Pancreatic neuroendocrine tumors: Are tumors smaller than 2 cm truly indolent?

BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are relatively rare but rank as the second most common pancreatic neoplasm. They can be functional, causing early metabolic disturbances due to hormone secretion, or non-functional and diagnosed later based on tumor size-related symptoms. Recent diagnoses of PNETs under 2 cm in size have sparked debates about their management; some practitioners advocate for surgical removal and others suggest observation due to the tumors' lower potential for malignancy. However, it is unclear whether managing these small tumors expectantly is truly safe. AIM: To evaluate poor prognostic factors in PNETs based on tumor size (> 2 cm or < 2 cm) in surgically treated patients. METHODS: This cohort study included 64 patients with PNETs who underwent surgical resection between 2006 and 2019 at a high-complexity reference hospital in Medellín, Colombia. To assess patient survival, quarterly follow-ups were conducted during the first year after surgery, followed by semi-annual consultations at the hospital's hepatobiliary surgery department. Qualitative variables were described using absolute and relative frequencies, and quantitative variables were expressed using measures of central tendency and their corresponding measures of dispersion. RESULTS: The presence of lymph node involvement, neural involvement, and lymphovascular invasion were all associated with an increased risk of mortality, with hazard ratios of 5.68 (95%CI: 1.26-25.61, P = 0.024), 6.44 (95%CI: 1.43-28.93, P = 0.015), and 24.87 (95%CI: 2.98-207.19, P = 0.003), respectively. Neural involvement and lymphovascular invasion were present in tumors smaller than 2 cm in diameter and those larger than 2 cm in diameter. The recurrence rates between the two tumor groups were furthermore similar: 18.2% for tumors smaller than 2 cm and 21.4% for tumors larger than 2 cm. Patient survival was additionally comparable between the two tumor groups. CONCLUSION: Tumor size does not dictate prognosis; lymph node and lymphovascular involvement affect mortality, which highlights that histopathological factors-rather than tumor size-may play a role in management.

Multicenter Study on the Performance of Imaging Tests Compared to Endosonography-Guided Fine-Needle Aspiration in the Diagnosis of Solid Pseudopapillary Neoplasms of the Pancreas.

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.

Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort.

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.

A Rare Presentation of a Solid Pseudopapillary Neoplasm of the Pancreas.

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.

Pancreatic neuroendocrine tumors: surgical resection / Tumores neuroendócrinos ressecáveis do pâncreas: abordagem cirúrgica

ABSTRACT Introduction: Pancreatic neuroendocrine tumors (pNET) correspond to about 3% of all tumors in pancreas and could be presented as a difficult diagnosis and management. Objective: To review the diagnosis and treatment of the pNET available in scientific literature. Method: A bibliographic survey was performed by means of an online survey of MeSH terms in the Pubmed database. A total of 104 articles were published in the last 15 years, of which 23 were selected as the basis for the writing of this article. Results: pNET is an infrequent neoplasia and their incidence, in USA, is about 1:100.000 inhabitants/year. Thereabout 30% of them produce hormones presenting as a symptomatic disease and others 70% of the cases could be silent disease. Magnetic Resonance Imaging (MRI) and/or Computed Tomography (CT) have similar sensitivy to detect pNET. They are very important when associated to nuclear medicine mainly Positron Emission Tomography (PET-CT) Gallium-68 to find primary tumor and its staging. The appropriate treatment should be chosen based on characteristics of the tumor, its staging and associated comorbidities. Conclusion: The surgical resection is still the best treatment for patients with ressectable pancreatic NETs. However, the size, grade, tumor functionality, stage and association with multiple endocrine neoplasia type 1 (MEN-1) are important to define who will be eligible for surgical treatment. In general, tumors bigger than 2 cm are eligible for surgical treatment, except insulinomas whose surgical resection is recommended no matter the size.

