Tumor Sólido Pseudopapilar de Páncreas en varón

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.

Neoplasia pseudopapilar sólida del páncreas (tumor de Frantz) en una joven de 13 años / Solid pseudopapillary tumor of the pancreas (Frantz tumor) in a 13-year-old girl

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.

Solid Pseudopapillary Epithelial Neoplasm of the Pancreas: A Rare Entity with Diagnostic Dilemma

The solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is a relatively uncommon entity. The aim of the present study was to summarize our experiences with regard to diagnostic dilemma, surgery, postoperative follow-up, and management. This retrospective data were collected during the period from January 1, 2018 to December 31, 2020. A total of four patients (three females and one male) were identified within an age range of 13 to 25 years. All the patients were presented with nonspecific symptoms such as abdomen lumps, swelling in the abdomen, and abdominal pain. To reach a definite diagnosis, imaging studies were conducted along with endoscopic ultrasound fine-needle aspiration (EUS-FNA) and biopsy. After confirmation of SPEN on biopsy, all the patients underwent surgery without any complications. Patients are on follow-up, and to date, no metastasis has been detected. SPEN is a rare pancreatic tumor with unusual pathological features leading to a diagnostic dilemma. The pathologist should be familiar with SPEN and its salient histological characteristics that differentiate it from other look-alike pancreatic tumors and can help in timely surgery and management.

Clinical characteristics analysis of 253 cases of pancreatic solid pseudopapillary neoplasm / 中华消化杂志

Objective:To summarize the clinical characteristics of pancreatic solid pseudopapillary neoplasm (pSPN).Methods:From March 2007 to August 2022, at the First Affiliated Hospital of Zhejiang University School of Medicine, the clinical data and follow-up results of 253 patients with pSPN and underwent surgical treatment were retrospectively analyzed. The diagnostic accuracy of preoperative ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) and puncture biopsy of aspiration were compared and analyzed. Kaplan-Meier method was performed to calculate disease-free survival rate. The gender differences in the clinical and pathological features of pSPN were compared. According to whether recurrence and metastasis occurred after the primary operation, the patients with pSPN were divided into recurrence and metastasis group ( n=3) and disease-free survival group ( n=250). Univariate analysis was used to analyze whether gender, age, married status, with clinical symptoms, history of alcohol drinking or smoking, puncture biopsy of aspiration before operation, location and maximum diameter of tumor, surgical method (open surgery, minimally invasive surgery), type of surgery (parenchymal preservation surgery, conventional surgery), and pathological features (cellular atypia, invasion of adjacent organs, lymphovascular invasion, peripancreatic fat invasion, perineural invasion, capsular invasion and pancreatic parenchyma invasion) were predictive factors of recurrence and metastasis of pSPN. Two independent sample t test, chi-square test and Fisher′s exact test were used for statistical analysis. Results:Among 253 patients with pSPN, 49 (19.4%) were males and 204 (80.6%) were females. The diagnostic accuracy of MRI and CT were both higher than that of ultrasound (73.4%(124/169), 64.0%(146/228) vs. 33.3%(78/234)), and the diagnostic accuracy of MRI was higher than that of CT, and the differences were statistically significant ( χ2=62.93, 43.58 and 3.89, P<0.001, <0.001 and =0.049). The diagnostic accuracy of puncture biopsy of aspiration combined with immunochemistry was higher than that without combined immunochemistry (100.0%(23/23) vs. 8/13), and the difference was statistically significant (Fisher′s exact test, P=0.003). Eight cases missed during follow-up, and all the other 245 patients survived with the 5- year and 10-year disease-free survival rates of 99.2% and 97.2%, respectively. Male pSPN patients were older than female patients at diagnosis ((40.0±13.2) years old vs. (32.6±11.9) years old) and the maximum diameter of tumor was smaller than that of female patients ((3.88±2.05) cm vs. (4.87±3.05) cm), and the differences were statistically significant ( t=3.83 and -2.15, P<0.001 and =0.032). The results of univariate analysis showed that lymphovascular invasion was significantly correlated with pSPN recurrence and metastasis (Fisher′s exact test, P=0.012), and the other factors were not correlated with pSPN recurrence and metastasis (all P>0.05). Conclusions:CT, MRI and puncture biopsy of aspiration are comparatively reliable diagnostic method for pSPN before operation. There are significant differences in pSPN tumor growth and age of onset between genders. The postoperative recurrent rate of pSPN is low and the prognosis is good. Even if recurrence and metastasis occur, the patients can still survive for a long time after surgical treatment.

