Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas / Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case

Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

Páncreas ectópico en antro gástrico asociado a síntomas de reflujo. Reporte de un caso / Ectopic pancreas in gastric antrum associated to reflux symptoms. Report of a case

RESUMEN El páncreas ectópico es una entidad poco común. Como tumor submucoso de origen congénito, frecuentemente presenta un curso asintomático, aunque con posibles complicaciones. Su diagnóstico de certeza se basa en la endoscopia, el ultrasonido endoscópico y la histología, que permiten adoptar una conducta expectante o quirúrgica. El paciente estudiado presentó un páncreas ectópico localizado en antro gástrico asociado a síntomas de reflujo gastroesofágico rebeldes a tratamiento, los cuales motivaron el estudio endoscópico, con el consecuente hallazgo de dicha entidad (AU).

ABSTRACT Ectopic pancreas is a little common entity. As congenital-originated sub mucous tumor, it frequently presents an asymptomatic course, though with possible complications. Its definitive diagnosis is based in the endoscopy, endoscopic ultrasound and histology, allowing to adopt an expectant or surgical behavior. The current patient presented an unresponsive-to-treatment ectopic pancreas located in the gastric antrum associated to gastro-esophageal reflux symptoms. This motivated the endoscopic study consequently leading to finding this entity (AU).

Tratamiento quirúrgico paliativo de los tumores periampulares, una opción de paliación a largo plazo / Palliative surgical treatment of periampullary tumors, an option of long-term palliation

RESUMEN Introducción: los tumores de la encrucijada duodeno-bilio-pancreática o periampulares corresponden a un grupo heterogéneo de tumores. Se originan dentro de los 2 cm de la papila duodenal mayor. En los tumores irresecables, el tratamiento debe estar dirigido a la paliación más efectiva. El tratamiento quirúrgico paliativo va dirigido a resolver la obstrucción biliar, duodenal y el dolor, con el fin de optimizar la calidad de vida de los pacientes. Objetivo: describir el comportamiento del tratamiento quirúrgico paliativo de los tumores periampulares. Materiales y métodos: se realizó una investigación observacional, descriptiva y prospectiva con los pacientes con tumor periampular irresecable tributarios a tratamiento quirúrgico paliativo, en el Servicio de Cirugía General del Hospital Universitario "Comandante Faustino Pérez Hernández", en la ciudad de Matanzas, desde enero del 2010 hasta diciembre del 2019. Resultados: el tumor de páncreas fue el más representado. Todos los pacientes fueron tributarios de derivación biliar quirúrgica paliativa, sin embargo, la derivación gástrica se realizó solo con confirmación endoscópica de infiltración tumoral u obstrucción duodenal y la esplacnicectomía química, siempre que fue factible técnicamente o las condiciones del paciente lo permitieron. La hepaticoyeyunostomía en Y de ROUX fue la derivación biliar de elección. La principal complicación quirúrgica fue la sepsis provocando las muertes. Conclusiones: la paliación quirúrgica es la alternativa de elección con mejores resultados a largo plazo, en los tumores periampulares irresecables con buen estado general, lo que contribuye a una mejor calidad de vida (AU).

ABSTRACT Introduction: the tumors of the duodenal-biliary-pancreatic junction or periampullary tumors correspond to a heterogeneous group of tumor. They originate inside the 2 cm of the major duodenal papilla. In unresectable tumors, the treatment should be intended for the most effective palliation. The surgical palliative treatment is intended for solving biliary, duodenal obstruction, and pain, with the aim of optimizing patients' life quality. Objective: to describe the behavior of the periampullary tumors palliative surgical treatment. Materials and methods: a prospective, descriptive, observational research was carried out in patients with unresectable periampullary tumor tributary to palliative surgical treatment, in the Service of General Surgery of the University Hospital "Comandante Faustino Perez Hernandez", of Matanzas, from January 2010 to December 2019. Results: pancreas tumor was the most represented one. All patients were tributary to biliary palliative surgical derivation, however, gastric derivation was performed only with endoscopic confirmation of tumor infiltration or duodenal obstruction, and chemical splanchnicectomy whenever it was technically feasible and the patient's conditions allowed it. Roux's Y-shaped hepaticojejunostomy was the elective biliary derivation. The main surgical complication was sepsis provoking deaths. Conclusions: surgical palliation is the election alternative with long- term better outcomes, in unresectable periampullary tumors with a good general status, contributing to better life quality (AU).

