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.

Neoplasia papilomucinosa intraductal de páncreas: experiencia a 10 años, en un único centro / Intraductal papillary mucinous neoplasm of the pancreas: A 10-year experience at a single center

RESUMEN Antecedentes: Debido a la mejoría de los métodos diagnósticos, la incidencia de la neoplasia papilar mucinosa intraductal del páncreas (NPMI) ha aumentado en los últimos años. Objetivo: Presentar la experiencia en el manejo alejado de pacientes con NPMI en un centro de referencia. Material y métodos : Se realizó análisis retrospectivo de pacientes que consultaron al Servicio de Ci rugía General y HPB del Hospital Universitario Fundación Favaloro, por patología pancreática tumo ral entre enero de 2010 y diciembre de 2019. Los pacientes se clasificaron en 2 grupos: A) aquellos con diagnóstico en consultorio de NPMI y B) aquellos en los que la NPMI fue un hallazgo en la pieza quirúrgica (B). Resultados: Ochenta y seis pacientes fueron analizados: 79 (90%) se incluyeron en el grupo A y 7 en el grupo B. De los pacientes del grupo A, en 57 casos (66%) se decidió conducta expectante y seguimiento (AS). De los 22 pacientes restantes, 18 fueron operados (29%) (AC) y 4 tienen la cirugía pendiente. Para el grupo A, la supervivencia a los 5 años fue de 89% para el grupo AS, de 86% en grupo AC, y del 43% para el grupo B (Breslow 0,001, Log-Rank 0,001 vs. grupo A). Conclusión: El diagnóstico y manejo de la NPMI está hoy estandarizado, en las tipo I y III está indicada cirugía, la tipo II debe seguirse por riesgo de malignización; cuando se le indica cirugía, la supervivencia a largo plazo debe ser similar a la del grupo en seguimiento.

ABSTRACT Background: The incidence of intraductal papillary mucinous neoplasm (IPMN) of the pancreas has increased over the past years along with the development of diagnostic imaging tests. Objective: The aim of this study is to describe our experience on long-term management of patients with IPMNs in a reference center. Material and methods: We conducted a retrospective and descriptive analysis of patients with pancreatic neoplasms followed-up at the Department of General Surgery and Hepato-Biliary Surgery, Hospital Universitario Fundación Favaloro, between January 2010 and December 2019. The patients were classified into 2 groups: group A (diagnosis of IPMN made in the outpatient clinic), and group B (diagnosis of IPMN in the pathological examination). Results: Eighty-six patients were analyzed: 79 (90%) in group A and 7 in group B. In group A, a watchful waiting with monitoring (AM) was decided in 57 cases (66%). Of the remaining 22 patients, 18 (29%) patients underwent surgery (AS) and 4 are waiting for surgery. Survival at 5 years was 89% in group AM, 86% in group AS and 43% in group B (Breslow 0.001, log-rank test 0,001 vs. group A). Conclusion : The diagnosis and management of IPMNs is currently standardized. Surgery is indicated in MD-IPMN and mixed type IPMN. Patients with BD-IPMN type should be monitored due to the risk of malignant transformation. When surgery is indicated, long-term survival should be similar to that of the surveillance group.

Adenocarcinoma mucinoso de vesícula biliar originado en neoplasia papilar intracolecística / Mucinous adenocarcinoma of the gallbladder originated from intracholecystic papillary neoplasm

RESUMEN La neoplasia papilar intracolecística con carcinoma mucinoso invasor y células en anillo de sello es una variedad de cáncer de vesícula, una patología agresiva y con mal pronóstico, la cual puede presentarse en forma similar a una colecistitis y, a pesar del tratamiento operatorio y quimioterapia posterior, la supervivencia y pronóstico son peores en relación con los otros cánceres de vesícula. Este artículo tie ne por objetivo describir un tipo histológico muy específico, de baja frecuencia, de cáncer de vesícula y el tratamiento realizado.

ABSTRACT Intracholecystic papillary neoplasm with invasive mucinous adenocarcinoma and signet ring cells is a rare, aggressive variety of gallbladder cancer, with symptoms mimicking cholecystitis. Survival and prognosis are worse that other types of gallbladder cancer despite surgery and chemotherapy. The aim of this article is to describe a case of a rare gallbladder cancer with specific histology and the treatment performed

Cystic liver neoplams: A single centre experience and literature review. / Neoplasias quísticas hepáticas: experiencia en un único centro y revisión de la literatura.

