RESUMO

El linfangioma quístico del páncreas (LQP), es un tumor extremadamente raro y representa solo el 1% de los linfangiomas abdominales. El objetivo de este manuscrito fue reportar un caso de LQP intervenido quirúrgicamente; y revisar la evidencia existente respecto de sus características morfológicas, terapéuticas y pronósticas. Mujer de 67 años, con LQP operada en Clínica RedSalud Mayor Temuco en septiembre de 2023. Se verificó un tumor sólido-quístico de 16 cm de diámetro mayor, adherido al páncreas a nivel del cuerpo de la glándula, de 867 gramos de peso, con líquido amarillento en su interior; la que fue extirpada completamente. Después del estudio histológico, se realizaron tinciones inmunohistoquímicas complementarias para CD31, D2-40 y calretina. La paciente tuvo un curso postoperatorio sin incidentes, siendo dado de alta al tercer día postoperatorio. En el control alejado, se encontraba en buenas condiciones generales. El LQP es un tumor muy poco frecuente. Las características clínicas e imágenes de este tipo de lesiones son inespecíficas. Debe considerarse en el diagnóstico diferencial de las lesiones quísticas pancreáticas. La resección quirúrgica completa con márgenes libres es el tratamiento de elección; y el pronóstico del LQP es favorable si se reseca por completo.

SUMMARY: Pancreas cystic lymphangioma (PCL) are extremely rare, accounting for only 1% of abdominal lymphangiomas. The aim of this study was to report a rare case of PCL, who underwent surgery; and review the existing evidence regarding its morphological, therapeutic and prognostic characteristics. 67-year-woman patient with PCL who underwent surgery at Clínica RedSalud Mayor Temuco in September 2023. In this case, a solid-cystic tumor with a major diameter of 16 cm was identified, adhering to the pancreas at the level of the body of the gland, weighing 867 grams and containing yellowish fluid in its interior. The tumor was completely excised. Subsequent to histological examination, additional immunohistochemical staining was performed for CD31, D2-40, and calretinin. The patient experienced an uneventful postoperative course and was discharged on the third postoperative day. During the follow-up, the patient remains in good general condition. LQP is a very rare tumor. Clinical features and images of this type of lesions are nonspecific. It should be considered in the differential diagnosis of pancreatic cystic lesions. Complete surgical resection with free margins is the treatment of choice; and the prognosis of LQP is favorable if it is completely resected.

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Los schwannoma son tumores de tejidos blandos que se originan en las células de Schwann de la vaina nerviosa en los nervios periféricos, craneales o viscerales, en cualquier parte del cuerpo. Se trata de una entidad extremadamente inusual. En el artículo se describe el caso de un paciente de piel negra, 47 años de edad y con antecedentes de salud, quien acudió a consulta de clasificación, refiriendo que desde hacía un mes había comenzado a presentar dificultad para orinar, hasta llegar a la retención aguda de orina. Al examen físico se constató, mediante tacto rectal, esfínter tónico, ampolla rectal vacía, próstata aumentada de tamaño grado IV con consistencia fibromuscular de superficie lisa y móvil; no dolorosa. Tras realizarle estudios imagenológicos y complementarios, recibió tratamiento quirúrgico en el Hospital Provincial Docente Oncológico María Curie, de Camagüey, Cuba. Se le diagnosticó un schwannoma retroperitoneal, confirmado por el informe de Anatomía Patológica. Actualmente la evolución del paciente es favorable, sin tratamiento médico, pero con seguimiento periódico.

Schwannoma are soft tissue tumors that originate in Schwann cells of the nerve sheath in peripheral, cranial or visceral nerves, anywhere in the body. This is an extremely unusual entity. The article describes the case of a 47-years-old black-skinned patient with a history of health who attended a triage consultation, reporting that one month ago he had started having difficulty urinating, leading to acute urinary retention. The physical examination confirmed, through digital rectal examination, a tonic sphincter, an empty rectal ampulla, a grade IV enlarged prostate with a fibromuscular consistency with a smooth and mobile surface; not painful. After carrying out imaging and complementary studies, he received surgical treatment at the María Curie Provincial Oncological Teaching Hospital, in Camagüey, Cuba. He was diagnosed with a retroperitoneal schwannoma, confirmed by the Pathology report. Currently the evolution of the patient is positive, without medical treatment, but with periodic follow-up.