Insulinoma simulando una epilepsia: reporte de caso / Insulinoma simulating epilepsy: case report

Insulinoma es un tumor neuroendocrino que surge de las células ß del páncreas y produce hiperinsulinemia endógena. Son neoplasias raras con una incidencia reportada de 4 casos por millón de habitantes por año. La presentación clínica típicamente cursa con síntomas adrenérgicos y neuroglucopénicos secundarios a hipoglicemia. Requiere estudios de niveles séricos de insulina, pro-insulina y péptido C, además de imágenes diagnosticas que confirmen los hallazgos. La mayoría de los insulinomas son benignos, su sitio primario más común es el páncreas y pueden extirparse quirúrgicamente. Se presenta el caso de un hombre de 36 años con déficit cognitivo leve y episodios de diaforesis con deterioro neurológico hasta convulsiones tónico clónicas generalizadas que curiosamente resolvían con uso doméstico de "panela molida". Se ingresó a urgencias por ataques recurrentes de hipoglicemia severa con requerimiento de altas dosis de dextrosa al 50% por acceso central, hasta confirmación diagnóstica, intervención y resección de tumor neuroendocrino pancreático bien diferenciado (G1 según clasificación OMS) tipo insulinoma en la cola del páncreas.

Insulinoma is a neuroendocrine tumor that arises from the ß cells of the pancreas and produces endogenous hyperinsulinemia. They are rare neoplasms with a reported incidence to 4 cases per million inhabitants per year. The clinical presentation typically presents with adrenergic and neuroglycopenic symptoms secondary to hypoglycemia. It requires studies of serum levels of insulin, pro-insulin and C-peptide, in addition to diagnostic images that confirm the findings. Most insulinomas are benign, their most common primary site is the pancreas, and they can be removed surgically. We present the case of a 36-year-old man with mild cognitive deficits and episodes of diaphoresis with neurological deterioration to generalized clonic tonic seizures that curiously resolved with domestic use of "ground brown sugar". He was admitted to the emergency department due to recurrent attacks of severe hypoglycemia with a high-dose requirement for 50% dextrose through central access, until diagnostic confirmation, intervention, and resection of a welldifferentiated pancreatic neuroendocrine tumor (G1 according to WHO classification) insulinoma in the tail of pancreas.

Hipoglucemia tumoral dependiente y no dependiente de células ß-pancreáticas, un reto diagnóstico / Tumor and non-tumor mediated hypoglycemia by pancreatic beta cells, a diagnostic challenge

La hipoglucemia es una urgencia médica frecuente que en la mayoría de los casos es secundaria al uso de fármacos hipoglucemiantes, orales o inyectados, indicados en pacientes con diabetes mellitus. No obstante, puede presentarse en forma espontánea y severa relacionándose con múltiples condiciones clínicas, incluyendo las neoplasias. Ante una hipoglucemia de origen paraneoplásico se deben reconocer los mecanismos fisiopatológicos que la generan y establecer el diagnóstico oportuno y preciso para disminuir las complicaciones propias de este síndrome clínico. Presentamos dos pacientes con cuadro de hipoglucemia refractaria al manejo médico inicial, de aparición similar con patologías diferentes. El primer caso corresponde a un paciente con insulinoma y el segundo con un hemangiopericitoma.