RESUMO Introdução: Os tumores neuroendócrinos pancreáticos (TNE-P) correspondem a cerca de 3% de todas as neoplasias no pâncreas e são de diagnóstico e manejo difíceis. Objetivo: Revisar o diagnóstico e tratamento desses tumores disponíveis na literatura científica atual. Método: Foi realizado levantamento bibliográfico por meio de pesquisa online de MeSH terms na base de dados do Pubmed. Ao todo foram analisados 104 artigos publicados nos últimos 15 anos, dos quais 23 foram selecionados como base para a redação deste artigo. Resultados: TNE-P é neoplasia pouco frequente com incidência estimada em 1: 100.000 habitantes/ano. Cerca de 30% produzem hormônios, ditos funcionantes, que se apresentam como doença sintomática e outros 70% podem se apresentar como doença silenciosa. Como recurso diagnóstico o uso de tomografia computadorizada ou ressonância nuclear magnética apresentam níveis de sensibilidade parecidas. Sua associação à recursos da medicina nuclear são de grande valia para localização de tumores primários e estadiamento, destacando-se o uso da tomografia por emissão de pósitrons (PET-CT) com Gálio-68. A decisão terapêutica deve ser baseada nas características tumorais, estadiamento e comorbidades associadas. Conclusão: A abordagem cirúrgica permanece como a terapia de escolha para pacientes com TNE-P ressecáveis. No entanto, a seleção de pacientes para tratamento cirúrgico deve seguir critérios baseados em funcionalidade do tumor, grau, estágio e associação com neoplasia múltipla tipo 1. De forma geral, tumores maiores de 2 cm têm indicação cirúrgica, excetuando-se os insulinomas, cuja proposta cirúrgica independe de seu tamanho.

Utilidad pronóstica del PET/CT en cáncer de páncreas / Prognostic value of PET/CT in pancreatic cancer

Background: Pancreatic cancer is the tenth most prevalent cancer in world, and represents the fourth cause of cancer death. It has a five year-survival of 5%. Aim: To assess the prognostic value of PET/CT in pancreatic cancer. Material and Methods: Sixty-nine patients with pancreatic adenocarcinoma who underwent staging 18F-fluorodeoxyglucose (FDG) PET/CT between December 2008 and July 2016 were selected. Gender, age, body-mass index, laboratory tests (Ca 19-9, hemoglobin, erythrocyte sedimentation rate, liver enzymes, lactate dehydrogenase), histological differentiation of tumor, American Joint Committee on Cancer (AJCC) stage, size and 18F-FDG uptake (maximal stan-dardized uptake value [SUVmax]) of the primary tumor, nodal involvement and distant metastasis detected by PET/CT were registered. Survival was assessed using Kaplan-Meier curves, Log Rank test and Cox multivariable analysis. Results: Mortality was 66.7%, during a mean observation time of 18 months (range 20 days-66 months). Curative surgery, lack of metastases detected by PET/CT, histologically well differentiated tumors, and SUVmax ≤ 4.3 were significantly associated with a better specific survival, determined by the Log Rank test. Histological differentiation was the only variable that had a statistically significant prognostic value in the multivariable analysis. Conclusions: The detection of distant metastases and the intensity of primary tumor 18F-FDG uptake during PET/CT provide useful prognostic information in pancreatic cancer patients.

Contribution of DNA repair xeroderma pigmentosum group D genotypes to pancreatic cancer risk in the Chinese Han population

Abstract This study aimed to determine the association between the polymorphisms and haplotypes in the xeroderma pigmentosum group D (XPD) gene and the risk of pancreatic cancer in the Chinese Han population. SNaPshot was used for genotyping six SNP sites of the XPD gene. Comparisons of the correlations between different genotypes in combination with smoking and the susceptibility to pancreatic cancer were performed. Individual pancreatic cancer risk in patients who carry mutant C alleles (AC, CC, and AC+CC) at rs13181 increased (p < 0.05). Taking non-smoking individuals who carry the AA genotype as a reference, and non-smoking individuals who carry mutant allele C (AC+CC), the risk of pancreatic cancer increased by 3.343 times in individuals who smoked ≥ 20 cigarettes daily, 3.309 times in individuals who smoked ≥ 14 packs per year, 5.011 times in individuals who smoked ≥ 24 packs per year, and 4.013 times in the individuals who smoked ≥ 37 packs per year (P < 0.05). In addition, haplotype analysis revealed that haplotype AGG, which comprised rs13181, rs3916874 and rs238415, was associated with a 1.401-fold increase in pancreatic cancer risk (p < 0.05). We conclude that the polymorphism of XPD Lys751Gln (rs13181) in combination with smoking contributes to increased risk of pancreatic cancer in the Chinese Han population. Haplotype AGG might be a susceptibility haplotype for pancreatic cancer.

Sweet´s syndrome and pancreatic neoplasm: an atypical association

El síndrome de Sweet es una dermatosis inflamatoria poco común, que se ha asociado a tumores malignos, principalmente de tipo hematológico. Presentamos un caso clínico de síndrome de Sweet asociado con una rara neoplasia pancreática, siendo uno de los pocos casos reportados en la literatura médica acerca de esta asociación.

