Pancreatic tumors in children: diagnosis and treatment / 中华肝胆外科杂志

Objective:To study the strategies in the diagnosis and treatment of pancreatic tumors in children.Methods:The clinical data of 18 children with pancreatic tumor managed at the Children's Hospital Affiliated to Chongqing Medical University from March 2015 to September 2020 were retrospectively studied. There were 8 males and 10 females, age ranged from 3 months to 14 years and 11 months, with a median age of 8 years and 2 months. Clinical data including age, gender, pathological data, surgical methods, chemotherapy, tumor location and treatment outcomes were collected. Follow-up was conducted by outpatient visits and by telephone.Results:Abdominal ultrasound, enhanced CT and/or MRI examinations were performed on all these patients, with findings of either a cystic or solid lesion of pancreas. All patients were treated by laparotomy under endotracheal intubation and general anesthesia. The operations were all completed successfully. Among the 18 patients, there were 11 patients with solid pseudopapillary tumors and 7 patients with pancreatoblastoma (PBL). The tumors were located in the head of the pancreas in 13 patients (including 3 patients who underwent pancreaticoduodenectomy, 1 patient who underwent resection of the head of the pancreas with preservation of the duodenum, and 9 patients who underwent resection of the tumors). The tumors were located in the body and tail of the pancrease in 5 patients (including 3 patients who underwent resection of the body and tail of the pancreas with preservation of spleen, and 2 patients who underwent resection of tumors). Because of huge tumors, 1 patient had bilateral lung, left supraclavicular fossa lymph node and retroperitoneal lymph node metastasis, 3 patients were confirmed to have PBL by biopsy, and these tumors were resected completely after neoadjuvant chemotherapy. Postoperative pathology showed that all the 3 patients had PBL and were given systematic chemotherapy. Postoperative pancreatic fistula occurred in 1 patient and chylous fistula in another patient, both were discharged home successfully after conservative treatments. All patients were followed-up for 2-7 years, and all children were tumor-free.Conclusion:It is not difficult to diagnose pediatric pancreatic tumors by ultrasound, CT and MRI before operation, and postoperative pathology was needed to confirm the diagnosis. Function-preserving surgical resection was the treatment of choice for pancreatic tumors in children.

Profil scanographique des tumeurs du pancréas dans 3 services de radiologie à Kinshasa: une série de cas rétrospective / Scanographic profile tumors from 3 radiology division in Kinshasa: retrospective serial cases

Contexte et objectif. Le pancréas étant un organe profond, son exploration clinique est parfois limitée et peu performante. Le diagnostic de ces pathologies repose principalement sur le dosage des enzymes pancréatiques (la lipasémie notamment) et l'imagerie médicale. L'objectif de la présente étude était de décrire le profil scanographique des tumeurs pancréatiques. Méthodes. Il s'agissait d'une série des cas documentaires menée dans 3 Services de radiologie à Kinshasa de janvier 2016 à juin 2021, ayant retenu 86 comptes rendus de tomodensitométrie (TDM) abdominale des patients avec pathologie pancréatique. Toutes les images archivées ont été relues par spécialistes en imagerie. Résultats. 66 cas de tumeurs pancréatiques ont été colligés. Le sexe masculin était prépondérant (sex-ratio H/F=1,6) avec un âge moyen de 55,7 ± 14,7 ans (16 à 92 ans). Le syndrome de cholestase (50 %) et la douleur abdominale (épigastrique) étaient les indications les plus retrouvées. Les contours étaient lobulés dans plus de la moitié des cas (56,1 %). Le canal de Wirsung était dilaté dans la plupart des tumeurs. Les tumeurs étaient résécables dans 26 % des cas. Conclusion. La TDM abdominale contribue au diagnostic des pathologies pancréatiques. Elles affectent le plus souvent des sujets âgés du sexe masculin et sont dominées par les tumeurs qui sont généralement diagnostiquées au stade non-résécable

