Neoplasia papilar intraductal de la vía biliar / Intraductal papillary neoplasm of the bile duct / Neoplasia papilar intraductal da via biliar

La neoplasia papilar intraductal de la vía biliar (NPIVB) es una entidad infrecuente caracterizada por el crecimiento exofítico papilar del epitelio biliar hacia la luz ductal. Previamente incluida en el grupo de tumores del mismo nombre de localización pancreática, presenta diferencias evidentes con ellos y desde 2010 se considera una entidad propia con demostrado potencial de malignización hacia colangiocarcinoma.

Papillary intraductal neoplasia of the bile duct (NPIVB) is a rare entity characterized by exophytic papillary growth of the biliary epithelium towards the ductal lumen. Previously included in the group of tumors of the same name in pancreatic location, it presents obvious differences with them and since 2010 it has been considered a separate entity with demonstrated potential for malignancy towards cholangiocarcinoma.

O neoplasma papilar intraductal da via biliar (NPIVB) é uma entidade infrequente por el creciento exofítico papilar do epitélio biliar hacia la luz ductal. Obviamente incluído no grupo de tumores do mismo nombre de localização pancreática, apresenta diferenças evidentes com ellos e desde 2010 se considerar uma entidade propia com potencial demonstrado de malignización hacia cholangiocarcinoma.

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.

The rare case of a cystic pancreatic neuroendocrine tumor

The pancreatic neuroendocrine tumors (PanNETs) most commonly present as solid neoplasms; however, very rarely, they may present primarily as cystic neoplasms. Most of the cystic PanNETs are non-secreting tumors, and the radiological features are not well defined. Hence pre-operative diagnosis is usually challenging and the tumors are misdiagnosed as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, serous cystic neoplasms, solid pseudopapillary neoplasms, and non-neoplastic cysts. However, the management depends on the accurate diagnosis of these cystic lesions, which poses a dilemma. Herein, we report the case of a cystic PanNET in the tail of the pancreas, which was clinically and radiologically misdiagnosed as a mucinous cystic neoplasm. This case is reported to highlight this issue to the medical community regarding the diagnostic difficulty in such rare non-functioning pancreatic neuroendocrine tumors.

Magnetic resonance cholangiopancreatography (mrcp) versus endosonography-guided fine needle aspiration (eus-fna) for diagnosis and follow-up of pancreatic intraductal papillary mucinous neoplasms / Análise comparativa entre colangiopancreatoressonância magnética (cprm) e punção ecoguiada com agulha fina (epaaf) para diagnóstico e seguimento das neoplasias intraductais mucinosas papilíferas pancreáticas

ABSTRACT Background: Intraductal papillary mucinous tumor (IPMN) are being diagnosed with increasing frequency. Computerized tomography scanning is commonly used as the primary imaging modality before surgery nonetheless magnetic resonance cholangiopancreatography (MRCP) provides better characterization. Endosonography-guided fine needle aspiration (EUS-FNA) has emerged as a way to reach pathological diagnose. Aim: To compare results of both methods with surgical pathology findings for classification of IPMN. Methods: Thirty-six patients submitted to surgical resection with preoperative suspect of IPMN were submitted preoperatively to MRCP and EUS-FNA. Images obtained were analyzed according to a classification determined for each method. ROC curve was used for statistical analysis, that compared the images tests with the purpose of finding the best method for diagnosis and classification of IPMN. Results: Sixteen patients underwent pancreatoduodenectomy, 16 to subtotal pancreatectomy and only four laparotomy. Pathological diagnosis was IPMN (n=33) and pancreatic intraepithelial neoplasia type 2 (n=3). Twenty-nine revealed non-invasive neoplasia and invasive form in four patients. MRCP and EUS-FNA have correctly diagnosed and classified (type of IPMN), in 62.5% and 83.3% (p=0.811), the affected segment location in 69% and 92% (p=0.638) and identification of nodules and/or vegetation presence in 45% and 90% (p=0.5). Regarding to histopathological diagnosis by EUS-FNA the sensitivity was 83.3%; specificity was 100%; positive predictive value was 100%; negative predictive value was 33.3% and accuracy was 91.7%. Conclusions: There was no significant difference in the diagnosis of IPMN. However, EUS-FNA showed better absolute results than MRCP to identify nodule and/or vegetation.