Extrapancreatic primary retroperitoneal solid pseudopapillary neoplasm: A case report

@#Solid pseudopapillary neoplasm (SPN) is a rare pancreatic neoplasm. This case reports a 63-year-old female previously diagnosed with bilateral adrenocortical carcinoma post resection who presents with persistent epigastric pain. Suspicions for recurrence prompted diagnostics revealing retropancreatic and left suprarenal foci, both suspicious for malignancy. Resection of both tumors yielded a moderately to poorly differentiated retropancreatic carcinoma with differentials not limited to recurrence and pancreatic neuroendocrine tumor with a left suprarenal lymph node. Immunohistochemistry was then done and was consistent with SPN having a strong expression for b-catenin, vimentin, pancytokeratin. It was non-specific to melan-A and negative for chromogranin A, synaptophysin, inhibin-a and CD10. At 6 months’ follow up, patient is clinically well and abdominal CT scan showed no recurrence. SPNs can masquerade as other neoplasms having similar clinical, radiologic and histopathologic features. Immunohistochemistry thus plays a crucial role for accurate diagnosis and management. Surgical resection still remains the treatment of choice and can provide a 95% overall survival rate, while limited evidence supports the use of adjuvant chemotherapy or radiation.

Neoplasia sólida pseudopapilar de páncreas: una serie de cinco casos y revisión de la literatura / Solid pseudopapillary neoplasm of the pancreas: A series of five cases and a literature review

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.

Progress and controversies in diagnosis and treatment of solid pseudopapillary neoplasm of the pancreas / 国际外科学杂志

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare low-grade malignant tumor. In recent years, with the improvement of national health check-up awareness and the advancement of imaging diagnostic technology, the detection rate of SPN has continued to rise. With the release of guidelines for many pancreatic cystic tumors at home and abroad, the diagnosis and treatment of SPN has gradually become standardized. However, its diagnostic and treatment strategies are still difficult and controversial. Therefore, the author summarizes the current status of diagnosis and treatment of SPN by referring to the literature and combining clinical experience, in order to deepen the understanding of rare pancreatic tumor by clinicians and further promote the standardized diagnosis and treatment of SPN in China.

Multiphasic enhanced CT-based radiomics signature for preoperatively predicting the invasive behavior of pancreatic solid pseudopapillary neoplasm / 中华放射学杂志

Objective:To explore the value of multiphasic CT-based radiomics signature in predicting the invasive behavior of pancreatic solid pseudopapillary neoplasm (pSPN).Methods:The multiphasic CT images of patients with pSPN confirmed by postoperative pathology in the First Affiliated Hospital of Zhengzhou University from January 2012 to January 2021 were analyzed retrospectively. There were 23 cases of invasiveness and 59 cases of non-invasiveness. The region of interest(ROI) was artificially delineated layer by layer in the plain scan, arterial-phase and venous-phase images, respectively. The 1 316 image features were extracted from each ROI. The data set was divided into training and validation sets with a ratio of 7∶3 by stratified random sampling, and synthetic minority oversampling technique (SMOTE) algorithm was used for oversampling in the training set to generate invasive and non-invasive balanced data for building the training model. The constructed model was validated in the validation set. The receiver operating characteristic(ROC) analysis was used to evaluate model performance and the Delong′s test was applied to compare the area under the ROC curve (AUC) of different predict models. The improvement for classification efficiency of each independent model or their combinations were also assessed by net reclassification improvement (NRI) and integrated discrimination improvement (IDI) indices.Results:After feature extraction, 2, 6 and 3 features were retained to construct plain-scanned model, arterial-phase and venous-phase models, respectively. Seven independent-phase and combined-phase models were established. Except the plain-scanned model, the AUC values of other models were greater than 0.800. The arterial-phase model had the best efficiency for classification among all independent-phase models. The AUC values of arterial-phase model in the SMOTE training and validation sets were 0.913 and 0.873, respectively. By combining the radiomics signature of the arterial-phase and venous-phase models, the AUC values of training and validation sets increased to 0.934 and 0.913 respectively. There were no significant differences of the AUC values between the scan-arterial venous-phase model and arterial venous-phase model in both training and validation sets (both P>0.05). The NRI and IDI indexes showed that the combined form of plain-scan model and arterial-venous-phase model could not significantly improve the classification efficiency in the validation set (both NRI and IDI<0). Conclusions:The arterial-phase CT-based radiomics model has a good predictive performance in the invasive behavior of pSPN, and the combination with a venous-phase radiomics model can further improve the model performance.

Tumores sólidos pseudopapilares en el departamento de patología de la Universidad de Antioquia: serie de casos / Solid pseudopapillary tumors in the pathology department of the Universidad de Antioquia: Series of cases

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.

Carcinoma pseudopapilar sólido del páncreas / Solid pseudo-papillary carcinoma of the pancreas

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)

Experiencia en el manejo del tumor sólido pseudopapilar de pancreas en un centro de referencia / Experience in the management of the solid pseudopapillary tumor of the pancreas in a reference center

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)

Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation: report of one case and literature review / 中华器官移植杂志

Objective:To Eveluate the safty and clinical efficacy of combined laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation in the treatment of solid pseudopapillary neoplasm.Methods:A 22 years old solid pseudopapillary neoplasm female patient who underwent distal pancreatectomy and an autologous islet transplantation at Tianjin First Central Hospital, clinical date for 6 months follow up was collected and analyzed.Results:The patient was well recovered after surgery, and during the post-operative follow up, the fasting blood glucose was 5.72 mmol/L, HbA1c was 6.1%, remained insulin independent, the liver function was kept well.Conclusions:Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation can effectively prevent diabetes after distal pancreatectomy.

Neoplasia sólida pseudopapilar pancreática: comunicación de cuatro casos clínicos y revisión de la literatura / Solid pseudopapillary tumor of the pancreas: four case reports and a review of the lilerature / Neoplasia sólida pseudopapilar pancreática: comunicação de quatro casos clínicos e revisão da literatura

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.

Tumor papilar sólido-quístico pancreático / Solid pseudopapillary tumor of the pancreas

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.

Neoplasia Pseudopapilar sólida del Páncreas. A Propósito de un Caso / Solid Pseudopapillary Neoplasia of the Pancreas. Case Report

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.

Tumor sólido pseudopapilar de páncreas: reporte de caso / Pseudopapilar solid tumor of pancreas: case Report

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.

Incidentally detected solid pseudopapillary neoplasm of pancreas in a child with an ovarian cyst: a case report

Solid pseudopapillary neoplasms (SPN) are the most common as well as very rare pediatric tumors of the pancreas. Most of the literature is derived from adult SPNs. As per world health organization, these tumors considered as low grade malignant with excellent survival outcomes after complete surgical resection. We report a case of incidentally detected SPN in a 16-year-old female child with an ovarian cyst. She underwent pylorus preserving pancreaticoduodenectomy and ovarian cystectomy. Histopathology revealed well differentiated SPN of the pancreas head and ovarian cystadenoma. She is symptom-free at the 6th month follow up.

Pancreatoduodenectomy for solid pseudopapillary tumor of the pancreas: a multi-institution study / Duodenopancreatectomia para o tumor pseudopapilar sólido do pâncreas: estudo multi-institucional

ABSTRACT Background: Solid pseudopapillary tumor of the pancreas is a rare low-grade malignant neoplasm. Most patients present with nonspecific symptoms until the tumor becomes large. Complete surgical resection by pancreatoduodenectomy is the treatment of choice for tumors located in the head of the pancreas Aim: To analyzed the clinicopathologic features, management, and outcomes of patients who had solid pseudopapillary tumor of the head pancreas and underwent surgical resection. Methods: Were analyzed 16 patients who underwent pancreatoduodenectomy for this condition. Results: Mean age was 25.7 years old, and 15 patients were female (93.7%). Nonspecific abdominal pain was present in 14 (87.5%). All underwent computed tomography and/or magnetic resonance imaging as part of diagnostic workup. The median diameter of the tumor was 6.28 cm, and surgical resection was performed with open or laparoscopic pancreatoduodenectomy without neoadjuvant chemotherapy. Postoperative complications occurred in six patients (37.5%) and included pancreatic fistula without mortality. The mean of hospital stay was 10.3 days. Median follow-up was 3.6 years, and no patient had local recurrence or metastatic disease. Conclusion: For these patients surgical resection with pancreatoduodenectomy is the treatment of choice showing low morbidity, no mortality, and good long-term survival.

RESUMO Racional: Tumor sólido pseudopapilar do pâncreas é neoplasia maligna rara, de baixo grau de malignidade. A maioria dos pacientes apresenta sintomas inespecíficos até que o tumor aumente de tamanho. A ressecção cirúrgica completa através a duodenopancreatectomia é o tratamento de escolha para os localizados na cabeça do pâncreas. Objetivo: Analisar as características clinicopatológicas, tratamento e resultados de pacientes com tumor sólido pseudopapilar do pâncreas localizado na cabeça do pâncreas submetidos à ressecção cirúrgica. Método: Foram analisados 16 pacientes com duodenopancreatectomia devido a esse tumor localizado na cabeça do pâncreas. Resultados: Havia 15 mulheres (93,7%) e a média de idade era de 25,7 anos. Dor abdominal não específica esteve presente em 14 pacientes (87,5%). Todos realizaram tomografia computadorizada do abdome e/ou ressonância nuclear magnética como parte da investigação. O diâmetro médio do tumor era de 6,28 cm e a ressecção cirúrgica foi realizada por duodenopancreatectomia, tanto por laparotomia quanto por videolaparoscopia, com ou sem quimioterapia neoadjuvante. As complicações pós-operatórias ocorreram em seis pacientes (37,5%) e incluíram fístula pancreática, sem mortalidade. O tempo médio de internação hospitalar foi de 10,3 dias. O tempo médio de seguimento foi de 3,6 anos e nenhum paciente apresentou recorrência local ou doença metastática. Conclusões: A ressecção cirúrgica através da duodenopancreatectomia é o tratamento de escolha para estes pacientes. Os resultados mostraram baixa morbidade, nenhuma mortalidade e boa sobrevida em longo prazo.

Spleen Preservation in Laparoscopic Distal Pancreatectomy for Solid Pseudopapillary Neoplasm is Oncologically Safe

PURPOSE: Laparoscopic distal pancreatectomy (LDP) has been widely performed for solid pseudopapillary neoplasm (SPN) involving the body or tail of the pancreas. However, it has not been established whether spleen preservation in LDP is oncologically safe for the treatment of SPN with malignant potential. In this study, we compared the short- and long-term outcomes between patients with SPN who underwent laparoscopic spleen-preserving distal pancreatectomy (LSPDP) vs laparoscopic distal pancreatectomy with splenectomy (LDPS). METHODS: We retrospectively reviewed the medical records of 46 patients with SPN who underwent LDP between January 2005 and November 2016. Patients were divided into 2 groups according to spleen preservation: the LSPDP group (n=32) and the LDPS group (n=14). Clinicopathologic characteristics and perioperative outcomes were compared between groups. RESULTS: There were no significant differences in pathologic variables, including tumor size, tumor location, node status, angiolymphatic invasion, or perineural invasion between groups. Median operating time was significantly longer in the LSPDP group vs the LDPS group (243 vs 172 minutes; p=0.006). Estimated intraoperative blood loss was also significantly greater in the LSPDP group (310 vs 167 ml; p=0.063). There were no significant differences in incidence of postoperative complications (≥ Clavien-Dindo class IIIa) or pancreatic fistula between groups. After a median follow-up of 35 months (range, 3S153 months), there was no recurrence or disease-specific mortality in either group. CONCLUSION: The results show that LSPDP is an oncologically safe procedure for SPN involving the body or tail of the pancreas.

Analysis of 4 children with pancreatic solid pseudopapillary neoplasm treated by total laparoscopic pancreaticoduodenectomy / 中华胰腺病杂志

Objective@#To investigate the application and surgical experience of total laparoscopic pancreaticoduodenectomy (TLPD) in treating children with solid pseudopapillary neoplasm (SPN) of pancreas.@*Methods@#Clinical data 4 children with SPN who underwent TLPD in Jilin University First Hospital from April 2017 to June 2018 were retrospectively analyzed.@*Results@#Among the 4 children, a case was male and 3 cases were female. Their age ranged from 9 to 14 year-old, the height ranged from 1.2 to 1.7 meters, and body weight ranged from 30 to75 kg. All patients complained of upper abdominal pain, one child had nausea and vomiting, and one child had abdominal mass. All patients underwent abdominal enhanced CT scan before operation, which showed a mass-like low-density shadow or mixed density shadow in the pancreatic head, with slightly uneven enhancement or no obvious enhancement. All 4 patients underwent TLPD, and the operation was successful without conversion to open surgery. The duration of operation time ranged from 250 to 365 minutes, the intraoperative blood loss ranged from 80 to 120 ml, the tumor size ranged from 4 to 8 cm, and the hospital stay ranged from 10 to 22 days. One patient developed grade B pancreatic fistula after surgery and was cured after conservative treatment. Pathological examinations of all patients confirmed the diagnosis of pancreatic SPN. All patients were followed up until February 2019, and no significant discomfort was observed and no recurrence or metastasis was found.@*Conclusions@#TLPD was safe and feasible in children in relatively large pancreatic surgery centers with extensive laparotomy and TLPD experience.