Context and objective. The pancreas being a deep organ, its clinical exploration is sometimes limited and inefficient. The diagnosis of pancreatic pathologies is mainly based on the dosage of pancreatic enzymes (lipasemia in particular) and medical imaging. The objective of the present study was to describe the scanographic profile of pancreatic tumors in 3 radiology departments in Kinshasa. Methods. It was a retrospective serial case study conducted in 3 radiology departments in Kinshasa from January 2016 to June 2021, having retained 86 reports of abdominal computed tomography (CT) scan of patients with pancreatic pathology. Results. 66 cases of pancreatic tumors were involved. Male patients were predominant (sex-ratio M/F=1.6) and the mean age of 55.7 ± 14.7 years (range: 16 to 92 years). Cholestasis syndrome (50 %) and abdominal (epigastric) pain were the most common indications. In tumors, the contours were lobulated (56.1 %). The Wirsung duct was dilated in most tumors. The tumors were resectable in 26 % of cases. Conclusion. The abdominal CT-scan contributes to the diagnosis of pancreatic pathologies. These pathologies mostly affect male elderly subjects, are predominantly tumoral and generally diagnosed in late unresectable stage.

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 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.

Differentiation of hypovascular pancreatic neuroendocrine tumors and mass-forming pancreatitis by enhanced CT / 医学研究生学报

ObjectiveTo explore the value of MSCT for differentiating the hypovascular pancreatic neuroendocrine tumors (Hypo-PNETs) from mass-forming pancreatitis.Methods21 patients with histological-confirmed MPFs and 19 patients with Hypo-PNETs who underwent preoperative dynamic contrast-enhanced CT were included. The CT images were analyzed including tumor size,density, border, CT values in each phase, dilatation of pancreatic duct and bile duct and metastases. The clinical data included age, sex and clinical symptoms.ResultsMFPs often occurred in male patient compared with Hypo-PNETs (85.7% vs 52.6%, P<0.05), and the difference is statistically significant; Metastases only occurred in Hypo-PNETs (P<0.05), and the difference is drastically significant. The well-defined margin often occurred in Hypo-PNETs (52.4% vs 47.6%, P=0.032). Solid tumor more happened in MFPs(95.2% vs 47.4%, P＜0.05). The CT values in the portal of MFPs were higher than Hypo-PNETs(P<0.05). The combined features showed accepted diagnostic performance for differentiating Hypo-PNETs from MFPs (89.5% of sensitivity and 95.2% of specificity).ConclusionMFPs often occurred in male patient, and CT values of portal phase, density, border and metastases may be valuable for differentiating Hypo-PNETs from MFPs.

Analysis of the clinical features of 20 cases of non-functioning pancreatic neuroendocrine tumors / 中国医师进修杂志

Objective To investigate the clinical features of non- functioning pancreatic neuroendocrine tumors and to summarize experiences in the diagnosis and treatment. Methods The clinical pathology and follow-up data of 20 patients with non-functioning pancreatic neuroendocrine tumors treated in Nanjing Drum Tower Hospital Affiliated to Medical School of Nanjing University, from January 2008 to February 2018 were retrospectively analyzed. Results Among the 20 patients, 9 patients (9/20) were asymptomatic, and the tumor was found unexpectedly by physical examination. Eleven patients (11/20) with symptoms were admitted to the hospital due to non-specific gastrointestinal symptoms or compression symptoms caused by tumor space occupying. None of the patients had endocrine disorders. There were nineteen patients who received surgical treatment. According to the grading system, there were 7 patients (7/19) with G1, and 12 patients (12/19) with G2 . Among the 14 patients with diameter of tumor greater than 2 cm, there are 4 patients (4/14) with lymph node metastasis, and 4 patients (4/14) with liver metastasis. Five patients with diameter of tumor less than or equal to 2 cm had no distant metastasis. Conclusions NF-pNETs is often with nonspecific symptoms. Imaging examination is an important diagnostic method. Operation is the primary therapy for NF-pNETs. Because most of the small NF-pNETs with no symptoms are benign and grow slowly, and the metastatic rate is very low. It is important to weigh the pros and cons of surgical treatment for these patients.