Hypoglycemia is a common medical emergency which is mostly secondary to the use of oral or injected hypoglycemic drugs indicated in patients with diabetes mellitus. However, it can present spontaneously and severely in relation to multiple clinical conditions, including neoplasms. When faced with hypoglycemia associated with paraneoplastic disorders, the pathophysiological mechanisms of hypoglycemia must be recognized and a timely and accurate diagnosis must be established in order to diminish complications inherent to this clinical syndrome. We herein present two patients with hypoglycemia refractory to initial medical management, sharing similar appearance with other pathologies. The first case corresponds to a patient with an insulinoma and the second to a patient with a hemangiopericytoma.

Cholestatic syndrome as initial manifestation of pancreatic metastasis of papillary thyroid carcinoma: case report and review

SUMMARY Most papillary thyroid carcinomas (PTC) harbor excellent prognosis. Although rare, distant metastases normally occur in lungs and/or bones. Here we describe a rare case of pancreatic metastasis presenting with rapid onset cholestatic syndrome. A literature review was also performed. A 73-year-old man with a high risk PTC was submitted to total thyroidectomy (TT) followed by radioiodine therapy. After initial therapy, he persisted with progressive rising serum thyroglobulin levels but with no evidence of structural disease. Recently, the patient presented with a rapid onset and progressive cholestatic syndrome. A 4 cm lesion in pancreas was identified, with echoendoscopy fine-needle aspiration biopsy (FNAB) confirming a pancreatic metastasis from PTC. The patient was submitted to a successful pancreaticoduodenectomy. Pancreatic metastases of PTC are rare and few long-term follow-up data are available to guide management. Fourteen cases were former reported, mean age was 65.7 years-old with mean time between PTC and pancreatic metastasis diagnosis of 7.9 years. Nine of them had another distant metastasis, nine were diagnosed by FNAB and just two received sorafenib.

Efectos de la curva de aprendizaje en los resultados de la duodenopancreatectomía cefálica en un hospital de nivel II / Effects of the learning curve on the results of cephalic pancreaticoduodenectomy in a level II hospital

Resumen Introducción: La duodeno pancreatectomía cefálica es una operación compleja cuyos resultados a corto plazo son multifactoriales. Objetivo: Evaluar el impacto de la curva de aprendizaje en los resultados a corto plazo de la duodenopancreatectomía cefálica en un hospital de nivel II. Materiales y Método: Se analizaron los datos obtenidos a partir de una base de datos mantenida prospectivamente desde 2005. Se definieron dos periodos de tiempo: de 2005 a 2011 y de 2012 a 2017. Se compararon la morbilidad, mortalidad y estancia postoperatoria de ambos períodos. Resultados: Durante el período de tiempo estudiado se hicieron 126 duodenopancreatectomías cefálicas, 61 durante la primera etapa y 65 durante la segunda. La tasa de transfusión intraoperatoria se redujo de 33% a 15% (p = 0,011). La tasa de transfusión postoperatoria se redujo de 39 a 23% (p = 0,021). No hubo diferencias significativas con respecto a la incidencia global de complicaciones postoperatorias (59% y 52,3%). La incidencia de abscesos intraabdominales fue significativamente menor en el segundo período (18% y 4,6%, respectivamente; p = 0,038). La tasa de reintervenciones se redujo significativamente, de 22% a 9% (p = 0,049). También se redujo significativamente la tasa de mortalidad, de 6,56% a 0% (p = 0,032). La estancia media postoperatoria disminuyó significativamente en el segundo período, pasando de 19,6 a 15,8 días (p = 0,001), con una mayor proporción de pacientes dados de alta en los 8 primeros días de postoperatorio (11,5% y 38,5%, respectivamente; p = 0,001). Conclusión: La curva de aprendizaje es un factor que permite mejorar los resultados de la duodenopancreatectomía cefálica, en un hospital de nivel II, hasta alcanzar valores similares a los de un hospital de nivel III.

