant potential, causing symptoms only when they arelarge. Case report: Female patient, 14 years of age, presented hematemesis and varicose veins in the gastric fundus. After submitting to computed tomography, a hypodense mass of hypovascular behavior was shown inthe pancreatic tail, raising the suspicion of primary pancreatic neoplasia promoting compression of the splenic vein. The patient underwent bodycaudal pancreatectomy associated with splenectomy in February 2021. The biopsy of the hepatic nodule, evidenced on abdominal ultrasound, confirmed metastasis and the patient underwent subsequent hepatic segmentectomy in July 2021. Conclusion: The case reported is extremely rare because it is a Frantz tumor in childhood associated with left portal hypertension due to tumor compression of the splenic vein, which consequently presented upper digestive hemorrhage as an initial clinical manifestation; similar cases are not easily found in the literature in the pediatric age range.
publishDate 2023
dc.date.none.fl_str_mv 2023-12-14
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Relato de Caso
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dc.identifier.uri.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/4350
10.32635/2176-9745.RBC.2023v69n4.4350
url https://rbc.inca.gov.br/index.php/revista/article/view/4350
identifier_str_mv 10.32635/2176-9745.RBC.2023v69n4.4350
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/4350/3275
https://rbc.inca.gov.br/index.php/revista/article/view/4350/3290
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info:eu-repo/semantics/openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by/4.0
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
text/html
dc.publisher.none.fl_str_mv INCA
publisher.none.fl_str_mv INCA
dc.source.none.fl_str_mv Revista Brasileira de Cancerologia; Vol. 69 No. 4 (2023): oct./nov./dec.; e-144350
Revista Brasileira de Cancerologia; Vol. 69 Núm. 4 (2023): oct./nov./dic.; e-144350
Revista Brasileira de Cancerologia; v. 69 n. 4 (2023): out./nov./dez.; e-144350
2176-9745
reponame:Revista Brasileira de Cancerologia (Online)
instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron:INCA
instname_str Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron_str INCA
institution INCA
reponame_str Revista Brasileira de Cancerologia (Online)
collection Revista Brasileira de Cancerologia (Online)
repository.name.fl_str_mv Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
repository.mail.fl_str_mv rbc@inca.gov.br
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