Sweet's syndrome is an uncommon inflammatory dermatosis, which has been associated with malignant tumors, mainly of hematological type. We report a clinical case of Sweet syndrome associated with a rare pancreatic neoplasm, which is one of the few cases reported in the medical literature about this association.

Pancreatic hamartoma in a premature Trisomy 18 female.

Pancreatic hamartomas are extremely rare tumors in adults and even more so in children. They are lesions characterized by acinar, islet and ductal components found in varying proportions and in a disorganized pattern. We report a case of a premature female with trisomy 18 diagnosed by amniocentesis. The newborn was delivered by cesarean section at thirty-three weeks of gestation and expired within one hour of birth. Postmortem examination exhibited numerous features associated with Trisomy 18 including lanugo on the torso and arms, micrognathia, microstomia, left low-set ear with small flat pinna, closed ear canal, clenched fists with overlapping fingers, rocker-bottom feet, narrow pelvis, large right diaphragmatic hernia and left pulmonary hypoplasia. Microscopic examination of the pancreas revealed an area, 1.2 cm in greatest dimension, with branching ducts and cysts lined by cuboidal epithelium intermingled within primitive mesenchymal proliferation and exocrine glands. The cysts measured up to 0.2 cm and were surrounded by a collarette of proliferating spindle cells as highlighted by Masson's trichrome stain. A diagnosis of pancreatic hamartoma was rendered. A total of thirty-four cases of pancreatic hamartomas have been reported in the literature including twenty-seven in adults, five in children and two in newborns. Our case may be the third pancreatic hamartoma reported in association with Trisomy 18. We recommend that careful examination of the pancreas be performed in individuals with Trisomy 18 to further characterize this lesion as one of the possible abnormal findings associated with this syndrome.

Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem.

Intravascular large B-cell lymphoma (IVLBCL) is a very rare extra nodal lymphoma that tends to proliferate within small blood vessels, particularly capillaries and postcapillary venules while sparing the organ parenchyma. The cause of its affinity for the vascular bed remains unknown. Because of its rarity and unremarkable clinical presentation, a timely diagnosis of IVLBCL is very challenging. Here, we describe a case of IVLBCL presenting as pancreatic mass that was ultimately diagnosed at autopsy. A 71-year-old Caucasian female presented with a 3-month history of fatigue, abdominal pain, and weight loss. She was referred to the emergency room with a new diagnosis of portal vein thrombosis and lactic acidosis. During her hospital course she was found to have a 1.9 × 1.8 cm lesion in the pancreatic tail on imaging; The cytologic specimen on the mass showed a high-grade lymphoma. A bone marrow biopsy showed no involvement. The patient's condition rapidly deteriorated and she, later, died due to multi-organ failure. An autopsy revealed diffuse intravascular invasion in multiple organs by the lymphoma cells. Based on our literature review-and to the best of our knowledge-there are virtually no reports describing the presentation of this lymphoma with a discernible tissue mass and associated multi-organ failure. The immunophenotypic studies performed revealed de novo CD5+ intravascular large B-cell lymphoma, which is known to be aggressive with very poor prognosis. Although it is a very rare lymphoma, it should be considered as a potential cause of multi-organ failure when no other cause has been identified. A prompt tissue diagnosis, appropriate high-dose chemotherapy and stem cell transplantation remain the only viable alternative to achieve some kind of remission.

Pancreatic hamartoma in a premature Trisomy 18 female

Pancreatic hamartomas are extremely rare tumors in adults and even more so in children. They are lesions characterized by acinar, islet and ductal components found in varying proportions and in a disorganized pattern. We report a case of a premature female with trisomy 18 diagnosed by amniocentesis. The newborn was delivered by cesarean section at thirty-three weeks of gestation and expired within one hour of birth. Postmortem examination exhibited numerous features associated with Trisomy 18 including lanugo on the torso and arms, micrognathia, microstomia, left low-set ear with small flat pinna, closed ear canal, clenched fists with overlapping fingers, rocker-bottom feet, narrow pelvis, large right diaphragmatic hernia and left pulmonary hypoplasia. Microscopic examination of the pancreas revealed an area, 1.2 cm in greatest dimension, with branching ducts and cysts lined by cuboidal epithelium intermingled within primitive mesenchymal proliferation and exocrine glands. The cysts measured up to 0.2 cm and were surrounded by a collarette of proliferating spindle cells as highlighted by Masson's trichrome stain. A diagnosis of pancreatic hamartoma was rendered. A total of thirty-four cases of pancreatic hamartomas have been reported in the literature including twenty-seven in adults, five in children and two in newborns. Our case may be the third pancreatic hamartoma reported in association with Trisomy 18. We recommend that careful examination of the pancreas be performed in individuals with Trisomy 18 to further characterize this lesion as one of the possible abnormal findings associated with this syndrome.

Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem

Intravascular large B-cell lymphoma (IVLBCL) is a very rare extra nodal lymphoma that tends to proliferate within small blood vessels, particularly capillaries and postcapillary venules while sparing the organ parenchyma. The cause of its affinity for the vascular bed remains unknown. Because of its rarity and unremarkable clinical presentation, a timely diagnosis of IVLBCL is very challenging. Here, we describe a case of IVLBCL presenting as pancreatic mass that was ultimately diagnosed at autopsy. A 71-year-old Caucasian female presented with a 3-month history of fatigue, abdominal pain, and weight loss. She was referred to the emergency room with a new diagnosis of portal vein thrombosis and lactic acidosis. During her hospital course she was found to have a 1.9 × 1.8 cm lesion in the pancreatic tail on imaging; The cytologic specimen on the mass showed a high-grade lymphoma. A bone marrow biopsy showed no involvement. The patient's condition rapidly deteriorated and she, later, died due to multi-organ failure. An autopsy revealed diffuse intravascular invasion in multiple organs by the lymphoma cells. Based on our literature review­and to the best of our knowledge­there are virtually no reports describing the presentation of this lymphoma with a discernible tissue mass and associated multi-organ failure. The immunophenotypic studies performed revealed de novo CD5+ intravascular large B-cell lymphoma, which is known to be aggressive with very poor prognosis. Although it is a very rare lymphoma, it should be considered as a potential cause of multi-organ failure when no other cause has been identified. A prompt tissue diagnosis, appropriate high-dose chemotherapy and stem cell transplantation remain the only viable alternative to achieve some kind of remission.

Endocarditis marántica como presentación de cáncer de páncreas: caso clínico / Non-bacterial thrombotic endocarditis: report of one case

Marantic or nonbacterial thrombotic endocarditis is characterized for the presence of vegetations formed by a meshwork of fibrin and other cellular material similar a blood clot, without the presence of microorganisms. It is often related with tumors and chronic inflammatory states. We report a 49 years old female with a history of weight loss and asthenia, presenting with multiple cerebrovascular attacks and fever. Blood cultures were negative and the fever did not subside with antibiotic treatment. Trans esophageal echocardiogram showed a mitral valve vegetation and thickening of the free edge of both leaflets. In search of the etiology of such a case, a primary pancreatic cancer with distant metastases was found. We cannot rule out the differential diagnosis with bacterial endocarditis with negative blood cultures, although the clinical context supports a non-infectious etiology.

Tumor de Frantz. presentacion de 2 casos / Frantz´s tumor. a two cases presentation

La neoplasia sólida pseudopapilar de páncreas es una neoplasia maligna de bajo grado, relativamente poco frecuente, que representa entre 0.9 y 2.7 % de todas las neoplasias malignas pancreáticas. Afecta principalmente mujeres jóvenes (89 % de los casos), con una edad promedio al diagnóstico de 28 años. La mayoría presentan sintomatología inespecífica relacionada a una masa intrabdominal y más de un tercio suelen ser descubiertas incidentalmente. Presentamos dos casos (una paciente de 19 años de edad diagnosticada de una neoplasia localizada en cuerpo de páncreas y una paciente de 28 años con tumor en la cabeza, que correspondían a tumor de Frantz. Analizamos las características biológicas de estos tumores, sus formas de presentación y las pruebas complementarias recomendadas para su diagnóstico, así como una búsqueda en la literatura.

The solid pseudopapillary neoplasm of the pancreas is a low-grade malignancy, relatively rare, representing between 0.9 and 2.7% of all pancreatic malignancies. It mainly affects young women (89% of cases), with a mean age at diagnosis of 28 years. Most have specific symptoms related to intra-abdominal mass and more than one third are usually discovered incidentally. We report two cases ( a 19-years-old woman diagnosed of a neoplasm in the tail and another 28 years old woman , with neoplasm in the head of the pancreas which could be classified as Frantz’s tumor. We analyze the biological characteristics of these tumors, their forms of presentation and the recommended explorations for the diagnostic.

