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

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. (AU)

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. (AU)

Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis.

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 13 mm, 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.

Eficacia del tratamiento con toxina botulínica en la hiperhidrosis palmar en nuestro medio / Efficacy of botulinum toxin in palmar hyperhidrosis in our environment

Introducción. La hiperhidrosis primaria (HP) es un trastorno de etiología desconocida caracterizada por una secreción incontrolada y excesiva de sudor en la superficie de las palmas de las manos, las axilas, las ingles y las plantas de los pies debido a un incremento de la actividad simpático colinérgica de los nervios. El objetivo de este estudio es determinar la eficacia de la toxina botulínica en esta afección. Material y método. Estudio prospectivo de 16 pacientes con hiperhidrosis severa (grado iv), a los que se les realizó la encuesta hyperhidrosis disease severity scale antes del tratamiento, al mes, a los 3 meses y a los 6 meses. Resultados. Un paciente fue no respondedor primario, por lo que abandonó el estudio. De los 15 restantes el 93,8% mejora, pasando de un estadio 4 al 1 a los 3 meses, y solo uno pasa de un estadio 4 a 3. A los 6 meses 3 empeoran pasando de un nivel 1 a nivel 3, mientras que el resto se mantiene en estadio 1. El cambio es estadísticamente significativo entre el inicio y el mes (p < 0,001) y el cambio es estadísticamente significativo entre el inicio y los 6 meses (p < 0,001). El empeoramiento de algunos pacientes no es estadísticamente significativo (p = 0,083). Entre los efectos secundarios observados: un caso de debilidad de la eminencia tenar autolimitada a 4 semanas. Conclusiones. La toxina botulínica es un tratamiento eficaz y seguro en el tratamiento de la hiperhidrosis palmar severa, realizándose un mejor control del dolor mediante bloqueo locorregional. Es necesario estandarizar la dosis a utilizar (AU)

Introduction. Primary hyperhidrosis (PH) is a disorder of unknown etiology characterized by uncontrolled and excessive secretion of sweat on the surface of the palms, armpits, groin and soles of the feet due to increased cholinergic activity of the sympathetic nerves. The aim of this study was to determine the effectiveness of botulinum toxin in this disease. Material and method. A prospective study was conducted in 16 patients with severe hyperhidrosis (grade IV). The Hyperhidrosis Disease Severity Scale was administered before treatment, and at 1, 3, and 6 months after treatment. Results. One patient was not the primary responder and therefore withdrew from the study. Of the 15 remaining patients, 93.8% improved, with a reduction in hyperhidrosis stage from 4 at baseline to 1 at 3 months. Only one patient showed a reduction in stage from 4 to 3. At 6 months, 3 patients showed a worsening, with an increase in stage from 1 to 3, while stage 1 was maintained in the remainder. The change was statistically significant between baseline and 1 month (P < .001) and between baseline and 6 months (P < .001). Worsening in some patients was not statistically significant (P = .083). Adverse effects included 1 case of self-limiting weakness of the thenar that resolved at 4 weeks. Conclusions. Botulinum toxin is safe and effective in treating severe palmar hyperhidrosis. Pain control was better through locoregional block. The dose used should be standardized (AU)