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Inicio Actas Urológicas Españolas Neoplasia papilar de polaridad inversa: un nuevo subtipo de tumor renal de buen ...
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Vol. 46. Issue 10.
Pages 600-605 (December 2022)
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Vol. 46. Issue 10.
Pages 600-605 (December 2022)
Artículo original
Neoplasia papilar de polaridad inversa: un nuevo subtipo de tumor renal de buen pronóstico
Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis
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M. Conde-Ferreirósa,
Corresponding author
martaconfe@gmail.com

Autor para correspondencia.
, J. Domínguez-de Diosa, L. Juaneda-Magdalenaa, A. Bellas-Pereirab, M.P. San Miguel Frailea, M.Á. Peteiro Canceloa, C. Gómez-de Maríaa, M. Álvarez Sarriaa, M.Á. Betancor Santosa, J. González-Carreróa, J.A. Ortiz-Reya
a Departamento de Anatomía Patológica, Hospital Álvaro Cunqueiro, Vigo, Pontevedra, España
b Departamento de Urología, Hospital Álvaro Cunqueiro, Vigo, Pontevedra, España
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Abstract
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Resumen
Introducción

La neoplasia papilar renal de polaridad inversa (NPRPI) ha sido recientemente reconocida como una entidad separada de la clasificación tradicional de los carcinomas papilares de células renales por sus peculiares características histopatológicas, inmunofenotípicas y moleculares, y un comportamiento indolente.

Material y métodos

En este trabajo aportamos 6 nuevos casos y realizamos una revisión de la literatura publicada al respecto hasta el momento actual, recopilándose un total de 104 casos.

Resultados

Nuestros casos de NPRPI corresponden a 5 hombres y una mujer, con edades comprendidas entre los 47 y los 91 años. En 5 casos la NPRPI resultó un hallazgo incidental en piezas de nefrectomía indicada por la presencia de otro tumor renal y en uno la NPRPI fue el motivo de la intervención quirúrgica. Nuestros casos presentan tamaños entre los 2 y los 13mm, y una histología papilar con revestimiento en monocapa de células eosinófilas con núcleos de bajo grado en localización apical. Inmunohistoquímicamente muestran una constante positividad para GATA3 y negatividad para vimentina. Se identificaron mutaciones en KRAS en el 50% de ellos. Tras un seguimiento comprendido entre uno y 60 meses, 5 de los pacientes seguían vivos sin recurrencia o metástasis y uno falleció a causa de un carcinoma urotelial.

Conclusiones

Nuestros casos concuerdan con las características clínicas y patológicas descritas en los publicados hasta el momento. Aportamos la primera serie nacional y corroboramos la existencia de unos criterios diagnósticos definidos y constantes que permiten considerar la NPRPI como una entidad propia distintiva.

Palabras clave:
Carcinoma papilar de células renales
Neoplasia papilar de polaridad inversa
KRAS
Carcinoma de células renales
Abstract
Introduction

Papillary renal cell neoplasm with reverse polarity (PRNRP) has recently been recognized as an entity separate from the traditional classification of papillary renal cell carcinomas, due to its specific histopathological, immunophenotypic and molecular characteristics, as well as its indolent behavior.

Material and methods

We provide 6 new cases and a review of the literature published until the present time, which comprises a total number of 104 cases.

Results

Our PRNRP cases correspond to 5 men and one woman aged between 47 and 91 years. In 5 of the 6 cases, the PRNRP was an incidental finding in nephrectomy specimens. Nephrectomy had been indicated due to the presence of another renal tumor, except for one case, in which surgical intervention was indicated due to PRNRP. Our cases present mass sizes between 2 and 13mm, as well as papillary histology with a monolayered lining of eosinophilic cells with low-grade nuclei in apical location. Immunohistochemically, they show a constant positivity for GATA3 and negativity for vimentin. KRAS mutations were identified in 50% of our cases. After a follow-up ranging between one and 60 months, 5 of the cases were still alive without recurrences or metastases, and one died from urothelial carcinoma.

Conclusions

Our cases agree with the clinical and pathological characteristics described in the PRNRP cases published to date. With the present study, we provide the first series of national cases corroborating the existence of well-defined and constant diagnostic criteria that allow PRNRP to be considered as a distinctive entity.

Keywords:
Papillary renal cell carcinoma
Papillary renal cell neoplasm with reverse polarity
KRAS
Renal cell carcinoma

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