Ultrasonido endoscópico y puncion aspiración con aguja fina en tumores del páncreas: experiencia de 2 años. Servicio oncológico hospitalario del IVSS 2010 / Endoscopic ultrasound and fine needle aspiration puncture in tumors of the pancreas: 2 years experience. Hospital oncology service IVSS 2010

La punción aspiración guiada por ultrasonido endoscópico (PAAF-USE) se ha convertido en una herramienta fundamental en el diagnóstico de los tumores de páncreas. Es un procedimiento con baja tasa de complicaciones que además provee información determinante que puede modificar la conducta terapéutica. Métodos: Estudio retrospectivo, descriptivo. Se incluyeron pacientes con diagnóstico de tumor de páncreas referidos para la realización PAAF-USE en el período comprendido entre 2008-2009. Resultados: 36 pacientes fueron estudiados, 58.30% (n=21) del sexo femenino. Se evidenciaron tumores sólidos en el 80.5%. Según la histología, fueron reportados adenocarcinoma en el 47.05% (n=16), cistoadenoma mucinoso en el 8.88% (n=3), cistoadenocarcinoma en el 5.88% (n=2), linfoma no Hodgkin en el 5.88% (n=2), tumor neuroendocrino (insulinoma) en el 2.94 % (n=1), cistodenoma seroso en el 2.94% (n=1), tumor sólido pseudopapilar en el 2.94% (n=1), tumor metastásico a páncreas en el 2.94% (n=1) y el 17.64% (n=6) fueron reportados negativos para malignidad. No hubo complicaciones asociadas al procedimiento. Conclusiones: Los hallazgos obtenidos por PAAF-USE permiten establecer el diagnóstico en un alto porcentaje de las lesiones tumorales del páncreas, con una baja tasa de complicaciones, permitiendo seleccionar conductas terapéuticas adecuadas.

Endoscopic ultrasound-guided fine-needle-aspiration (EUS-FNA) has become an essential tool for the diagnosis and staging of pancreatic neoplasms. It is a procedure with low complication rates and also provides crucial information which could alter the therapeutic management. Methods: A retrospective analysis of patients referred for USE-FNA with presumed pancreatic neoplasms between January 2008 and December 2009. Results: 36 patients were studied, 58.30% (n = 21) were females. Solid tumors were evident in 80.5%. According to their histology, adenocarcinoma were reported in 47.05% (n = 16), mucinous cystadenoma in 8.88% (n = 3), cystadenocarcinoma in 5.88% (n = 2), non-Hodgkin lymphoma in 5.88% (n = 2), neuroendocrine tumor (insulinoma) in 2.94% (n = 1), serous cystoadenoma in 2.94% (n = 1), solid pseudopapillary tumor in 2.94% (n = 1), metastatic tumor 2.94 % (n = 1) and 17.64% (n = 6) were reported negative for malignancy. There were no complications associated with the procedure. Conclusions: The fi ndings obtained by EUS-FNA make diagnosis possible in a high percentage of tumoral lesions of the pancreas, with no procedural complication rates overall in this study. Consequently, the accurate histological diagnosis will have the potential to affect the selection of an appropriate treatment.

Pancreatectomía central: relato de casos y descripción de la técnica / Central pancreatectomy: cases report and technique description

The conventional techniques of pancreatic resections, most of the time imply in the withdrawal of ex-tensive segments of pancreatic parenchyma, having as possible complications at the long time, the appearance of endocrine or exocrine insufficience. The central pancreatectomy consists in an alternative of pancreatic resection to resect benign or low grade malignant tumours located in the pancreatic isthmus. We present the clinical characteristics, image, and the surgical technique used, for withdraw of pancreatic isthmus nonfunctioning pancreatic neuroendocrine neoplasm and pancreatic serous cystic adenoma.

Las técnicas convencionales de resección pancreática para el tratamiento de enfermedades neoplásicas, en la mayoría de las veces implican la extirpación de segmentos extensos del parénquima pancreático, existiendo como eventuales complicaciones a largo plazo, el surgimiento de insuficiencia pancreática endocrina o exocrina. La pancreatectomía central se constituye en una alternativa de resección pancreática, que limita esta resección solamente al sector comprometido, especialmente en las neoplasias benignas o de bajo grado de malignidad. Presentamos las características clínicas, de imagen y la técnica empleada en dos pacientes, para el tratamiento quirúrgico de un tumor endocrino no funcionante y de un cistoadenoma seroso, localizados en el cuello del páncreas, mediante la pancreatectomía central.