RESUMO Racional: A neoplasia intraductal mucinosa papilífera (NIMP) está sendo diagnosticada com maior frequência. O método mais utilizado para diagnóstico é a tomografia computadorizada. No entretanto, a colangiopancreatoressonância (CPRM) proporciona melhor caracterização tipo e extensão. A ecoendoscopia com punção por agulha fina (EPAAF), por sua vez, permite o diagnóstico histológico. Objetivo: Comparar resultados da CPRM e EPAAF com os achados cirúrgicos e patológicos para o diagnóstico e classificação da NIMP. Método: Foram estudados trinta e seis pacientes submetidos à ressecção cirúrgica por suspeita de NIMP que foram submetidos à CPRM e EPAAF pré-operatórias. Imagens obtidas por ambos os métodos foram analisadas utilizando-se padronização contendo o tipo e a classificação da lesão e os achados foram comparados, tendo como referência a análise patológica do espécime cirúrgico para definir-se qual o melhor método na caracterização do NIMP. Resultados: Vinte e nove revelaram neoplasia não-invasiva e quatro invasiva. A CPRM e a EPAAF fizeram o diagnóstico e classificaram corretamente (tipo de NIMP) em 62,5% e 83,3% (p=0,811), a localização do segmento pancreático acometido em 69% e 92% (p=0,638) e a identificação da presença de nódulos e/ou vegetações em 45% e 90 % (p=0,5). Quanto ao diagnóstico histológico pela EPAAF a sensibilidade foi 83,3%; especificidade 100%; VPP 100%; VPN 33,3%; e acurácia 91,7%. Conclusões: Os métodos diagnósticos não apresentaram diferença estatística. No entanto, a EPAAF mostrou resultados absolutos melhores do que a CPRM na identificação de nódulo e/ou vegetação intracístico.

Cáncer de páncreas: alteraciones genéticas, cambios morfológicos y sus implicaciones terapéuticas / Pancreas cancer: genetic alterations, morphological changes and its therapeutic implications

Resumen El cáncer de páncreas es una enfermedad mortal, principalmente porque se descubre muy tarde y es muy resistente a la quimioterapia y radioterapia. El tipo más común de cáncer de páncreas (más del 90%) se desarrolla a partir de las células exocrinas del páncreas y se denomina adenocarcinoma ductal pancreático (ACDP). Se han reconocido tres lesiones precursoras de cáncer de páncreas ductal: neoplasia intraepitelial pancreática (PanIN), neoplasia papilar-mucinosa intraductal (NPMI) y neoplasia quística mucinosa (NCM). Uno de los primeros eventos genéticos implicados en la patogénesis de ACDP es una mutación en el punto de activación en el oncogén KRAS, una mutación conductora oncogénica que se encuentra en más del 90% de todos los cánceres de páncreas. Además, se ha informado que hasta un 10% de los cánceres de páncreas están asociados con el historial familiar. Aunque la causa es multifactorial, el tabaquismo y la historia familiar son dominantes. Existe heterogeneidad morfológica en muchos cánceres humanos, pero parece ser un hallazgo particularmente común en ACDP. La cirugía solo es posible en 15-20% de los casos: tumores confinados al páncreas con posible afectación ganglionar local no muy extensa y que no produce afectación vascular o está limitada.

Abstract Pancreatic cancer is a deadly disease, mainly because it is generally discovered very late and it is very resistant to chemotherapy and radiation therapy. The most common type of pancreatic cancer (over 90%) develops from the exocrine cells of the pancreas and is named pancreatic ductal adenocarcinoma (PDAC). Three precursor lesions of ductal pancreatic cancer have been recognized: pancreatic intraepithelial neoplasia (PanIN), intraductal papillary-mucinous neoplasm (IPMN) and mucinous cystic neoplasm (MCN). One of the earliest genetic events involved in PDAC pathogenesis is an activating point mutation in the KRAS oncogene, an oncogenic driver mutation found in more than 90% of all pancreatic cancer. In addition, it has been reported that up to 10% of pancreatic cancer is associated with family history. Although the cause is multifactorial, cigarette smoking and family history are dominant. Morphological heterogeneity exists in many human cancers, but seems to be a particularly common finding in PDAC. Surgery is only possible in 15-20% of cases: tumors confined to the pancreas with possible local nodal involvement not very extensive and that do not produce vascular involvement or is limited.