RESUMO

Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications.

RESUMO

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica

Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon

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Retroperitoneal cystic lymphangiomas (RCL) are rare benign tumors of the lymphatic system. They account for less than 1% of all lymphangiomas. Surgical resection is the recommended treatment option; however, obtaining a pre-operative diagnosis is often difficult and, in most cases, the final diagnosis is only possible following histological assessment of the surgical specimen. This report describes a case of RCL in a 58-year-old female cirrhotic patient who presented to our center with dull aching abdominal pain and distension. To our knowledge, this is the first case of a RCL in a cirrhotic patient reported in the literature.

Los linfangiomas quísticos retroperitoneales (LQR) son tumores benignos raros del sistema linfático y representan menos del 1% de todos los linfangiomas. La resección quirúrgica es la opción de tratamiento recomendada; sin embargo, obtener un diagnóstico preoperatorio suele ser difícil y, en la mayoría de los casos, el diagnóstico final solo es posible tras la evaluación histológica de la pieza operatoria. En este estudio, describimos un caso de un LQR en una paciente cirrótica de 58 años que se presentó en nuestro centro con dolor abdominal sordo y distensión. Hasta donde sabemos, este es el primer caso de LQR en un paciente cirrótico reportado en la literatura.

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Los liposarcomas son neoplasias malignas raras que se originan en el tejido adiposo, con mayor frecuencia en el retroperitoneo. De todas las variantes histológicas, el diferenciado es el más común y el de mejor pronóstico. El diagnóstico se establece con pruebas imagenológicas con o sin ayuda de biopsia. El tratamiento consiste en la resección completa del tumor, que puede o no acompañarse de terapia adyuvante. Se presenta el caso de un paciente de 41 años con distensión abdominal y pérdida de peso, en el que se confirma un liposarcoma retroperitoneal mediante una TC abdominal, por lo que fue sometido a una resección quirúrgica completa que resultó exitosa. El paciente se recuperó satisfactoriamente y se mantuvo sin recidivas durante el seguimiento. Se concluye que el diagnóstico y terapéutico varían de acuerdo con las características del tumor y su riesgo de recidiva; por lo que hasta la actualidad continúa siendo un desafío multidisciplinario.

SUMMARY Liposarcomas are rare malignant neoplasms that originate in adipose tissue, most frequently in the retroperitoneum. Of all histological variants, differentiated liposarcoma is the most common and has the best prognosis. Diagnosis is established by imaging tests with or without the aid of biopsy. Treatment consists of complete resection of the tumour, which may or may not be accompanied by adjuvant therapy. We present the case of a 41-year-old patient with abdominal distension and weight loss, who was confirmed to have retroperitoneal liposarcoma by abdominal CT scan and underwent successful complete surgical resection. The patient recovered satisfactorily and remained free of recurrence during follow-up. It is concluded that diagnosis and therapy vary according to the characteristics of the tumour and its risk of recurrence, which is why it remains a multidisciplinary challenge to this day.

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Introducción: Los tumores de la glándula suprarrenal son inusuales y por lo general son hallados de forma incidental por estudios de imágenes. Dentro de este grupo los mielolipomas son uno de los tumores más raros, considerados el 2% de los tumores suprarrenales. Caso Clínico: Presentamos una paciente femenina de 60 años de edad con antecedentes de dolor a tipo cólico de forma esporádica a nivel del hipocondrio derecho. La ecografía abdominal detectó colelitiasis y una masa sugerente de adenoma suprarrenal izquierdo. La tomografía abdominal corroboró el tumor suprarrenal gigante y la litiasis vesicular. Se realizó suprarrenalectomía y colecistectomía convencional sin complicaciones. El diagnóstico histopatológico mostró un mielolipoma suprarrenal y una colecistitis crónica. Discusión: El mielolipoma suprarrenal es infrecuente, la etiología se desconoce, por lo general es asintomático y su hallazgo es incidental, habitualmente son unilaterales, menores a 4cm y la incidencia aumenta con la edad. Conclusiones: Cuando los mielolipomas alcanzan dimensiones mayores de 10cm se recomienda realizar una suprarrenalectomía convencional.