Introduction: The duodenum pancreatectomy cephalic is a complex operation whose short-term results are multifactorial. Aim: To assess the impact of the learning curve on the short-term outcomes of cephalic duodenopancreatectomy at a level II hospital. Materials Method: We analyze the data obtained from a database maintained prospectively since 2005. Two time periods were defined: from 2005 to 2011 and from 2012 to 2017. The morbidity, mortality and postoperative stay of both periods were compared. Results: 126 cephalic duodenopancreatectomies were performed, 61 during the first period and 65 during the second. The intraoperative transfusion rate was reduced from 33% to 15% (p = 0.011). The postoperative transfusion rate was reduced from 39 to 23% (p = 0.021). There were no significant differences with respect to the overall incidence of postoperative complications (59% and 52.3%, respectively). However, the incidence of intra-abdominal abscesses was significantly lower in the second period (18% and 4.6%, respectively, p = 0.038). The rate of reoperations was significantly reduced, from 22% to 9% (p = 0.049). The mortality rate was also significantly reduced, from 6.56% to 0% (p = 0.032). The mean postoperative stay decreased significantly in the second period, from 19.6 to 15.8 days (p = 0.001), with a higher proportion of patients discharged in the first 8 postoperative days (11.5% and 38.5%, respectively, p = 0.001). Conclusion: The learning curve is a factor allows improving the results of cephalic pancreaticoduodenectomy, in a level II hospital, until reaching values similar to those of a level III hospital.

Granuloma anular, pronóstico de diabetes mellitus. A propósito de un caso / Annular granuloma, prognosis of diabetes mellitus. On the purpose of a case

RESUMEN El granuloma anular es una dermatosis de relativa frecuencia en niños, jóvenes y adultos. Está caracterizado por lesiones cutáneas eritemato-pápulo-nodulares, que adoptan una disposición anular. Su etiopatogenia es desconocida, pero con numerosos factores predisponentes, desencadenantes o asociados a ella; como es la diabetes mellitus y/o procesos neoplásicos o paraneoplásicos. Resulta importante el estudio de pacientes con este diagnóstico por su asociación con entidades como las antes mencionadas. Se realizó el reporte de un caso en adulto mayor de 65 años, con diagnóstico de granuloma anular, diabetes mellitus y neoplasia de páncreas.

ABSTRACT Annular granuloma is a dermatosis relatively frequent in children, young and adult people. It is characterized by erythematous-papular-nodular skin lesions adopting annular disposition. Its etio-pathogenesis is unknown, but there are many predisposal, unleashing factors, or associated to this disease, like diabetes mellitus and/or neoplastic or paraneoplastic processes. It is important to study the patients diagnosed with the disease due to its association with entities like those before mentioned. The reported case is the case of a patient elder than 65 years, diagnosed with annular granuloma, diabetes mellitus and pancreas neoplasia.

Difficultés diagnostiques et modalités thérapeutiques d'une rupture canalaire traumatique isolée du pancréas

Introduction : Les traumatismes du pancréas sont plus fréquents. Ils rentrent souvent dans un contexte de polytraumatisme, les traumatismes isolés étant beaucoup plus rares. La présentation clinique est variée, à l'origine de retard de consultation. La tomodensitométrie abdominale reste l'examen diagnostique de référence. La chirurgie intègre l'arsenal thérapeutique avec de nombreuses options. Les auteurs se proposent de rapporter ce cas opéré au CHU de Libreville afin de relever les difficultés diagnostiques et les modalités thérapeutiques de cette entité rare. Observation : Mr SM, 37 ans, consultait pour un traumatisme abdominal post-rixe. L'examen physique notait un état de choc, une sensibilité diffuse abdominale, sans défense ni contracture. La TDM abdominale objectivait un hématome rétro-péritonéal et un épanchement péritonéal associé. L'amylasémie était à 660 UI/mL, associée à une anémie normochrome normocytaire à 9 g/dL. L'exploration chirurgicale révélait un hématome de la tête du pancréas et du deuxième duodénum. L'évacuation de l'hématome avec pose d'un drain étaient réalisés. Les suites post-opératoires étaient compliquées d'une fistule pancréatique abondante. La seconde laparotomie objectivait une fistule pancréatique avec rupture canalaire à la jonction tête-corps. Une duodéno-pancréatectomie céphalique selon Whipple était réalisée. Le patient décédait à J3 post-opératoire. Conclusion : Les traumatismes pancréatiques isolés sont rares. Leur gravité est matérialisée par l'atteinte canalaire. Le diagnostic repose sur la TDM abdominale qui doit être est systématique en cas de traumatisme abdominal et la CPRE. La DPC est une option chirurgicale avec des complications lourdes

