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Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.

Should rare subtypes of pancreatic cancer be treated as its most common variant (ductal adenocarcinoma)? The most common type of pancreatic cancer is ductal adenocarcinoma. While much attention has been given to the molecular aspects and treatment aspects of this disease, rare variants of pancreatic cancer have been underappreciated. Some of them present unique molecular features that suggest different treatment approaches could lead to better outcomes. In this review, we summarize information on the clinical, pathological, and molecular features of rare subtypes of pancreatic cancer, along with subtype-specific data on treatment.

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Introduction: Imaging diagnosis of pancreatic solid-pseudopapillary neoplasms (SPNs) is difficult. Preoperative diagnosis by endosonography-guided fine-needle aspiration (EUS-FNA) is possible and has been reported in the literature in pancreatic tumors. However, its usefulness is still controversial. The aim of this study was to determine the accuracy of the EUS-FNA in the diagnosis of patients with SPN and describe the findings in computerized tomography (CT), magnetic resonance cholangiopancreatography imaging (MRI/MRCP), and EUS therefore comparing the imaging methods alone to the findings of microhistology (McH) obtained by EUS-FNA. Materials and Methods: We retrospectively reviewed the medical records of patients undergoing EUS-FNA with suspected SPN in imaging studies in 5 Brazilian high-volume hospitals (two university hospitals and three private hospitals). The demographic data; findings in CT, MRI/MRCP, and EUS; and McH results obtained by EUS-FNA were noted prospectively. The final diagnosis was obtained after the anatomopathological examination of the surgical specimen in all patients (gold standard), and we compared the results of CT, MRI/MRCP, EUS, and the McH with the gold standard. Results: Fifty-four patients were included in the study, of which 49 (90.7%) were women with an average age of 33.4 (range 11-78) years. The most common symptom presented was abdominal pain, present in 35.2% patients. SPN was detected incidentally in 32 (59%) patients. The average size of the tumors was 3.8 cm (SD: 2.26). The most common finding at EUS was a solid, solid/cystic, and cystic lesion in 52.9%, 41.1%, and 7.8% patients, respectively. The final diagnosis was 51 patients with SPN and 3 with nonfunctioning pancreatic neuroendocrine tumors (NF-NET). The correct diagnosis was made by CT, MRI/MRCP, EUS isolated, and EUS-FNA in 21.9%, 28.88%, 64.71%, and 88.24%, respectively. EUS-FNA associated with CT and MRI increased diagnostic performance from 22.72% to 94.11% and from 29.16% to 94.11%, respectively. Conclusions: SPN are rare, incidentally identified in most cases, and affect young women. Differential diagnosis between SPN, NF-NET, and other types of tumors with imaging tests can be difficult. EUS-FNA increases preoperative diagnosis in case of diagnostic doubt and should be used whenever necessary to rule out NF-NET or other type of solid/cystic nodular lesion of the pancreas.

Introdução: O diagnóstico por imagem da neoplasia pseudopapilar sólida do pâncreas (NPS) é difícil. O diagnóstico pré-operatório obtido pela endosonografia com punção aspirativa por agulha fina (USE-PAF) é possível e tem sido relatado na literatura em tumores do pâncreas. No entanto, sua indicação é controversa e merece discussão. O objetivo do estudo foi determinar a acurácia da USE-PAF no diagnóstico de pacientes com NPS, descrever os achados da tomografia computadorizada (TC), colangiopancreatografia por ressonância magnética (RM/ CPRM) e USE, comparando os métodos de imagem isolados aos achados da microhistologia (McH) obtida pela USE-PAF. Material e Métodos: Revisamos retrospectivamente os prontuários de pacientes submetidos à USE-PAF com suspeita de NPS em exames de imagem de 5 hospitais brasileiros de alto volume (dois universitários e três privados). Foram anotados prospectivamente os dados demográficos, os achados da TC, RM/CPRM e USE e o resultado da McH obtida pela USE-PAF. O diagnóstico final foi obtido após o anatomopatológico da peça operatória em todos os pacientes (padrão-ouro). Comparamos os resultados da TC, RM/CPRM, EUS isoladas e da McH obtida pela USE-PAF com o padrão-ouro. Resultados: Cinquenta e quatro pacientes foram incluídos no estudo, 49 (90.7%) eram mulheres com média de idade de 33.4 (11­78) anos. O sintoma mais frequente foi dor abdominal, presente em 35.2%. A NPS foi detectada acidentalmente em 32 (59%) pacientes. O tamanho médio da lesão foi de 3.8 cm (SD: 2.26). O achado mais comum à USE foi lesão sólida, sólida/ cística e cística em 52.9%, 41.1% e 7.8%, respectivamente. O diagnóstico final foi NPS (51) e tumor neuroendócrino pancreático não funcionante [NF-NET] (3). O diagnóstico correto feito pela TC, RM, USE e USE-PAF foi feito em 21.9%, 28.9%, 64.7% e 88.2%, respectivamente. A USEPAF associada a TC e a RM aumentou o desempenho diagnóstico de 21.9% para 94.1% e de 28.8% para 94.1%, respectivamente. Conclusões: NPS são raras, identificadas de forma acidental na maioria dos casos e afetam principalmente mulheres jovens. O diagnóstico diferencial entre NPS, NF-NET e outros tipos de lesões com exames de imagem isolados pode ser difícil. A USE-PAF aumenta a chance do diagnóstico pré-operatório em caso de dúvida diagnóstica e deve ser usado sempre que necessário para descartar NF-NET ou outro tipo de lesão nodular sólida ou sólido/cística do pâncreas.