Middle segment pancreatectomy in treatment for pancreatic neck and body tumors: clinical analysis of 26 cases / 中华内分泌外科杂志

Objective To investigate the clinical efficacy of middle segment pancreatectomy (MSP) in treatment for pancreatic neck and body tumors.Methods From Jan.2000 to Dec.2016,the clinicopathological data of 26 patients with pancreatic neck or body tumors were analyzed retrospectively.Results All of the 26 patients received middle segment pancreatectomy.14 cases had serous or mucinous cystadenomas.The tumor diameter was 2.3 to 5.6 cm (averaging from 3.8 cm).9 cases had solid pseudopapillary tumors.The tumor diameter was 3.3 to 5.0 cm(averaging from 3.6 cm);One case had insulinoma and the diameter was 2.0 cm.Two cases had nonfunctional pancreatic neuroendocrine tumors,and the diameter was 2.1 and 2.4 cm.The post-complication morbidity rate was 53.8％.The main post-complications of the group were pancreatic fistula (9 cases,34.2％),intra-abdominal infection(1 case,3.8％),delayed gastric emptying (2 cases,7.6％),lymphatic fistula (1 case,3.8％),and abdominal infection (1 case,3.8％).All of the 26 patients healed after surgery without undergoing surgery again.There were no mortality during perioperative period.All patients were followed up for 6 to 60 months.All of the 26 patients had good life quality,without new-onset diabetes or cancer recurrence during the follow-up period of 6 to 60 months after operation.Conclusion Middle segment pancreatectomy is safe and feasible in treatment of benign or low-graded malignant pancreatic neck and body tumors and is capable of preserving pancreatic endocrine and exocrine function.

The role of endoscopic ultrasound in detection of small pancreatic tumors / 中国癌症杂志

Early detection of pancreatic cancer is difficult and most of patients with pancreatic cancer present with locally advanced stage disease or distant metastasis on diagnosis. Micrometastasis occurs in the majority of pancreatic cancers larger than 2 cm in diameter and making early diagnosis and treatment essential to improve the prognosis. Small pancreatic tumors less than 2 cm in diameter can be missed on transcutaneous ultrasound, CT and MRI. Endoscopic ultrasound (EUS) has been considered the most sensitive modality in evaluation of pancreatic lesions. It can be placed in close proximity to the pancreas and provide real-time, high resolution imaging using a high frequency ultrasound probe to find lesions as small as 5 mm. Recently, small pancreatic tumors have been increasingly detected with the widely used EUS. The development of new techniques such as EUS elastography, contrast-enhanced harmonic EUS (CEH-EUS) and fine needle aspiration biopsy (EUS-FNAB) have enhanced the ability of EUS in detection of small solid pancreatic tumors as well as in differentiation of malignancies from benign tumors.

A comparative study on MRI appearance and pathological grading for pancreatic neuroendocrine tumors / 中华内分泌外科杂志

Objective To explore the value of conventional and functional MRI image parameter in preoperative grade of pancreatic neuroendocrine tumours (pNET).Methods Data of 35 cases of pNET were retrospectively analyzed,including tumor size,intensity of T1WI's and T2WI's signal,enhancement feature,and apparent diffusion coefficients (ADC)value.All kinds of tumors were classified into G1 to G3 according to WHO's classification in 2010.Results There were 40 pNETs (mean size(4.2±2.9)cm) among the 35 patients (20 male,and 15 female).By WHO's tumor grading,the number of patients from G1 to G3 was 18(45％),12(30％),and 10(25 ％),respectively.A higher grade was associated with larger tumor size (P＜0.05).Intensity of T1WI and T2WI signal has no significance for predicting tumor grade.Enhancement feature had statistical significance on tumor's grade (P＜0.05).ADC value decreased with the increase of tumor's grade,(2.23±0.80) × 10-3,(1.80±0.73) × 10-3,(0.92±0.24) ×10-3 mm2/s respectively for tumors from grade G1 to G3.Conclusion Enhancement features of MRI image and ADC value are conducive to preoperative grading of pNET.