Cáncer de páncreas en edad pediátrica / Pancreatic cancer in pediatric age

Introducción: Los tumores malignos pancreáticos en pediatría son extremadamente infrecuentes. La sobrevida en el cáncer pancreático a cinco años es baja. Objetivo: Informar a la comunidad médica acerca de una variante poco frecuente de tumor maligno pancreático en edad pediátrica. Presentación del caso: Paciente masculino de 17 años de edad, de la raza negra, que asiste a consulta en julio de 2017 por dolor en hemiabdomen superior, se considera una gastritis y se medica con dieta y antiácidos. Posteriormente comienza con dolor abdominal recurrente, pérdida de peso, anorexia, dispepsias, ictericia en piel y mucosas. Acude al gastroenterólogo quien indica una endoscopia digestiva alta y realiza el diagnóstico del tumor mediante biopsia endoscópica transduodenal. Se opera y reseca gran tumor de cabeza del páncreas junto con primera, segunda y tercera porción del duodeno (pancreatoduodenectomía). El tumor en conjunto midió aproximadamente 15 X 20 cm de diámetro y fue una cirugía completa sin lesión microscópica residual. El resultado de la biopsia indicó que se trataba de un adenocarcinoma acinar del páncreas pobremente diferenciado. Conclusión: Existen pocos casos publicados en la edad pediátrica con esta variante de tumor pancreático. Se documenta la importancia de la cirugía en la cura de la enfermedad(AU)

Introduction: Pancreatic malignancies in pediatrics are extremely infrequent, among them ductal adenocarcinoma and acinar adenocarcinoma. Survival in pancreatic cancer at five years is low. Objective: To inform the medical community about an uncommon variant of pancreatic malignant tumor in pediatric age. Case presentation: Male patient of 17 yesar of age, of the black race, who attended consultation in July of 2017 for pain in upper abdomen, is considered a gastritis and is medicated with diet and antacids. Subsequently begins with recurrent abdominal pain, weight loss, anorexia, dyspepsia, and skin and mucous. Go to the gastroenterologist who indicates an upper gastrointestinal endoscopy and perform the diagnosis of the tumor by transduodenal endoscopic biopsy. A large head tumor of the pancreas is operated on and resected together with the first, second and third portion of the duodenum (pancreatoduodenectomy). The tumor as a whole measured approximately 15 X 20 cm in diameter and was a complete surgery without residual microscopic lesion. The result of the biopsy indicated that it was an acinar adenocarcinoma of the poorly differentiated pancreas. Conclusion: There are few cases published in the pediatric age with this variant of pancreatic tumor. The importance of surgery in the cure of the disease is documented(AU)

Insulinoma descubierto en paciente con aparente trastorno mental: reporte de un caso / Insulinoma found in patient with apparent mental disorder: a case report

El Insulinoma pancreático es un tumor neuroendocrino de las células beta de los islotes de Langerhans, tiene una incidencia de 4 casos por 1 000 000 habitantes. Se reporta el caso de una mujer de 36 años con alteración del comportamiento asociado a melancolía. Psiquiatría describe un examen mental anormal y diagnosticó depresión doble; sin embargo, la paciente tuvo la triada de Whipple y razón insulina/glucosa mayor a 0,3. El insulinoma fue localizado mediante resonancia magnética y tratado con cirugía. Los insulinomas pueden simular trastornos mentales, dificultando un diagnóstico temprano. Se compara los datos de la atención médica en nuestra localidad con otros obtenidos de la literatura a nivel mundial. Concluimos que a pesar de las limitaciones técnicas se debe incluir al insulinoma en el diagnóstico diferencial de trastornos mentales con hallazgo de hipoglucemia.

Pancreatic Insulinoma is a neuroendocrine tumor of the beta cells of the islets of Langerhans, has an incidence of 4 cases per 1 000 000 persons. We report the case of a 36-year-old woman with altered behavior associated with melancholy. Psychiatry describes an abnormal mental examination and diagnosed double depression; however, the patient had the Whipple triad and insulin/glucose ratio higher than 0.3. The insulinoma was localized by magnetic resonance imaging and treated with surgery. Insulinomas can mimic mental disorders, making early diagnosis difficult. We compare health care data in our locality with others obtained from the global literature. We conclude that despite the technical limitations, insulinoma should be included in the differential diagnosis of mental disorders with hypoglycemia.

Instrumentación anterior y posterior para tratamiento de fractura por Metástasis Vertebral / Anterior and posterior instrumentation for fracture treatment due to Vertebral Metastasis

Las fracturas tumorales en columna vertebral son parte de la etiología de las fracturas patológicas a este nivel, que por edad de presentación, así como la clínica observada puede otorgar directrices del tipo de tumor que afecta al esqueleto axial. Por tanto, resulta importante su identificación, clasificación y terapéutica a seguir, en especial en aquellos casos donde se encuentra afectada la parte neurológica distal. Teniendo en consideración estos aspectos planteamos este caso clínico.

Tumoral fractures in spine are part of the etiology of pathologic fractures in this level, age of presentation and the observed clinical can give guidance on the type of tumor that affects the axial skeleton, it is important to identify, classification and therapeutic to follow, especially those who are affected neurological distal part. Considering this aspect propose clinical case below.

Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells reported in an asymptomatic patient: a rare case and literature review

Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UC-OGC) is a rare and poorly described pancreatic malignancy. It is comprised of mononuclear, pleomorphic, and undifferentiated cells as well as osteoclast-like giant cells (OGC's). It constitutes less than 1% of pancreatic non-endocrine neoplasia and is twice as likely to occur in females as in males. Its histopathologic properties remain poorly understood. It is suspected that UC-OGC is of epithelial origin that can then transition to mesenchymal elements. As part of this study, we describe a case of a malignant pancreatic neoplasm that was discovered in a 69-year old patient as an incidental finding. We also provide an overview of previously published data to highlight UC-OGC's clinical and pathologic features.

Twelve years of experience using cholecystojejunal by-pass for palliative treatment of advanced pancreatic cancer / Derivação colecistojejunal para o tratamento paliativo do câncer de pâncreas avançado

ABSTRACT Background: The cholecistojejunal bypass is an important resource to treat obstructive jaundice due to advanced pancreatic cancer. Aim: To assess the early morbidity and mortality of patients with pancreatic cancer who underwent cholecystojejunal derivation, and to assess the success of this procedure in relieving jaundice. Method: This retrospective study examined the medical records of patients who underwent surgery. They were categorized into early death and non-early death groups according to case outcome. Results: 51.8% of the patients were male and 48.2% were female. The mean age was 62.3 years. Early mortality was 14.5%, and 10.9% of them experienced surgical complications. The cholecystojejunostomy procedure was effective in 97% of cases. There was a tendency of increased survival in women and patients with preoperative serum total bilirubin levels below 15 mg/dl. Conclusion: Cholecystojejunal derivation is a good therapeutic option for relieving jaundice in patients with advanced pancreatic cancer, with acceptable rates of morbidity and mortality.

RESUMO Racional: A derivação colecistojejunal é um importante recurso para o tratamento de pacientes com icterícia obstrutiva secundária ao câncer de pâncreas avançado. Objetivo: Avaliar a morbimortalidade precoce dos doentes com câncer de pâncreas submetidos à derivação colecistojejunal, assim como avaliar o alivio da icterícia. Método: Estudo retrospectivo de prontuários de pacientes que foram operados. Eles foram categorizados de acordo com a resolução dos casos em: morte precoce e sem morte precoce. Resultados: 51,8% dos pacientes eram homens e 48,2% mulheres. A média etária foi de 62,3 anos. A mortalidade precoce foi de 14,5%. 10,9% evoluíram com complicações cirúrgicas. A colecistojejunostomia foi efetiva em 97% dos casos. Houve tendência à maior sobrevida em mulheres e pacientes com níveis séricos de bilirrubina total pré-operatório menor do que 15mg/dl. Conclusão: A derivação colecistojejunal constitui boa opção terapêutica para alívio da icterícia em pacientes com câncer de pâncreas avançado, apresentando morbimortalidade aceitável.

Use of cinacalcet and sunitinib to treat hypercalcaemia due to a pancreatic neuroendocrine tumor

SUMMARY Neuroendocrine tumors (NETs) can secrete hormones, including ectopic secretions, but they have been rarely associated with malignant hypercalcemia. A 52-year-old man with a history of diabetes mellitus was diagnosed with a pancreatic tumor. A pancreatic biopsy confirmed a well-differentiated pancreatic NET (pNET). The patient subsequently developed liver metastasis and hypercalcemia with high 1,25 OH vitamin D and suppressed parathyroid hormone (PTH) levels. Hypercalcemia was refractory to chemotherapy, intravenous saline fluids, diuretics, calcitonin and zoledronate. Cinacalcet administration (120 mg/day) resulted in a significant calcium reduction. Hypocalcemia was observed when sunitinib was added three months later and cinacalcet was stopped. Subsequently, the calcium and PTH levels normalized. After six months, we observed 20% shrinkage of the pancreatic tumor and necrosis of a liver metastasis. Cinacalcet is an allosteric activator of the calcium receptor agonist, and it is used for severe hypercalcemia in patients with primary (benign and malignant) hyperparathyroidism. In this patient, cinacalcet demonstrated a calcium lowering effect, normalized hypophosphatemia, and improved the clinical condition of the patient. The mechanism through which cinacalcet improved PTH-rp mediated hypercalcemia is still unclear, but studies have suggested that a potential mechanism is the activation of calcitonin secretion. Sunitinib is an oral multi-targeted tyrosine kinase inhibitor used to treat advanced pNETs. The hypocalcemic effects of sunitinib have not been previously described in a patient with pNET. Here, we report for the first time the successful combination of cinacalcet and sunitinib in the treatment of a pNET patient presenting with malignant hypercalcemia.

Hemorragia digestiva alta en paciente con cistoadenoma mucinoso de páncreas / Digestive high hemorrhage in patient with mucinous cystadenoma of pancreas

Objetivo: Describir una rara y grave presentación clínica de una patología de difícil diagnóstico inicial. Materiales y métodos: Se presenta el caso de una mujer de 45 años, que ingresa al servicio por múltiples episodios de melena y hematemesis, que cursa con shock hipovolémico y cuyo diagnóstico se constata en una laparotomía exploradora de urgencia. Resultados: Se realiza pancreatectomía corporocaudal; biopsia sin hallazgos malignos; tumor quístico mucinoso de tipo cistoadenoma mucinoso de páncreas y linfonodos sin tumor maligno. Discusión: El cistoadenoma mucinoso del páncreas constituye el 10% de los quistes pancreáticos; la gran mayoría son asintomáticos o cursan con dolor abdominal. El diagnóstico se realiza fundamentalmente por técnicas de imagen, la tomografía axial computarizada o el ultrasonido diagnóstico. La hemorragia digestiva alta es una rara presentación clínica, de baja sospecha diagnóstica que puede iniciar con importante compromiso hemodinámico. La causa del sangrado se atribuye a que este tumor se comunicaba por el ducto pancreático hacia el duodeno. Conclusión: El tratamiento de elección es la resección quirúrgica. La técnica quirúrgica a emplear está determinada por la localización del tumor y su naturaleza.

Purpose: Describe a rare and severe clinical presentation of a disease difficult initial diagnosis. Materials and methods: For a woman of 45, who entered the service for multiple episodes of melena and hematemesis, hypovolemic shock that causes, whose diagnosis is found in an emergency exploratory laparotomy is presented. Results: Subtotal pancreatectomy body and tail is made. Without biopsy findings malignant mucinous cystic tumor mucinous cystadenoma type of pancreas, lymph nodes malign tumor. Discussion: Mucinous cystadenoma pancreas constitutes 10% of pancreatic cysts, the vast majority are asymptomatic or present with abdominal pain. The diagnosis is made primarily by imaging techniques, computed tomography or ultrasound diagnosis. Upper gastrointestinal bleeding is a rare clinical presentation, diagnosis low suspect may debut with important hemodynamic compromise. The cause of bleeding is attributed to this tumor communicated by the pancreatic duct into the duodenum. Conclusion: The treatment of choice is surgical resection. The surgical technique used is determined by the location of the tumor and its nature.

Glucagonoma syndrome associated with necrolytic migratory erythema / Síndrome do glucagonoma associado a eritema necrolítico migratório

Summary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. Case report: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications. Conclusion: the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy. .

Resumo Introdução: Introdução: o glucagonoma é um tumor neuroendócrino do pâncreas derivado das células alfa das ilhotas de Langerhans. É marcado pela produção tumoral autônoma de glucagon e caracterizado, dentre outros sintomas, por eritema necrolítico migratório (ENM), uma lesão eritematosa circinada com áreas de necrose e descamação. Trata-se de uma doença rara com incidência mundial estimada em 1 caso para cada 20 milhões pessoas. Relato de caso: apresentamos um caso de glucagonoma associado a ENM em um paciente de sexo masculino, 56 anos de idade, com quadro de lesões cutâneas, principalmente em membros inferiores e região inguinal, hiperglicemia e perda ponderal. Biópsias das lesões cutâneas foram realizadas e exames de imagem do abdome evidenciaram uma massa de 10 x 9 cm em região pancreática. O paciente foi submetido à pancreatectomia corpocaudal e esplenectomia total com autoimplante do baço em omento maior. O laudo histopatológico foi de tumor de células alfa pancreáticas. Imuno-histoquímica evidenciou expressão de glucagon e cromogranina A na maioria das células tumorais, compatível com diagnóstico de glucagonoma. O paciente apresentou seguimento de 3 anos em ambulatório sem intercorrências clínicas. Conclusão: o ENM é importante para o reconhecimento clínico do glucagonoma, sendo seu diagnóstico precoce fundamental para uma terapia curativa de sucesso. .

A Case Report: Cavitary Infarction Caused by Pulmonary Tumor Thrombotic Microangiopathy in a Patient with Pancreatic Intraductal Papillary Mucinous Neoplasm

Pulmonary tumor embolism is commonly discovered at autopsy, but is rarely suspected ante-mortem. Microangiopathy is an uncommon and distinct form of simple tumor pulmonary embolism. Here, we present a 52-year-old male with tumor thrombotic microangiopathy and pulmonary infarction, which might have originated from intraductal papillary mucinous tumor of the pancreas. Multiple wedge-shaped consolidations were found initially and aggravated with cavitation. These CT features of pulmonary infarction were pathologically confirmed to result from pulmonary tumor thrombotic microangiopathy.