Neoplasia mucinosa papilar intraductal como hallazgo en paciente con pancreatitis / Intraductal papillary mucinous neoplasm as a finding in a patient with pancreatitis

Introducción: La neoplasia mucinosa intraductal papilar (NMIP) del páncreas es una masa quística dependiente del sistema ductal pancreático. Presentación del caso: Paciente de 65 años, femenino, antecedentes mórbidos de diabetes mellitus tipo 2 sin tratamiento e hipertensión arterial esencial en tratamiento. Consulta por cuadro clínico de 9 horas caracterizado por epigastralgia opresiva/urente de inicio súbito, con irradiación en faja a dorso, intensidad EVA 10/10, asociado a náuseas, sin vómitos, diarrea ni fiebre. Ingresa hemodinámicamente estable, afebril, hidratada, sin signos de irritación peritoneal, sin masas palpables y con ruidos hidroaéreos presentes. Destaca en exámenes de laboratorio: amilasa 390 UI/L, lipasa 1760.9 U/L.Se diagnostica pancreatitis aguda y por sospecha de etiología biliar se realiza colangiopancreatografia por resonancia magnética (CPRM). Como hallazgo se describe formación quística de 13mm a nivel de la transición entre cuerpo y cola del páncreas, compatible con NMIP de rama secundaria y se diagnostica pancreatitis aguda leve Balthazar B de etiología litiásica. Se realiza manejo de la pancreatitis y debido a las características del NMIP encontrado el plan es seguimiento tomográfico en 2 a 3 años. Discusión: Las NMIP son hallazgos imagenológicos frecuentemente incidentales, pues la mayoría de los pacientes se mantienen asintomáticos. La característica imagenológica habitual corresponde a una lesión quística multilocular lobulada situada en el proceso unciforme y en contigüidad con el conducto pancreático principal dilatado. Según las características de la lesión, el manejo puede ser quirúrgico o seguimiento. Las tasas de supervivencia global a 5 años se acercan a 61-87%

Introduction: Pancreatic intraductal papillary mucinous neoplasm (IPMN) is a cystic mass dependent on the pancreatic ductal system. Case report: Female, 65 years old, with morbid history of type 2 diabetes without treatment and essential hypertension under treatment. Consults by clinical profile of 9 hours characterized by oppressive/ burning epigastralgia of sudden onset, with irradiation in sash to back, intensity EVA 10/10, associated with nausea, without vomiting, diarrhea or fever. Is hemodynamically stable, afebrile, hydrated, with no signs of peritoneal irritation, with no palpable masses and with bowel sounds present. Highlights in laboratory tests: amylase 390 UI / L, lipase 1760.9 U / L. Acute pancreatitis is diagnosed and due the suspicion of biliary etiology a magnetic resonance cholangiopancreatography is performed. A cystic formation of 13mm is described at the transition level between body and tail of the pancreas, compatible with branch duct type IPMN and acute mild pancreatitis Balthazar B of lithiasic etiology is diagnosed. Management of pancreatitis is performed and because of the characteristics of the IPMN found the plan is tomographic follow-up in 2 to 3 years. Discussion: IPMN are frequently incidental imaging findings, as most patients remain asymptomatic. The usual imaging characteristic corresponds to a lobulated multilocular cystic lesion located in the unicular process and in contiguity with the main dilated pancreatic duct. Depending on the characteristics of the lesion, the management may be surgical or follow-up. The 5-year survival rates approach 61-87%.

Ultrasonido endoscópico en patologías del páncreas / Endoscopic ultrasound in pancreatic diseases

El ultrasonido endoscópico (EUS) ha revolucionado el diagnóstico y el manejo de muchas patologías de la vía digestiva, particularmente la patología pancreática, convirtiéndose en un examen prácticamente imprescindible en el abordaje diagnóstico y terapéutico de un paciente con un problema de páncreas. Es necesario dejar en claro que el método no es único y que para lograr una sensibilidad alta y cumplir el objetivo de realizar lo más adecuado, debe sumarse a otros métodos de acuerdo a cada caso, como la ecografía, la tomografía axial computarizada (TAC), la resonancia magnética (MRI) en sus diferentes modalidades, y las pruebas del laboratorio clínico microbiológico y patología. En este artículo se revisarán algunos casos de enfermedades evaluadas con este método, que muestran por qué el EUS, es una herramienta clave para el médico de urgencias y de consulta externa, el internista, el cirujano, el médico del servicio hospitalario y el personal de salud en general, al momento de definir, clasificar y orientar el manejo de determinadas patologías en el tubo digestivo. El EUS es una importante ayuda y no debe ser extraña al personal médico, debe tenerla presente junto a las demás pruebas diagnósticas en patología pancreática. Se señalarán los aspectos más relevantes en cada caso y las indicaciones del EUS.

Endoscopic ultrasound (EUS) has revolutionized the diagnosis and management of many diseases of the digestive tract, particularly the pancreatic ones, becoming a practically essential test in the diagnosis and therapeutic management of a patient with a pancreatic problem. It's necessary to establish the final diagnosis are necesary many tests to achive high sensitivity. It should join with other methods according to each case, such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) in its various forms, and chemical, microbiological and pathology tests. In this article we reviewed some cases of pathologies evaluated by this diagnosis test, which demonstrate why the EUS, is a key for the emergency and outpatient physician, internist, surgeon, doctor of the hospital service and staff health in general, when defining, classifying and guide the management of certain diseases in the digestive tract. The EUS is an important tool and should not be foreign to the medical staff, who must consider it, with other diagnostic tests for pancreatic disease. This article point out the most important aspects in each case and indications of EUS.

Lesões Císticas do Pâncreas / Prancreatic cystic lesions

As lesões císticas do pâncreas são peculiares em relação ao diagnóstico e ao tratamento. O recurso da ecoendoscopia promoveu melhor processo na diferenciação entre as lesões com características benignas e as malignas. Trata-se de afecção de alta possibilidade de cura na ocasião do diagnóstico precoce, oferecendo-se tratamento efetivo e impactante. Didaticamente, podemos dividir em tipos de lesões macro e microcística: a primeira poderá apresentar-se com características de malignidade; já a segunda demonstra-se ser lesão benigna. As lesões císticas tipo IPMN estão detalhadas neste artigo e o tratamento cirúrgico é a opção justificada naqueles pacientes com lesões no ducto pancreático principal. Caso contrário, nos pacientes com IPMN dos ductos secundários, o acompanhamento poderá ser feito pela ecoendoscopia. Finalmente, as lesões císticas do pâncreas devem ser avaliadas por abordagem multidisciplinar, alcançando-se assim o melhor tratamento possível ao paciente.

The cystic pancreatic lesions have particularities regarding diagnostic and treatment ways. The resources on EUS raised up better process to be diferetiating those cases with benign and malignant features. It is a lesion with high probability to bring an early diagnosis and with an effective and prompt treatment. It will be didactly share the types of lesions in macro and microcystics, the first one could be raised up the features of malignancy, on the other hand the last one push up the benigns lesions. IPMN lesions are detailed in the manuscript and in those patients with main pancreatic duct lesions the surgical treatment is the rationale otpion. Otherwise, patients with side branch IPMN, the follow up could be taken by using EUS findings. Finally, pancreatic cystic lesions should be assessed by multidisciplinary approach intending the best treatment chance.

Neoplasia mucinosa papilar intraductal (NMPI) / Intraductal papillary mucinous neoplasm (IPMN)

A neoplasia mucinosa papilar intraductal (IPMN) é um tumor cístico do pâncreas. Os primeiros relatos de IPMN datam do início da década de 80. Sua incidência ainda é desconhecida, porém a prevalência dessas neoplasias em um estudo realizado com a população de Minessota nos Estados Unidos, mostrou 2.04 casos para 100.000 hab4. A etiologia permanece obscura, mas a evidência crescente sugere o envolvimento de várias vias responsáveis pelo aparecimento da neoplasia, incluindo uma associação com síndromes hereditárias. IPMN ocorre mais comumente nos homens, com idade média ao diagnóstico entre 64 e 67 anos. No momento do diagnóstico, podem ainda ser benignos, com ou sem displasia ou francamente malignos, se apresentando já como um carcinoma invasivo. Em geral, os pequenos IPMNs localizados nos ramos secundários são benignos, particularmente em doentes assintomáticos, nesses casos podem ser submetidos a tratamentos conservadores. Em contraste, os tumores do ducto principal devem ser ressecados e cuidadosamente examinados à procura de um componente invasivo. Na ausência de invasão, a sobrevida é excelente, 94-100%. Para os doentes com carcinoma invasivo IPMN-associado, o prognóstico é melhor do que aqueles com um adenocarcinoma ductal isolado, com uma sobrevivência de 5 anos de 40% a 60% em algumas séries. No entanto, nenhuma vantagem de sobrevivência pôde ser demonstrada em relação aos tipos histológicos (tubular convencional versus mucinosos). Diversas variantes histomorfológicas são reconhecidas, embora o significado clínico desses subtipos não está bem definido.