Introduction: Adrenal gland tumors are unusual and are usually found incidentally by imaging studies. Within this group, myelolipomas are one of the rarest tumors, considered 2% of adrenal tumors. Clinical case: We present a 60-year-old female patient with a history of sporadically colicky pain at the level of the right hypochondrium. Abdominal ultrasound revealed cholelithiasis and a mass suggestive of a left adrenal adenoma. Abdominal tomography confirmed a giant adrenal tumor and gallstones. An adrenalectomy and conventional cholecystectomy were performed without complications. The histopathological diagnosis showed an adrenal myelolipoma and chronic cholecystitis. Discussion: Adrenal myelolipoma is infrequent, the etiology is unknown, it is usually asymptomatic and its finding is incidental, they are usually unilateral, smaller than 4cm and the incidence increases with age. Conclusions: When myelolipomas reach dimensions greater than 10cm, conventional adrenalectomy is recommended. In selected cases.

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Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an al- tered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical mani- festations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pedia- tric age is uncommon.

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica.

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OBJECTIVES: Retroperitoneal tumor resection commonly disturbs major vessels; therefore, surgical teams can recruit vascular surgeons to prevent injuries and improve the prognosis of oncologic patients. The objective of the present study is to establish long-term survival after retroperitoneal tumor resection surgery with an emphasis on the potential impact of preventing or repairing major vessel injuries when tumors are adjacent to the aorta or vena cava. METHODS: Retrospective case series including all cases of surgical removal of retroperitoneal tumors between 2007 and 2020 in a highly specialized hospital in Mexico City. Long-term survival was defined as 5 years after surgical intervention. Descriptive statistics, group-comparison tests, and regression analysis were performed using Stata 16. RESULTS: From a total of 70 cases, vascular injury occurred in 30 (42.8%) and the vascular surgeon intervened in 19 (27.1%) of them, 4 (21%) were performed by a vascular surgeon with planned intervention, and in 9 (47.3%) cases the vascular surgeon was called to join the surgery due to emergency. Intraoperative bleeding was 2-fold greater in the group with an emergent participation of vascular surgery in contrast with the planned intervention group (4, 235 mL vs 2, 035 mL, p = 0.04). The regression model revealed a significant association between the intervention of a vascular surgeon and long-term survival (OR 59.3, p = 0.03) after adjusting for sociodemographic and characteristics of oncologic nature. CONCLUSIONS: Planned intervention of vascular surgeons in retroperitoneal tumor resection may have a positive impact not only in trans-operatory period, but also on long-term survival.

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Resumo O leiomiossarcoma de veia cava inferior (LVCI) é um raro tumor maligno mesenquimal. Seu tratamento cirúrgico é um desafio, pois necessita combinar margens cirúrgicas livres com reconstrução vascular, usando prótese ou enxerto autólogo, sutura primária ou ligadura simples sem reconstrução da veia. A ligadura é possível graças ao lento crescimento do tumor, permitindo o desenvolvimento de circulação venosa colateral. Apresentamos um caso de LVCI tratado por ressecção radical sem reconstrução vascular. Paciente feminina, 48 anos, com dor abdominal em hipocôndrio direito, astenia e sintomas dispépticos pós-prandiais. Tomografia de abdome revelou massa de formação expansiva localizada no segmento infra-hepático da veia cava inferior com redução da luz do vaso. Na cirurgia, o clampeamento da veia não indicou repercussões hemodinâmicas, sugerindo formação de circulação colateral suficiente. Decidiu-se pela ressecção radical em toda a porção da veia cava retro-hepática e ligadura da veia cava sem reconstrução vascular. A paciente evoluiu sem intercorrências.

Abstract Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications.