Prospective study on stereotactic body radiotherapy for small pancreatic neuroendocrine tumors: tolerance and effectiveness analysis.

INTRODUCTION: Surgery is the standard treatment for pancreatic neuroendocrine tumors (pNETs), obtaining favorable results but associating high morbidity and mortality rates. This study assesses stereotactic body radiation therapy (SBRT) as a radical approach for small (< 2 cm) nonfunctioning pNETs. MATERIALS AND METHODS: From January 2017 to June 2023, 20 patients with small pNETs underwent SBRT in an IRB-approved study. Endpoints included local control, tolerance, progression-free survival, and overall survival (OS). Diagnostic assessments comprised endoscopy, CT scans, OctreScan or PET-Dotatoc, abdominal MRI, and histological confirmatory samples. RESULTS: In a 30-month follow-up of 20 patients (median age 55.5 years), SBRT was well-tolerated with no grade > 2 toxicity. 40% showed morphological response, 55% remained stable. Metabolically, 50% achieved significant improvement. With a median OS of 41.5 months, all patients were alive without local or distant progression or need for surgical resection. CONCLUSION: SBRT is a feasible and well-tolerated approach for small neuroendocrine pancreatic tumors, demonstrating effective local control. Further investigations are vital for validation and extension of these findings.

Solid and papillary neoplasm of the pancreas (SPNP): a case report.

Background: Solid and papillary neoplasm of the pancreas (SPNP) is a rare pancreatic tumor, well known for its predilection for young women and large volume. The tumor has a favorable prognosis and differentiating it from other pancreatic tumors with aggressive behavior is necessary. Case Description: We present the case of a 34-year-old female without relevant background. She presented with abdominal pain and by fine needle biopsy was diagnosed as ductal carcinoma. During the evaluation, an abdominal ultrasound revealed a pancreatic growth that was "bulky, solid, with irregular margins, in homogeneously hypoechoic, with anechoic areas of necrosis, located lateral to the tail of the pancreas and medial to the upper pole of the left kidney and the lower splenic pole". The patient was admitted, and surgery was performed. At the laparotomy, a tumor of 15 cm in diameter was detected. The tumor was located in the tail of the pancreas, was well encapsulated, and of solid consistency. Caudal pancreatectomy with a splenectomy was carried out. The final pathology diagnosis was a SPNP. Conclusions: In the presence of a large abdominal mass of pancreatic relevance, even in older women, the possibility of having an SPNP should always be evaluated. Given the low malignancy potential of this tumor and the excellent prognosis with radical surgical treatment, the preoperative diagnosis should always be particularly accurate. Surgical resection is recommended as the treatment of choice.

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.

Frantz tumor in a 58 year old woman; case report and literature review.

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

18F-FDG PETCT and 68Ga-DOTA PETCT mismatch with in vivo histopathological characterization of low-grade neuroendocrine pancreatic tumor.

BACKGROUND: Pancreatic neuroendocrine tumor (PNET) is a subgroup of neuroendocrine tumor (NET) that has unique biology and natural history. The histological classification has a major role in the management of this pathology, but in recent years Gallium 68 dotatate (68Ga-DOTA) scanning is at the center of a discussion about how these imaging technologies can modify clinical management of neuroendocrine tumors and how their results are correlated to Ki67 index. METHOD: We hereby describe a case of a patient that investigated an unspecific stable pancreatic nodule suspected of high-grade NET after evaluation with 68Ga-DOTATOC positron emission tomography-computed tomography (PETCT) and 18F-Fluorodeoxyglucose (18F-FDG) PETCT. RESULTS: The images corroborate the hypothesis of high-grade NET based on the standard uptake value (SUV) described in both image exams (16.4 in 18FDG PETCT and 9.2 in 68Ga-DOTATOC PETCT). After surgery, the histopathological analyses revealed a localized grade 2 well-differentiated NET, Ki-67 of 4.7, glucose transport proteins 1 (GLUT1) negative by immunohistochemistry, evidencing a rare case of mismatch between the functional image and the in vivo characterization of the neoplasm. CONCLUSION: Functional imaging of neuroendocrine tumors with different modalities of PETCT is a well-described strategy for evaluating PNET and can dictate conducts in some cases. However, histopathological analysis is crucial to confirm the grade and prognosis related to this disease.

Glucagonoma del páncreas: la piel puede llevarnos al diagnóstico / Pancreatic glucagonoma: observing the skin can lead to diagnosis

Resumen Presentamos el caso de una paciente que tenía un tumor del páncreas -denominado glucagonoma- y cuyo diagnóstico se sospechó por las manifestaciones cutáneas, las cuales nos condujeron realizar una tomografía axial computarizada (TAC). En ella se halló una masa. La paciente se remitió a cirugía y presentó una buena evolución.

Abstract This is a case report of a patient with a pancreatic tumor, known as glucagonoma, whose diagnosis was suspected because of skin manifestations which led to performing a CT scan, finding the mass. She underwent surgery with satisfactory results.

[Tumores neuroendocrinos pancreáticos. Nuestra experiencia]. / Pancreatic neuroendocrine tumors. Our experience.

Pancreatic neuroendocrine tumors are rare. It is a heterogeneous group of neoplasms with very different behavior and prognosis. They can appear sporadically or associated with genetic syndromes. They are divided into functioning and non-functioning. A descriptive retrospective study of patients diagnosed with pancreatic neuroendocrine tumor was performed. The incidental diagnosis of these tumors is increasingly. The only curative treatment for these tumors is surgical excision, depending on the location and characteristics of the tumor and the patient. In selected cases a conservative attitude is recommended.

Los tumores neuroendocrinos pancreáticos son poco frecuentes. Es un grupo heterogéneo de neoplasias con comportamiento y pronóstico muy diferentes. Pueden aparecer de manera esporádica o asociados a síndromes genéticos. Se dividen en funcionantes y no funcionantes. Se realizó un estudio retrospectivo descriptivo de los pacientes diagnosticados de tumor neuroendocrino pancreático. Como se refleja en nuestra serie, cada vez es más frecuente el diagnostico incidental de estos tumores. El único tratamiento curativo de estos tumores es la exéresis quirúrgica, según la localización y las características del tumor y del paciente. En casos seleccionados se puede optar por una actitud conservadora.

Surgical Management of Solid Pseudopapillary Tumor of the Pancreas.

BACKGROUND AND OBJECTIVES: Although solid pseudopapillary tumor (SPT) of the pancreas is rare, its diagnosis has increased severalfold in the past decades. We present our experience in the management of SPT, including a patient who experienced tumor rupture during laparoscopy pancreatic resection. METHODS: Data on all patients with SPT who were subjected to surgical treatment were retrospectively obtained. RESULTS: Of 20 patients evaluated, 17 (85%) were females. The mean age was 31 years. Tumor size varied from 2.7 × 1.5 to 13.5 × 10.0 cm, with a mean of 6.4 × 7.6 cm. The most common location was the tail and/or body of the pancreas (14 patients [70%]). Pancreatic tumor resection was performed in 19 patients (50%). The type of resection depended on tumor location and size: distal pancreatectomy (n = 13), pancreatoduodenectomy (n = 5), and central pancreatectomy (n = 1) Pancreatic resection was performed via laparoscopy in 7 patients who underwent distal pancreatectomy. Tumor resection was not performed in only 1 patient (5%), due to invasion of mesenteric vessels and presence of liver metastases. One patient had tumor rupture during laparoscopic resection, with no apparent macroscopic dissemination of the tumor. All 19 patients who underwent SPT resection had no tumor recurrence, including a patient with capsule invasion and another patient with tumor rupture during surgical dissection. The mean follow-up time was 38 months (range, 6-72 months). CONCLUSION: Complete SPT resection is possible in most patients, with a low recurrence rate. Because of its large size, laparoscopic resection of SPT's should be performed only by experienced surgeons to avoid tumor rupture.

Bazo accesorio intrapancreático que imita tumor de cola de páncreas / Intrapancreatic accessory spleen mimicking a tumor of the pancreatic tail

La presencia de bazos accesorios en la cavidad abdominal es relativamente frecuente (10-15% de la población general). De esos, el 1,7 % puede ser de localización intrapancreática. La existencia de un bazo accesorio intrapancreático obliga a hacer el diagnóstico diferencial con tumores sólidos de la cola de páncreas. Presentamos un caso, resuelto mediante pancreatectomia corporocaudal laparoscópica y realizamos una revisión bibliográfica.

Accessory spleens in the abdominal cavity are relatively frequent (10-15% of the general population). Of these, 1.7% may present intrapancreatic localization. An accessory spleen located in the pancreas requires making a differential diagnosis with solid tumors of the tail of the pancreas. We report on a case treated by laparoscopic pancreatectomy.

Gemcitabine: metabolism and molecular mechanisms of action, sensitivity and chemoresistance in pancreatic cancer.

Gemcitabine is the first-line treatment for pancreatic adenocarcinoma, but is increasingly used to treat breast, bladder, and non-small cell lung cancers. Despite such broad use, intrinsic and acquired chemoresistance is common. In general, the underlying mechanisms of chemoresistance are poorly understood. Here, current knowledge of gemcitabine metabolism, mechanisms of action, sensitivity and chemoresistance reported over the past two decades are reviewed; and we also offer new perspectives to improve gemcitabine efficacy with particular reference to the treatment of pancreatic cancer.