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Objetivo: Comunicar el caso de un varón diagnosticado de tumor sólido pseudopapilar de páncreas (TSPP). Esta patología afecta fundamentalmente a mujeres jóvenes, siendo extremadamente infrecuente en hombres. Caso Clínico: Varón de 40 años diagnosticado por TC de masa intraabdominal sólida bien delimitada, de 12,5 × 13 × 8,5 cm, heterogénea, con calcificaciones periféricas y realce tras la administración de contraste en fase portal, dependiente de cuerpo-cola pancreática. Se realiza puncion, no concluyente. La laparotomía exploradora mostró tumoración sólida dependiente de cola pancreática que incluye vasos esplénicos. Se liberó el tumor de forma periférica, requiriendo pancreatectomía distal y esplenectomía. El resultado AP informó neoplasia pseudopapilar sólida de cola pancreática de bajo grado de malignidad, bien diferenciado (G1). Alta hospitalaria al 12° día sin complicaciones. Actualmente libre de enfermedad tras dos años de la intervención. Discusión: Este tumor infrecuente representa el 1%-3% de las neoplasias pancreáticas. El 90% afecta a mujeres jóvenes, con proporción de mujeres a hombres de 4:1. En estos, tiene mayor potencial maligno con peor pronóstico. Conclusión: La baja incidencia en varones puede dificultar el diagnóstico. Sin embargo, debemos tenerlo en cuenta en el diagnóstico diferencial, ya que el tratamiento quirúrgico radical aumenta significativamente la supervivencia al evitar la recurrencia local y las metástasis a distancia, lo que supone un reto quirúrgico.

Objective: To report the case of a male diagnosed with a solid pseudopapillary tumor of the páncreas. This pathology fundamentally affects young women, being extremely infrequent in men. Clinical Case: A 40-year-old man diagnosed by CT with a well-defined solid intra-abdominal mass, 12.5 × 13 × 8.5 cm, heterogeneous, with peripheral calcifications and enhancement after contrast administration in the portal phase, dependent on the body-tail of the pancreas. Fine needle puncture is performed, inconclusive. The exploratory laparotomy showed a solid tumor dependent on the pancreatic tail that included splenic vessels. The tumor was released peripherally, requiring distal pancreatectomy and splenectomy. The AP result reported solid pseudopapillary neoplasm of the pancreatic tail of low grade of malignancy, well differentiated (G1). Hospital discharge on the 12th day without complications. Currently free of disease two years after the intervention. Discussion: This rare tumor represents 2%-3% of pancreatic neoplasms. 90% affects young women, with a ratio of women to men of 4:1. In these, it has greater malignant potential with worse prognosis. Conclusion: The low incidence in males can make diagnosis difficult. However, we must take it into account in the differential diagnosis, since radical surgical treatment significantly increases survival by avoiding local recurrence and distant metastases, which is a surgical challenge.

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RESUMEN El tumor sólido pseudopapilar de páncreas (TSP) es un tumor infrecuente, de bajo grado de malignidad, que representa el 1-3% de todas las neoplasias pancreáticas, con predilección por el sexo femenino, y es el tumor sólido de páncreas más frecuente en la infancia. Mujer de 13 años que consultó por dolor abdominal de 5 días de evolución, en hipocondrio derecho e irradiado a espalda, con náuseas y vómitos. TSP es diagnosticado con ecografía, tomografía computarizada (TC) y resonancia magnética (RM). Se realizó duodenopancreatectomía cefálica. La paciente fue dada de alta. El examen anatomopatológico informó un TSP de páncreas. El abordaje quirúrgico de estos tumores, abierto o laparoscópico, permite una excelente supervivencia alejada.

ABSTRACT Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm with a low malignant potential and represents 1- 3% of all pancreatic tumors. They usually occur in women and are the most common solid pancreatic tumor in children. A 13-year-old girl visited the emergency department due to abdominal pain in the right hypochondrium radiating to the back, nausea and vomiting lasting 5 days. A SPT was diagnosed by ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). The patient underwent cephalic pancreaticoduodenectomy and was discharged. The pathological study reported a SPT of the pancreas. The surgical approach of these tumors through open surgery or laparoscopy offers excellent long-term survival.

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Resumen Introducción: las neoplasias sólidas pseudopapilares de páncreas representan alrededor del 3% de los tumores pancreáticos quísticos resecados. Se presentan con mayor frecuencia en mujeres jóvenes entre la segunda y tercera décadas de la vida. Es un tumor con poco potencial maligno que se ubica principalmente en la cola; sin embargo, puede encontrarse en cualquier localización anatómica pancreática. Métodos: se presentan 5 casos de cuatro mujeres y un hombre, entre 16 y 36 años, que consultaron principalmente por dolor abdominal. Resultados: cuatro pacientes fueron llevados a pancreatectomía distal sin esplenectomía por laparoscopia, y una paciente a pancreatoduodenectomía por laparotomía. Se logró extraer por completo y satisfactoriamente el tumor a los cinco pacientes. No se evidenció metástasis. Los tumores se localizaron en la cabeza (1), cuello (1) y predominantemente en cuerpo y cola (3). El reporte histopatológico posoperatorio confirmó el diagnóstico en los cinco casos, en la paciente más joven se había realizado una biopsia percutánea previa a la intervención quirúrgica. Conclusiones: la resección quirúrgica completa del tumor con preservación del bazo es el tratamiento de elección en pacientes con neoplasia sólida pseudopapilar de páncreas.

Abstract Introduction: Solid pseudopapillary neoplasms of the pancreas represent around 3% of resected cystic pancreatic tumors. They occur more frequently in young women between the second and third decades of life. It is a tumor with little malignant potential located mainly in the tail; however, it can be found in any pancreatic anatomical location. Materials and methods: We reported five cases of four women and one man, between 16 and 36 years of age, who consulted mainly for abdominal pain. Results: Four patients underwent distal pancreatectomy without laparoscopic splenectomy, and one underwent laparotomy pancreatoduodenectomy. The tumor was completely and satisfactorily removed from all five patients. No metastasis was found. The tumors were located on the head (1), neck (1), and predominantly on the body and tail (3). The postoperative histopathological report confirmed the diagnosis in all five cases; in the youngest patient, a percutaneous biopsy had been performed before surgical intervention. Conclusions: Complete surgical resection of the tumor with preservation of the spleen is the treatment of choice in patients with solid pseudopapillary neoplasm of the pancreas.

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Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.

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Solid pseudopapillary neoplasms (SPN) are rare and represent a minority of all pancreatic cystic tumors. Symptoms, if present, are generally nonspecific and upper gastrointestinal bleeding is extremely uncommon as an initial presentation. A 31-year-old woman with no prior medical history presented with a 3-week period of epigastric pain, which she reported as persistent, exacerbated by eating, and associated with progressive asthenia, fatigue, and exertional dyspnea. One month prior, she had had experienced 2 episodes of melena. Upon physical examination her skin was pale, with no other changes. Through diagnostic investigation, an esophagogastroduodenoscopy showed a giant ulcer in the duodenal bulb and a suspect fistulous orifice localized on the posterior wall of the duodenal bulb. In addition, a bulge on the duodenal bulb, suggestive of an extrinsic compression, was noticed. An abdominal computed tomography scan and magnetic resonance imaging showed a mixed solid and cystic lesion in the head of the pancreas in direct proximity to the duodenum, which contained an image compatible with a fistula. The patient successfully underwent pancreaticoduodenectomy. The histopathology, including microscopic analysis and immunohistochemistry, was consistent with an SPN of the pancreas. This case emphasizes that the evaluation of patients presenting with upper gastrointestinal bleeding due to a giant duodenal ulcer and an extrinsic mass effect noted on the duodenum should include cross-sectional images of the abdomen. In this case, the finding of a large well-encapsulated pancreatic solid and cystic mass on abdominal images was suggestive of a pancreatic neoplasm diagnosis, including an SPN.

Neoplasias sólidas pseudopapilares (NSP) são raras e representam uma minoria de todos os tumores císticos do pâncreas. Os sintomas, se presentes, geralmente são inespecíficos e a hemorragia gastrointestinal superior é extremamente incomum como apresentação inicial. Uma mulher de 31 anos sem história médica prévia apresentava a três semanas dor epigástrica, a qual ela relatava como persistente, exacerbada pela alimentação e acompanhada de astenia progressiva, fadiga e dispneia aos esforços. Além disso, um mês antes, ela teve dois episódios de melenas. Ao exame físico havia palidez cutânea, sem outras alterações. Através da investigação diagnóstica, uma esofagogastroduodenoscopia mostrou uma úlcera gigante no bulbo duodenal e a suspeita de um orifício fistuloso localizado na parede posterior do bulbo duodenal. Adicionalmente, um abaulamento sobre o bulbo duodenal, sugestivo de uma compressão extrínsica, foi notado. A tomografia computadorizada de abdome e a imagem de ressonância magnética mostraram uma lesão mista de aparência sólida e cística na cabeça do pâncreas, em proximidade direta com o duodeno, a qual continha uma imagem compatível com uma fístula. A paciente foi submetida a pancreaticoduodenectomia com sucesso. A histopatologia, incluindo análise microscópica e imunohistoquímica, foi compatível com NSP do pâncreas. Este caso enfatiza que a avaliação dos pacientes, que apresentam hemorragia gastrointestinal superior devido à úlcera duodenal gigante e um efeito de massa extrínseco notado sobre o duodeno, deve incluir imagem abdominal. Neste caso, o achado de uma grande massa pancreática sólida e cística bem encapsulada na imagem abdominal, sugeria o diagnóstico de uma neoplasia pancreática, incluindo a NSP.

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Resumen Introducción: el tumor sólido pseudopapilar corresponde al 1 %-2 % de todos los tumores sólidos del páncreas, con un curso poco agresivo. Serie de casos: en el departamento de patología de la Universidad de Antioquia se diagnosticaron 16 casos entre enero de 2004 y julio de 2019. Dos de los casos correspondían a pacientes pediátricos, uno de ellos de sexo masculino con posterior metástasis hepática, dos a mujeres mayores de 40 años y el resto a mujeres entre 17 y 26 años. Un caso presenta células de aspecto sarcomatoide, otros dos presentaron células gigantes multinucleadas y hubo un caso con pleomorfismo grave y presencia de mitosis atípicas. Se observó invasión de la cápsula en 6 de los casos, uno de ellos con invasión linfovascular y 3 casos con invasión perineural. Todos los casos mostraron hemorragia o necrosis, y presentaron en el perfil de inmunohistoquímica positividad para β-catenina, CD10, receptores de progesterona y CD56. La sinaptofisina fue positiva focalmente.

Abstract Introduction: Solid-pseudopapillary tumors represent 1 %-2 % of all the pancreatic solid tumors, with low malignant potential. Series of cases: In the department of pathology at the Universidad de Antioquia were diagnosed sixteen cases between January 2004 and July 2019. Two of those cases were pediatric patients, one male with subsequent hepatic metastasis. Two additional cases were represented by two females older than 40 years old and the rest of the cases were females between 17 and 26 years old. One of the cases had sarcomatoid aspect cells, two others revealed multinucleate giant cells, and one last case of severe pleomorphism and presence of atypical mitoses. Capsule invasion was observed in 6 cases: one of the cases with lymphovascular invasion and the other 3 cases with perineural invasion. All cases showed hemorrhage or necrosis, and the immunohistochemical profile was positive for β-catenin, CD10, progesterone receptors, and CD56. Synaptophysin was focally positive.

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INTRODUCTION: Frantz tumor or solid pseudopapillary neoplasm is a very rare tumor with low malignant potential, it constitutes 1-2 % of exocrine tumors of the pancreas. DESCRIPTION OF THE CASE: We present the case of a 58-year-old female patient with a 4-month history of occasional abdominal pain in the epigastrium, the tomography detected a distal tumor of the pancreas. Is taken to distal pancreatectomy. DISCUSSION: It was first described by Franz in 1959 as a papillary tumor of the pancreas, in 2010 it was reclassified as a solid pseudopapillary tumor of low grade of malignancy. They appear in 8-16.6 % in patients before 13 years of age, in young women they present between the second and fourth decade of life. The treatment for excellence is surgical resection. CONCLUSION: Pancreatic tumors represent a surgical challenge in any place of presentation.

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BACKGROUND: Solid pseudopapillary neoplasms (SPN) of the pancreas represents approximately 2% of non-endocrine tumors of the pancreas. It is described in the literature as a rare and predominant tumor in young women. AIM: To report a case series with SPN and analyzing clinical, surgical, anatomopathological characteristics, as well as the prognosis and review of literature. METHODS: Retrospective analysis of patients undergoing surgery, with histological diagnosis of SPN between 1998 and 2018, using standardized and prospectively completed forms, performed at the Surgery Service of the Upper Digestive System at Hospital São Rafael/Rede D'Or in Salvador - BA. Review of literature through a database search in MEDLINE/PubMed of retrospective articles. RESULTS: Fourteen female patients with the average age of 31.6 years (range min-max) were selected. Twelve patients (85.7%) were asymptomatic, being an incidental diagnosis or due to screening for other reasons. One patient had abdominal pain due to gastric compression and another patient had jaundice. The 14 patients were staged with computerized tomography or magnetic resonance imaging. None had evidence of metastasis. In 8 patients (57.1%), the tumor was in the tail and body. The average size was 6.7 cm (range min-18). The type of surgery was according to the anatomical location of the tumor. There was no lymph node involvement. In two cases, vascular resection with the use of a prosthesis was required for reconstruction. The surgical margins were free. In all cases, postoperative immunohistochemistry confirmed that it was a solid pseudo-papillary neoplasia of the pancreas. There has been no disease recurrence in any case so far. CONCLUSION: The tumors had a benign, indolent and histopathological behavior compatible with the literature. Curative surgery is recommended in all cases.

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El tumor sólido pseudopapilar del páncreas, conocido también como tumor de Frantz, es una enfermedad rara: neoplasia bien delimitada, de lento crecimiento, no agresiva pero maligna, habitualmente con pronóstico favorable. El tratamiento de elección es quirúrgico. Aunque algunos de ellos son agresivos a nivel local, la mayoría de los pacientes se curan con la resección completa del tumor. Se reportó el caso de una mujer de 30 años, ingresada en el Servicio de Cirugía General del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, con diagnóstico presuntivo clínico e imagenológico, de tumor pseudopapilar sólido del páncreas, con confirmación histológica tras la resección quirúrgica. Este infrecuente tumor debe ser considerado en el diagnóstico diferencial de los tumores pancreáticos, fundamentalmente en mujeres jóvenes(AU)

The solid pseudo-papillary carcinoma, also known as Frantz´s tumor, is a rare disease. It is a well-defined neoplasia, of low growth, non-aggressive but malignant, usually with a favorable prognosis. The elective treatment is the surgery. Although some of them are locally aggressive, most patients are healed with the complete tumor resection. The authors reported the case of a woman, aged 30 years who entered the Service of General Surgery of the University Hospital Comandante Faustino Pérez Hernández, with a presumptive clinical and imaging diagnosis of pancreas solid pseudo-papillary tumor, histologically confirmed after surgical resection. This infrequent tumor should be taken into account in the differential diagnosis of pancreatic tumors, mainly in young women(AU)

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Solid pseudopapillary tumor of the pancreas is a rare entity, more frequent in women between the 2nd and 4th decades. The diagnosis is usually incidental and it can be reached by computed tomography or magnetic resonance imaging. Subsequent pathological confirmation is necessary for an adequate treatment. A retrospective study of six cases was carried out. All the patients were female, between 14 and 56 years of age, in which 50% the tumor were an incidental finding. We had three cases located in the head and three in the body of the pancreas. We performed three pancreaticoduodenectomies and three distal pancreatectomies with splenic preservation, without disease recurrence.

El tumor sólido-quístico de páncreas es poco frecuente y predomina en mujeres entre la segunda y la cuarta décadas de la vida. Los pacientes son generalmente asintomáticos. El diagnóstico se realiza por imágenes con tomografía o resonancia magnética, y con la posterior confirmación patológica para poder ofrecer un tratamiento adecuado. Presentamos una serie de seis casos. Todas las pacientes fueron de sexo femenino, de entre 14 y 56 años. El 50% fueron un hallazgo incidental. Tuvimos tres casos localizados en la cabeza y tres en el cuerpo del páncreas. Se realizaron tres duodenopancreatectomías cefálicas y tres pancreatectomías distales con preservación esplénica, con buena evolución y sin recidiva.

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INTRODUCTION: Preoperative diagnostic imaging of pancreatic solid pseudopapillary neoplasms (SPNs) is challenging. A few studies have investigated the role of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) for the diagnosis of SPN. We investigated the diagnostic yield of cell-blocks and immunohistochemistry (IHC) for SPN using EUS-FNA specimens without cytological evaluation. PATIENTS AND METHODS: We retrospectively analysed the histopathology records of patients with suspected SPN, who underwent EUS-FNA biopsy between January 1997 and January 2020. Diagnosis based on cell-blocks (haematoxylin-eosin staining with complementary IHC) was compared with the definitive surgical diagnosis. RESULTS: This study included 25 patients (24 were women). Patients' mean age was 33.7 years (range 12-78 years). The most common symptom was abdominal pain. SPN was an incidental finding in 52% of the patients. The mean lesion size was 4.3 cm (range 1.2-11.4 cm), and the most common endosonographic features included solid-cystic (56%) or solid (40%) tumours. Final diagnoses included SPNs (n = 23) and non-functioning neuroendocrine tumours (n = 2). The overall accuracy of EUS-FNA was 80%. Tumour cells showed immunopositivity for ß-catenin, CD10, CD99 and progesterone receptor (PR) in 93.7%, 87.5%, 83.3% and 66.6% of patients, respectively. No SPN showed immunopositivity for chromogranin A. CONCLUSIONS: Intention-to-diagnose analysis showed that the diagnostic accuracy of EUS-FNA for SPNs using cell blocks and complementary IHC without cytological evaluation was fairly good. Evaluation of ß-catenin, CD 10, CD99 and PR expression must be included in the IHC panel for diagnostic confirmation of SPNs using EUS-FNA biopsy specimens.

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PURPOSE: Solid pseudopapillary neoplasm (SPN) is an uncommon pathology with a low-grade malignancy. Surgery is the milestone treatment. Nevertheless, despite appropriate management, some patients present recurrence. Risk factors associated with recurrence are unclear. The objective was to identify the clinicopathological factors associated with recurrence in patients with SPN treated with pancreatic resection. METHODS: Medical records of patients treated with pancreatic resection during 2006-2020 were evaluated. Patients with histological diagnosis of SPN were included. Survival analysis was performed to identify the clinicopathological factors related to recurrence. RESULTS: Seventy-four patients were diagnosed with SPN; 70 (94.6%) patients were female, and the median age was 20 years old. The median tumor diameter was 7.9 cm. Multivisceral resection was performed in 9 (12.2%) patients. Four (5.4%) patients presented lymph node metastasis.R0 resection was achieved in all cases. Six (8%) patients presented recurrence and the liver was the most frequent recurrence site (n = 5).After a median follow-up of 40.2 months, 9 (12%) patients died. Five (6.8%) patients died of disease progression. The 1-3- and 5-year overall survival (OS) was 97.1%, 90.2% and 79.9%, respectively. The 1-3-and-5-year recurrence-free survival (RFS) was 98.4%, 89.9% and 87%, respectively. In the univariate Cox-regression analysis, age ≥ 28 years(HR = 8.61, 95% CI 1.1-73.8),tumor diameter ≥ 10 cm(HR = 9.3, 95% CI 1.12-79.6),invasion of adjacent organs (HR = 7.45, 95% CI 1.5-36.9), lymph node metastasis (pN +) (HR = 16.8, 95% CI 2.96-94.9) and, AJCC Stage III (HR = 10.1, 95% CI 1.2-90.9) were identified as predictors for recurrence. CONCLUSIONS: SPN is more frequently diagnosed in young women with a good overall prognosis after an R0 surgical resection even with disease recurrence. Age ≥ 28 years, larger tumors ≥ 10 cm, invasion of adjacent organs, lymph node metastasis(pN +) and, AJCC Stage III were predictors factors of recurrence in resected SPN.

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El tumor sólido pseudopapilar de páncreas tiene una incidencia de 0,13-2,7%, afectando preferentemente a mujeres jóvenes. Presentamos la experiencia en el manejo de tumores sólidos pseudopapilares de páncreas durante 10 años en el Hospital Coromoto de Maracaibo. Métodos: Estudio retrospectivo realizado en el Hospital Coromoto de Maracaibo desde enero 2010 hasta diciembre 2019. Se analizaron las variables edad, sexo, ubicación del tumor, procedimiento quirúrgico, tamaño del tumor, tiempo quirúrgico, tiempo de hospitalización y complicaciones. Resultados: De 183 casos totales de cirugía pancreática,15 casos presentaron diagnóstico anatomopatológico de tumor sólido pseudopapilar, representando un 8,20% del total de las cirugías pancreáticas; con edades comprendidas entre 15 a 56 años, con una media de 27,93 de los cuales el 93,33% fueron del género femenino. La ubicación más frecuente fue el cuerpo y la cola del páncreas con un 53,33%. El procedimiento quirúrgico más empleado fue la pancreatectomía distal en 8 casos. El tamaño promedio de los tumores fue de 6,47cm y el tiempo quirúrgico empleado fue de 254 minutos para las pancreatectomías distales y 412 minutos para los procedimientos de Whipple, con una media de hospitalización de 4,6 y 7,2 días respectivamente. Presentaron complicaciones 2 pacientes y sin decesos. Conclusiones: El tumor sólido pseudopapilar es una neoplasia pancreática de comportamiento incierto, con una baja incidencia entre todos los tumores de páncreas, donde la laparoscopia es un método de abordaje seguro en las pancreatectomías distales, sin diferencias en estancia hospitalaria y tiempo quirúrgico. La resección quirúrgica con márgenes libres es curativa(AU)

The solid pseudopapillary tumor of the pancreas has an incidence of 0.13-2.7%, preferentially affecting young women. We present the experience in the management of solid pseudopapillary tumors of the pancreas for 10 years at the Coromoto Hospital in Maracaibo. Methods: Retrospective study carried out at the Coromoto Hospital in Maracaibo from January 2010 to December 2019. The variables age, sex, tumor location, surgical procedure, tumor size, surgical time, hospitalization time and complications were analyzed. Results: Of 183 total cases of pancreatic surgery, 15 cases had a pathological diagnosis of a solid pseudopapillary tumor, representing 8.20% of the total of pancreatic surgeries; with ages ranging from 15 to 56 years, with an average of 27.93 of which 93.33% were female. The most frequent location was the body and tail of the pancreas with 53.33%. The most used surgical procedure was distal pancreatectomy in 8 cases. The average size of the tumors was 6.47cm and the surgical time used was 254 minutes for distal pancreatectomies and 412 minutes for Whipple procedures, with a mean hospital stay of 4.6 and 7.2 days, respectively. Two patients presented complications and no deaths. Conclusions: The solid pseudopapillary tumor is a pancreatic neoplasm of uncertain behavior, with a low incidence among all pancreatic tumors, where laparoscopy is a safe approach to distal pancreatectomies, without differences in hospital stay and surgical time. Surgical resection with free margins is curative(AU)

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Los tumores pseudopapilares del páncreas son tumores de origen pancreático poco frecuentes y de etiología desconocida. Comprenden entre el 0.2 y 2.7% de los carcinomas de páncreas. Hasta 2015 hay 900 casos reportados en la literatura, siendo una minoría en etapa diseminada. Son tumores voluminosos, de bajo potencial maligno, que se presentan con mayor frecuencia en mujeres jóvenes entre 18 y 35 años. Generalmente son asintomáticos o manifiestan clínicamente síntomas inespecíficos como dolor abdominal o presencia de masa abdominal. Anatómicamente se localizan con mayor frecuencia en la cola del páncreas, seguidos por la cabeza y el cuerpo. El tratamiento de elección es la resección quirúrgica. El rol de la quimioterapia en la enfermedad irresecable o avanzada no está claramente definido. Son tumores de excelente pronóstico, con sobrevida a 5 años de casi 100%.Se presentan cuatro casos clínicos y se hace una revisión de la literatura.

Pseudopapillary tumors of the pancreas are tumors of pancreatic origin with a low frequency and an unknown etiology. They account for 0.2 - 2.7 % of all pancreatic carcinomas. Up to 2015 there were approximately 900 well documented cases with only a small minority of them in a metastatic stage. This tumors could reach large proportions and they occur predominantly in young women between 18 and 35 years of age. Most of patients are asymptomatic or have non specific symptoms including abdominal pain or palpable abdominal mass. The most common localization is the tail of the pancreas, followed by the head and the body. Complete resection is the treatment of choice. It is not clearly stablished the rol of chemotherapy in metastatic disease. There are tumors with a favorable prognosis, with an overall 5 year survival rate about 95%. Herein, we report four clinical cases and a literatura review.

Os tumores pseudopapilares do pâncreas são tumores de origem pancreática pouco frequentes e de etiologia desconhecida. Compreendem entre 0.2 e 2.7% dos carcinomas de pâncreas. Até 2015 há 900 casos relatados na literatura, sendo uma minoria em etapa disseminada. São tumores volumosos, de baixo potencial maligno, que se apresentam com maior frequência em mulheres jovens entre 18 e 35 anos. Geralmente são assintomáticos ou apresentam clinicamente sintomas inespecíficos como dor abdominal ou presença de massa abdominal. Anatomicamente, localizam-se mais frequentemente na cauda do pâncreas, seguidos por cabeça e corpo. O tratamento de escolha é a ressecção cirúrgica. O papel da quimioterapia na doença irressecável ou avançada não está claramente definido. São tumores de excelente prognóstico, com sobrevida a 5 anos de quase 100%. Apresentam-se quatro casos clínicos e faz-se uma revisão da literatura.

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RESUMEN Antecedentes: la neoplasia sólida pseudopapilar del páncreas es una entidad rara, que típicamente se presenta en mujeres jóvenes. Suele presentar síntomas abdominales inespecíficos. Es un tumor maligno de bajo grado de malignidad. Objetivos : el objetivo del siguiente informe de serie de casos es presentar 9 casos tratados en un cen tro y realizar una revisión bibliográfica del tema. Material y métodos: estudio retrospectivo descriptivo de los casos con diagnóstico anatomopatoló gico de neoplasia sólida pseudopapilar en el Servicio de Cirugía General, desde febrero de 2013 hasta septiembre de 2019. Se contemplaron como variables: edad, sexo, localización del tumor, tratamiento quirúrgico realizado, tiempo operatorio, complicaciones, estancia hospitalaria y seguimiento alejado. Resultados: fueron 9 casos, todos de sexo femenino con media de edad de 30 años (rango 20 a 70 años). La localización más frecuente fue en cola de páncreas en 4 casos (45%). Todas las pacientes fueron sometidas a cirugía, con abordaje laparoscópico en el 60% de los casos (n = 5); la resección pancreática distal con preservación esplénica fue la conducta más utilizada (n = 6). Se constataron tres complicaciones, de las cuales dos fueron colecciones abdominales como consecuencia de una fístula pancreática que se abordaron por vía percutánea, y la restante fue un retardo del vaciamiento gástrico por lo cual la paciente requirió internación prolongada. Conclusión: la neoplasia sólida pseudopapilar pancreática es una enfermedad poco frecuente, ma ligna pero con bajo riesgo de malignidad. Presenta buena sobrevida cuando se somete a cirugía de carácter curativo; la laparoscopia es la vía de abordaje de elección en centros con experiencia.

ABSTRACT Background: Solid pseudopapillary tumor of the pancreas is a rare condition that affects young women. The most common symptom is unspecific abdominal pain. It is a malignant tumor of low malignant potential. Objective: The aim of this study is to report a case series of patients treated in a single center and perform a bibliographic review. Material and methods: We conducted a retrospective study of the cases with pathological diagnosis of solid pseudopapillary tumor of the pancreas treated in the Department of General Surgery between February 2013 and September 2019. The following variables were analyzed; age, sex, tumor location, surgical treatment, operative time, complications, length of hospital stay and long-term follow-up. Results: Nine patients were included; all of them were women with mean age of 30 years (range: 20 - 70 years). The most common location of the tumor was the tail of the pancreas (n = 4; 45%). Surgery was performed in all the cases; five cases underwent video-assisted laparoscopy and spleen-preserving distal pancreatectomy was the technique more commonly used (n = 6). Three complications were recorded: two abdominal collections due to biliary leaks were trated by percutaneous approach and the other patient presented delayed gastric emptying and required prolonged hospitalization. Conclusion: Solid pseudopapillary tumor pf the pancreas is a rare low-grade malignant neoplasm. The prognosis is favorable after surgery and laparoscopy is the preferred approach in centers with experience.

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Introducción: La neoplasia pseudopapilarsólida del páncreas es una patología poco frecuente, que se presenta en mujeres jóvenes y es de comportamiento incierto. La prevalencia es del 1-2% y el tratamiento es quirúrgico. En el presente caso se describe a una paciente de sexo femenino de 10 años de edad que muestra esta patología y su correspondiente manejo. Caso clínico:Paciente de sexo femenino, de 10 años de edad, con antecedentes de una nefrectomía hace 6 años por hidronefrosis, refiere dolor abdominal localizado en egigastrio tipo cólico y pérdida de peso. En los estudio de imagen a nivel peritoneal se observa lesión de aspecto ocupativo de tipo sólido neoplásico con área central de probable necrosis y que desplaza en sentido posterior al páncreas así como al estómago y se asocia con adenomegalias peritoneales y retroperitoneales. Evolución:Lapaciente fue sometido a una espleno-pancreatectomía distal.El estudio anatomo-patológico concluyó en neuplasia pseudopapilar sólida del páncreas.Continúa en valoración por consulta externa. Conclusión:Neoplasia Pseudopapilar sólida del Páncreasdebe ser sospechada en pacientes con una masa sólida o quística pancreática con un estudio de imágenes compatibles. Palabras clave:Neoplasia pseudopapilar sólida del páncreas, prevalencia, inmunohistoquímica, receptores progestágenos,Informe de caso

Introduction:Solid pseudopapillary neoplasia of the pancreas is a rare pathology that occurs in young women and has an uncertain behavior. The prevalence is 1-2% and the treatment is surgical. In the present case, a 10-year-old female patient with this pathology and its corresponding management is described. Clinical case: A 10-year-old femalepatient with a history of nephrectomy 6 years ago due to hydronephrosis, complains of colicky localized abdominal pain in the egigastrium and weight loss. In peritoneal imaging studies, a neoplastic solid type occupational lesion with a central area of probable necrosis is observed, which displaces the pancreas as well as the stomach posteriorly and is associated with peritoneal and retroperitoneal adenomegaly. Evolution: The patient underwent a distal spleno-pancreatectomy. The anatomopathological studyconcluded in solid pseudopapillary neuplasia of the pancreas. He continues to be evaluated by external consultation. Conclusion: Solid Pseudopapillary Neoplasia of the Pancreas should be suspected in patients with a solid or cystic pancreatic mass with a compatible imaging study. Key words:Solid pseudopapillary neoplasm of the pancreas, prevalence, immunohistochemistry, progestin receptors, Case Report.

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Resumen Introducción: El tumor sólido pseudopapilar del páncreas es una rara entidad que representa menos del 1% de las neoplasias pancreáticas. Suele presentarse en mujeres jóvenes y solo da síntomas de carácter compresivo una vez que alcanza un gran tamaño. Dado su comportamiento biológico incierto el tratamiento es la cirugía. Caso Clínico: Presentamos el caso de una mujer de 23 años con historia de 1 año de evolución de dolor epigástrico y baja de peso. El estudio imagenológico demostró una masa heterogénea sólida-quística dependiente de la cabeza del páncreas de aspecto neoplásico. Se realizó una biopsia incisional laparoscópica cuyo resultado fue de un tumor maligno indiferenciado, por lo que se optó por la resección quirúrgica. Se realizó una pancreatoduodenectomía abierta sin incidentes con un postoperatorio favorable. Los análisis histopatológicos e inmunohistoquímico fueron compatibles con un tumor sólido pseudopapilar de páncreas.

Introduction: The pseudopapillary solid tumor of the pancreas is a rare entity that represents less than 1% of pancreatic neoplasms. It usually occurs in young women and only gives symptoms of a compressive nature once it has reached a large size. Given its uncertain biological behavior, the treatment is surgery. Case Report: We present the case of a 23-year-old woman with a 1-year history of epigastric pain evolution and weight loss. The imaging study demonstrated a solid-cystic heterogeneous mass dependent on the head of the pancreas of neoplasic appearance. A laparoscopic incisional biopsy was performed, the result of which was an undifferentiated malignant tumor, which was why the surgical resection was chosen. An open pancreatoduodenectomy was performed without incident with a favorable post operative. Histopathological and immunohistochemical analyzes were compatible with a solid pseudopapillary tumor of the pancreas.

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