INTRODUCTION: The hepatic cystic tumour is a very rare neoplasm, representing about 5% of all cystic liver neoplasms. The preoperative diagnosis is difficult and can lead to confusion. The aim of this study is to analyze a number of cases operated at our centre with an histologic diagnosis of liver cystic neoplasms and also to describe the sintomathology, diagnosis and management as per the recent classification. METHODS: A retrospective analysis was performed including all the cystic liver neoplasms operated between January 2000 and December 2019. The study was performed based on the pre-existing pathology archives. The 2010 previous cases were reclassified following the new 2010 OMS classification. RESULTS: The study sample was of 10 patients, identifying 6 of them as mucinous cystic liver neoplasms, and the other 4 as intraductal papillary biliary neoplasms. The majority of the patients were women (8/10) and the median age was 47 years. Regarding the treatment, 3 hepatectomy and 7 enucleations were performed. Frozen section intraoperatively was not required in any case. In one case, variable cellular atypia with areas of adenocarcinoma was observed, and the patient received neoadyuvant chemotherapy with taxol and carboplatin. In all cases the resection margins were negative. CONCLUSION: Cystic liver neoplasms are infrequent tumours with a difficult differential diagnosis. Therefore, with a high radiological suspicious, the treatment should be a complete resection to avoid recurrences and malignancies.

Intraductal papillary neoplasm of the bile duct: Radiologic findings in a new disease. / Neoplasia papilar intraductal de la vía biliar: radiología en una nueva entidad.

Intraductal papillary neoplasm of the biliary tract (B-IPN) is a scarcely known entity in our daily practice due to its low prevalence. Until its new definition in the fourth edition of the WHO classification of the digestive tract tumors of 2010 the disease was grouped under a heterogeneous and imprecise terminology. In addition, in recent years there has been progress in the knowledge of its etiopathogenesis, its natural history and its findings in image. The purpose of this paper is to review these data underlining the radiological findings of the disease and its differential diagnosis.

Neoplasia papilar intracolecística: reporte de un caso y revisión de la evidencia existente / Intracholecystic papillary neoplasia: report of a case and review of existing evidence

La neoplasia papilar intracolecística (NPIC), es un tumor compuesto por células neoplásicas preinvasivas, que forman masas de hasta 1,0 cm, clínicamente detectables. El objetivo de este estudio fue reportar un caso de NPIC y revisar la evidencia existente. Se trata de un paciente sexo masculino, de 33 años de edad, asintomático, que en el curso de un examen de salud, se realiza una ecotomografía abdominal, en la que se verifica una lesión polipoide vesicular de 32 x 19 mm de diámetro. Se programa para colecistectomía electiva, la que se realizó por vía laparoscópica, cirugía que se pudo realizar sin inconvenientes. Una vez extirpado el espécimen, se fue a estudio histopatológico en el que tras un mapeo vesicular se concluye NPIC, colecistitis crónica inespecífica, colesterolosis y pólipos colesterínicos. El paciente ha evolucionado sin inconvenientes. Presentamos un caso de NPIC en un paciente joven, cuyo diagnóstico fue confirmado por anatomía patológica tras una colecistectomía electiva, descartándose la presencia de carcinoma invasivo y displasia de alto grado, por lo que el pronóstico es favorable.

Intracholecystic papillary neoplasm (ICPN) is a tumor composed of pre-invasive neoplastic cells, with up to 1.0 cm clinically detectable masses. The objective of this study was to report a case of NPIC and review the evidence in the literature. A 33-year-old asymptomatic male patient had an abdominal ultrasonography during a health examination, in which a vesicular polyp lesion of 32 x 19 mm in diameter was verified. Thepatient was subsequently scheduled for elective cholecystectomy, which was performed laparoscopically and the surgery was uneventful. Once removed, the specimen was studied histopathologically and after performing vesicular mapping, we determined an ICPN, chronic non-specific cholecystitis, cholesteroliasis and cholesteric polyps. The patient has evolved without reported problems. We present a case of ICPN in a young patient, whose diagnosis was confirmed by pathological anatomy after an elective cholecystectomy, ruling out the presence of invasive carcinoma and high-grade dysplasia, with a favorable prognosis.

Manejo de las lesiones mamarias de alto riesgo diagnosticadas mediante biopsia percutánea

La presencia de una lesión de riesgo en la mama aumenta la probabilidad de desarrollar un carcinoma o puede indicar la existencia de malignidad concomitante. El manejo adecuado posterior al diagnóstico percutáneo es muchas veces controvertido, por lo que es importante que el radiólogo conozca las implicancias diagnósticas y terapéuticas de las lesiones mamarias de alto riesgo para su detección y seguimiento. Este trabajo tiene como objetivo revisar la literatura y presentar la evidencia actual sobre el manejo de las hiperplasias atípicas ductal y lobulillar, el carcinoma lobulillar in situ, la cicatriz radiada y las lesiones columnares, papilares y de tipo mucocele, luego del diagnóstico con técnicas de intervencionismo percutáneo.

The presence of a high-risk breast lesion confers a higher probability to develop a breast tumour or may indicate concomitant malignancy. The management of such lesions after percutaneous biopsy remains controversial. Radiologists should be familiar with the diagnosis and management for the follow up of high risk lesions of the breast. The purpose of this study is to present current evidence for the management of atypical duct hyperplasia, lobular hyperplasia, radial scar, spinal column lesions, papillary lesions and mucocele-like lesions.