Insulinoma, a propósito de dos casos / Insulinoma, a propos of two cases

El Insulinoma es el tumor neuroendocrino pancreático más frecuente. Se manifiesta por signos adrenérgicos y de neuroglucopenia. Su diagnóstico se confirma documentando la existencia de hipoglucemia junto a una inapropiada secreción de insulina. Se desarrollan a partir de las células de los islotes de Langerhans. Tanto los funcionantes como los no funcionantes son embriológica e histológicamente muy similares y sólo difieren en su capacidad para producir o no hormonas. Presentamos 2 casos de insulinomas cuya localización tumoral no fue posible con técnicas de imagen convencionales, pero sí con ultrasonografía transoperatoria. La evolución fue favorable con remisión total de los síntomas(AU)

Insulinoma is the most common neuroendocrine pancreatic tumor. It manifests with adrenergic and neuroglycopenia signs. Its diagnosis is confirmed by documenting the existence of hypoglycemia together with inadequate insulin secretion. They developed from Langerhans islet cells and both functioning and non-functioning ones are embryologically and histologically very similar and just differ in their capacity to produce hormones or not. Here are two insulinoma cases where tumoral location was not possible with the conventional imaging techniques but with transoperative ultrasonography. The progression was favorable with total remission of symptoms(AU)

Tumor sólido pseudopapilar del páncreas: estudio descriptivo de una serie de casos / Solid pseudopapilary tumors of the pancreas: descriptive study of a case series

Background: Rare neoplasm that occurs most often in young women, neoplasm with low degree of malignancy. Aim: to describe the clinical characteristics and postoperative results and long-term in a series of patients. Material and Methods: Descriptive study of patients with confirmed histological diagnosis operated at our hospital between January 2000 and June 2011. Biodemographical, preoperative, clinical, surgical technique and postoperative results were recorded. Results: 11 patients, ten of them female, with an average age of 28 years (14-43 years). The most common symptom was abdominal pain. Nine patients had lesions of the body and tail of pancreas and 2 patients had lesions in head of pancreas. Corporo-caudal pancreatectomia were done in 9 cases (6 laparoscopic surgeries) and 2 pancreatoduodenectomy (1 totally laparoscopic). The average size of tumors was 5.5 cm (2.2-13 cm), the margin was negative in all cases and no positive lymph nodes. Six patients had a complication: 4 pancreatic fistulas (type A) which were controlled with drainage installed during surgery. One patient had postoperative abdominal collection requiring laparoscopic drainage. There was no operative mortality in this series. The average follow-up was 21 months (5-93 months). There was no recurrence or mortality at the end of follow-up. Conclusions: Is an uncommon neoplasm and occurs primarily in young women. The complete surgical resection is the best treatment, and can be done by lapa-roscopy surgery with low morbidity.

Introducción: Es una neoplasia poco frecuente, con bajo grado de malignidad, se presenta principalmente en mujeres jóvenes. El objetivo de este estudio es describir las características clínicas y los resultados postoperatorios a corto y largo plazo de una serie de pacientes intervenidos en nuestro centro. Material y Método: Estudio descriptivo de pacientes con diagnóstico histológico confirmado, operados en nuestro hospital entre enero de 2000 y junio de 2011. Se consignaron variables biodemográficas, estudio preoperatorio, manifestaciones clínicas, técnica quirúrgica y resultados postoperatorios. Resultados: La serie está constituida por 11 pacientes, diez de ellas de sexo femenino y con una edad promedio de 28 años (14-43 años). El síntoma más frecuente fue el dolor abdominal. Nueve pacientes presentaron lesiones córporocaudales del páncreas y 2 tumores de la cabeza del páncreas. Se realizaron 9 pancreatectomías córporocaudales (6 laparoscópicas) y 2 duodenopancreatectomías (1 totalmente laparoscópica). El tamaño promedio de los tumores fue 5,5 cm (2,2-13 cm), el margen fue negativo en todos ellos y no había linfonodos positivos. Seis pacientes presentaron alguna complicación: 4 fístulas pancreáticas (tipo A) que fueron controladas con el drenaje instalado durante la cirugía. Un paciente presentó una colección abdominal postoperatoria que requirió de drenaje laparoscópico. No hubo mortalidad operatoria en esta serie. El seguimiento promedio fue de 21 meses (5-93 meses). No hubo recurrencia ni mortalidad al cierre del seguimiento. Conclusión: El TSP es una neoplasia infrecuente. La resección quirúrgica completa es el tratamiento de elección, puede ser laparoscópica.