Middle segment pancreatectomy in treatment for benign pancreatic tumors and its effect on pancreatic exocrine function / 中华内分泌外科杂志

Objective To investigate the application of middle segment pancreatectomy (MSP) in treating benign pancreatic tumors and the effect on exocrine function.Methods From Jan.2010 to Feb.2013,19 cases with benign pancreatic tumor undergoing MSP were enrolled in our study.Pre-and postoperative pancreatic exocrine function were studied.Results No perioperative death happened.The tumor diameter was 4.2 to 7.0cm,operative time was 80 to 320 mins,and intraoperative blood loss was 200 to 1000 ml.The retained pancreas was＞l.5 cm at pancreatic head side,and＞5 cm at pancreatic caudal side.Three days after surgery,the peritoneal drainage and amylase was 2.5-12.5 ml and 568.4-1013 IU/L,respectively.Seven days after surgery,the peritoneal drainage and amylase was 1.0-19.8 ml and 45.4-76.3 IU/L,respectively.The postoperative hospitalization stay were 10 to 25 days.Three cases (all single anastomosis) had pancreatic fistula after surgery and healed after 3 months without undergoing surgery again.Patients were followed up for 2 months to 3 years.All patients have a good life quality,without new-onset diabetes and cancer recurrence.MSP had no side effects on pancreatic exocrine function.Conclusion MSP is safe and feasible in treatment of benign pancreatic tumors.

Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.

EUS Elastography: Advances in Diagnostic EUS of the Pancreas

Elastography is an imaging modality for the evaluation of tissue stiffness, which has been used for the analysis of superficial organs, such as those of the breast and prostate. The measurement of tissue elasticity has been reported to be useful for the diagnosis and differentiation of tumors, which are stiffer than normal tissues. Endoscopic ultrasonography elastography (EUS-EG) is a promising imaging technique with a high degree of accuracy for the differential diagnosis of solid pancreatic tumors. Recent introduction of second generation EUS-EG allows for the quantitative analysis of tissue stiffness. Here, we review our knowledge and preliminary experience with the use of EUS-elastography for the diagnosis of pancreatic disease.

Neoplasia mucinosa papilar intraductal del páncreas: Presentación de caso y revisión de la literatura / A pancreatic intraductal papillary mucinous neoplasm: A case report and literature review

La neoplasia mucinosa papilar intraductal (NMPI) es un tumor pancreático raro que cada día se diagnostica más debido al aumento de los estudios de imágenes para la evaluación del páncreas; los pacientes con este tipo de tumores pueden presentarse a los servicios de urgencias con dolor abdominal agudo que en múltiples ocasiones se cronifica y se acompaña de episodios repetitivos de pancreatitis. En el presente artículo describimos un caso de un paciente NMPI como causa de pancreatitis aguda, quien consulta a urgencias por dolor abdominal severo.

Intraductal papillary mucinous neoplasms (IPMNs) are rare pancreatic tumors that are diagnosed more and more frequently as imaging for evaluation of the pancreas becomes more widespread. Patients with these tumors may arrive at an emergency room with acute abdominal pain that often becomes chronic, accompanied by repeated episodes of pancreatitis. In this paper we describe the case a patient with IPMN which was a cause of acute pancreatitis. This patient came to the emergency room for severe abdominal pain.

Pancreatic Tumors: Emphasis on CT Findings and Pathologic Classification

Pancreatic tumors can be classified by their morphologic features on CT. The subtypes include solid tumors, mixed cystic and solid lesions, unilocular cysts, multilocular cystic lesions, and microcystic lesions. Endoscopic US and MRI can provide detailed information for classifying pancreatic lesions. Each subtype has different kinds of tumors and malignant potential, thus the classification can be useful for a better differential diagnosis and treatment planning. For this purpose, we suggest an appropriate modified classification system by using the imaging features of pancreatic tumors with an emphasis on CT findings and illustrate various findings of typical and atypical manifestations.