Stent metálico versus plástico en la obstrucción biliar maligna distal / Metal stent versus plastic stent for malignant distal biliary stricture

Antecedentes. En nuestro medio no se conocen estudios que comparen los stents metálicos (SM) y los stents plásticos (SP) en el manejo de la obstrucción biliar maligna distal. Pacientes y métodos. Estudio retrospectivo con 80 pacientes tratados en el Hospital Pablo Tobón Uribe y la Clínica el Rosario entre enero de 2002 y junio de 2008. Cuarenta pacientes se manejaron con SP y otros 40 con SM.Resultados. Se evaluaron 48 (60%) hombres y 32 mujeres (promedio 66±6 años) con obstrucción maligna biliar distal inoperable. El éxito clínico fue igual para ambos procedimientos (92%) La estancia hospitalaria fue significativamente menor para los pacientes con SM que para aquellos con SP (IC 95% 1,6-7,2, p<0,01). La obstrucción del stent biliar recurrió en 19 pacientes de los 40 SP (47,51%) y 13 de 40 (32,5%) con SM (p=0,2) La media de la permeabilidad fue más prolongada en los pacientes con SM que en los plásticos (IC 95% 41,1-90,8, p<0,01) Las complicaciones tempranas se sucedieron en el 5% de los SM y 12,5% de los SP (p=0,4); las complicaciones tardías fueron del 42,5% en los SP y del 30% en los SM (p=0,3) La sobrevida fue de 159,4±9,4 días en los pacientes con SP y de 189,5±7,6 días para los pacientes con SM (p=0,07).Conclusiones. Los stents metálicos y plásticos son efectivos para el tratamiento de la obstrucción maligna distal inoperable, pero los stents metálicos cursan con una mayor permeabilidad, menor estancia hospitalaria y menor número de reintervenciones por complicaciones, pero la sobrevida para ambos es igual.

Background: It is not known in our studies that compare metallic stents (SM) and plastic stents (SP) in the management of distal malignant biliary obstruction. Patients and Methods: Retrospective trial of 80 patients treated in Hospital Pablo Tobón Uribe and Clínica el Rosario between January 2002 and June 2008. Forty patients received a PS and 40 patients were treated with MS. Results. In this study, 48 men (60%) and 32 women (median 66±6 years) with inoperable malignant distal common bile duct strictures were evaluated. Clinical success was equal for two groups (92%). Hospital stay was significantly smaller for patients with MS than the patients with PS (CI 95% 1.6-7.2, p<0.01) Recurrent biliary obstruction was noted in 19 of 40 (47.5%) patients of PS and 27 of 108 (25%) patients of MS. The median first stent patency was longer in the metal group than in the plastic stent group (CI 95% 41.1-90.8, p<0.01). Early complications occurred in 5% of MS and 12.5% of patients with PS (p=0.4), later complications were seen in 42.5% of PS and 30% of MS (p=0.3) Median survival time after stent insertion was 159.4±9.4 days for PS and 189.5±7.6 days for MS (p=0.07).Conclusions. Plastic and metal stents are effective in the treatment of inoperable distal malignant obstruction, but compared with plastic stent, metal stents had better permeability, lower hospital stay, lower procedures for complications and equal survival.

Tumor de Frantz: presentación de un caso / Frantz's tumor: report of a case

El tumor papilar sólido-quístico de páncreas es una neoplasia muy poco frecuente y de origen incierto. Suele afectar a mujeres jóvenes, cursa con escasa sintomatología y es considerado en general como una neoplasia de bajo grado de malignidad. Su tratamiento es quirúrgico, presentando un excelente pronóstico tras resección completa incluso si existen metástasis. Presentamos el caso de una paciente de 14 años de edad diagnosticada de una neoplasia localizada en cola de páncreas que correspondía a un tumor de Frantz. Analizamos las características biológicas y ultraestructurales de estos tumores, sus formas de presentación y las pruebas complementarias recomendadas para su diagnóstico.

Solid-cystic-papillary tumor of pancreas is an uncommon neoplasm and unknown origin. It generally occurs in young women, the symptomatology is limited and is considered a low-grade malignant tumor. Treatment is surgical and the prognosis is usually good after complete resection, even if metastatic dissemination exists. We report a case of a 14-years-old woman diagnosed of a neoplasm in the tail of the pancreas which could be classified as Frantz's tumor. We analyze the biological and ultrastructural characteristics of these tumors, their forms of presentation and the recommended explorations for the diagnostic.