Tumor sólido-pseudopapilar del páncreas presentación de 3 casos clínicos / Solid-pseudopapillary tumor of the pancreas presentation of 3 clinical cases

El tumor sólido-pseudopapilar del páncreas (TSPP) es una neoplasia infrecuente (1-2% de los tumores exocrinos), que afecta predominantemente a mujeres jóvenes, con bajo potencial de malignidad (15% da metástasis). Su diagnóstico preoperatorio es difícil, principalmente debido a que a la imagenología no tiene una característica que la pueda diferenciar de otros quistes pancreáticos, por lo que generalmente es un hallazgo histopatológico. El tratamiento quirúrgico presenta buena sobrevida, incluso en presencia de metástasis. Presentamos 3 casos con cuadro clínico, imagenología, tratamiento e histopatología, con el fin de aportar más información, sobre esta infrecuente patología.

The solid-pseudopapillary tumors of the pancreas (TSPP) are a uncommon neoplasm (1-2% of exocrine tumors). Are more frequent in young women and presents a low malignant potential (15% develop metastases). Its preoperative diagnosis is very difficult, because these tumors haven`t radiological features that makes distinguish from the other pancreatic cysts tumors. Usually are a histopathological finding. Surgical therapy provides good survival, even in the presence of metastases. We present 3 cases with clinical, imaging, treatment and histopathology to provide more information about this rare disease.

Treatment of intraductal papillary mucinous neoplasms, neuroendocrine and periampullary pancreatic tumors using robotic surgery: a safe and feasible technique.

In the last few years, robotic surgery has started to take its place in pancreatic surgery. Robotic surgery provides advantages such as enhanced visualisation and freedom of dissection within a confined space and also allows economical surgery. The aim of this study was to evaluate the feasibility, safety and short-term outcome of the robotic approach using the da Vinci robotic system in pancreatic/peripancreatic tumors other than pancreatic carcinomas. Fifteen patients with eight intraductal papillary mucinous neoplasms, four pancreatic neuroendocrine and three periampulary tumors were included in this initial series. Seven left pancreatectomies, five pancreatoduodenectomies and two total pancreatectomies were performed at Albert Einstein Hospital, São Paulo, Brazil. The mean operating room time for all the procedures was 503 min (315-775 min). Blood transfusion was necessary in one patient (3 units). The mean length of stay for all patients was 16 days (5-52 days). Large series of robotic pancreatic surgery should be described and the decision as to its routine use will come from cumulative experience. This surgical system allows difficult procedures to be performed more easily, effectively and precisely.

Manejo paliativo endoscópico de tumor de páncreas con compromiso biliar y duodenal: reporte de un caso / Palliative endoscopic management of pancreatic tumor with biliary and duodenal involvement: a case report

Las neoplasias de la región periampular engloban una serie de lesiones que por su cercanía se comportan de manera similar. Entre ellas encontramos a las neoplasias malignas de la ampolla de váter, del duodeno, del colédoco y del páncreas, de éstas el 95% son adenocarcinomas. El síndrome ictérico obstructivo es la forma de presentación más frecuente. Otras de las formas de presentación es la obstrucción de tracto de salida gástrico debido a infiltración del duodeno en cerca del cinco 5% de los pacientes. En la actualidad, la única opción curativa para esta enfermedad es la resección quirúrgica: la pancreatoduodenectomía. El tratamiento paliativo de elección, desde la descripción realizada por Soehendra para el manejo de la obstrucción biliar, en la actualidad es el drenaje endoscópico...

Periampullary region neoplasm`s encompasses a series of injuries, because of its proximity they behave similarly. Among them we find malignant neoplasm of the ampulla of Vater, duodenum, bile duct and pancreas; 95% of these are adenocarcinomas. Pancreatic cancer is the most common of these tumors, constituting the fifth leading cause of cancer deaths. The obstructive jaundice syndrome is the most common presentation. Other presentation is the obstruction of gastric outflow due to infiltration of the duodenum in about 5% of patients. Currently, the only curative option for this disease is surgical resection (pancreaticoduodenectomy), the palliative treatment of choice...

Neoplasia papilar cística do pâncreas / Papyllary cystic tumor of the pancreas

Papyllary cystic tumor of the pancreas, so-called Frantz's tumor, is rare. Clinical presentation of this disease is usually a slowly growing abdominal mass with or without abdominal pain, affecting predominantly young females. Its pathogenesis is still unknown . Surgical resection is usually curative, and prognosis is excellent. The authors report two pancreatic tumor cases(Frantz's tumor) in women aged 26 and 31 years old. Pre operative assessment showed a solid-cystic tumor of the tail and body of the pancreas. An extended distal pancreatectomy was performed without splenic preservation.