Case Report
Hourglass retroperitoneal ganglioneuroma
Ganglioneuroma retroperitoneal en reloj de arena
Rev Neurol 2002
, 35(7),
650–655;
https://doi.org/10.33588/rn.3507.2002301
Abstract
INTRODUCTION Ganglioneuromas are neuroblastic tumours with a higher degree of histological differentiation and clinical benignity. They are well-defined, encapsulated tumours that are made up of mature ganglionar cells. They are located mainly in the sympathetic ganglia of the mediastinum and the retroperitoneum. In this latter place they can be very large and even penetrate through the intervertebral foramen in the spinal canal in the shape of an hourglass and produce medullar compression. They are tumours that are produced in childhood or infancy.
CASE REPORT In the course of the diagnosis of an acute digestive haemorrhage in a 29-year-old female patient, an abdominal computerised axial tomography (CAT) scan revealed, by chance, an abdominal retroperitoneal tumour that had introduced itself into the spinal canal in the shape of an hourglass. The patient had had lumbar pain that irradiated to the anterior side of the abdomen although up to that moment it was not considered very important. From the CAT scan and magnetic resonance imaging of the lumbar region a large, solid tumour was detected which protruded out in the shape of an hourglass from the vertebral canal at the point between L2 and L3. The patient was submitted to a surgical intervention in two stages and the tumour was removed. Because it was highly vascularised, hours before the intervention an arterial embolization of the tumour was performed in order to limit surgical bleeding.
CONCLUSIONS Due to the unspecific and late symptomatology of ganglioneuromas, we maintained the therapeutical criterion of carrying out a biopsy puncture, as well as a follow-up and control if the histological study did not show up any kind of malignity. Because of the possibility of this kind of tumour becoming malign and the fact that there is little chance of guaranteeing a correct diagnosis by means of biopsy puncture, extirpation should be carried out as completely and early as possible
CASE REPORT In the course of the diagnosis of an acute digestive haemorrhage in a 29-year-old female patient, an abdominal computerised axial tomography (CAT) scan revealed, by chance, an abdominal retroperitoneal tumour that had introduced itself into the spinal canal in the shape of an hourglass. The patient had had lumbar pain that irradiated to the anterior side of the abdomen although up to that moment it was not considered very important. From the CAT scan and magnetic resonance imaging of the lumbar region a large, solid tumour was detected which protruded out in the shape of an hourglass from the vertebral canal at the point between L2 and L3. The patient was submitted to a surgical intervention in two stages and the tumour was removed. Because it was highly vascularised, hours before the intervention an arterial embolization of the tumour was performed in order to limit surgical bleeding.
CONCLUSIONS Due to the unspecific and late symptomatology of ganglioneuromas, we maintained the therapeutical criterion of carrying out a biopsy puncture, as well as a follow-up and control if the histological study did not show up any kind of malignity. Because of the possibility of this kind of tumour becoming malign and the fact that there is little chance of guaranteeing a correct diagnosis by means of biopsy puncture, extirpation should be carried out as completely and early as possible
Resumen
Introducción Los ganglioneuromas son los tumores neuroblásticos de mayor diferenciación histológica y benignidad clínica. Son tumores bien limitados, encapsulados, y formados por células ganglionares maduras. Se localizan principalmente en los ganglios simpáticos de mediastino y retroperitoneo. En este último lugar pueden alcanzar un gran tamaño, e incluso penetrar por el agujero de conjunción intervertebral en el canal espinal en forma de reloj de arena y producir compresión medular. Son tumores de la edad infantil y juvenil.
Caso clínico En el curso del diagnóstico de una hemorragia digestiva aguda, en una enferma de 29 años, se practicó una tomografía axial computarizada (TAC) abdominal, y se descubrió casualmente un tumor abdominal retroperitoneal que se introducía en el canal espinal en reloj de arena. Había tenido previamente un dolor lumbar irradiado en la cara anterior del abdomen, sin que hasta entonces se le diera ningún significado. Por la TAC y la resonancia magnética lumbares, se detectó un tumor sólido de grandes proporciones que emergía en forma de reloj de arena del canal vertebral por el agujero de conjunción entre L2 y L3. Se intervino quirúrgicamente en dos tiempos y se extirpó radicalmente. Por su gran vascularización, se practicó horas antes una embolización arterial del tumor para limitar la hemorragia quirúrgica.
Conclusiones Debido a la sintomatología inespecífica y tardía del ganglioneuroma, se ha mantenido el criterio terapéutico de realizar una punción biopsia así como un seguimiento y control si el estudio histológico no evidencia ninguna malignidad. Ante la posibilidad de malignización de estos tumores y la escasa garantía de una punción biopsia en el diagnóstico certero, se deberá realizar la extirpación lo más completa y precoz posible
Caso clínico En el curso del diagnóstico de una hemorragia digestiva aguda, en una enferma de 29 años, se practicó una tomografía axial computarizada (TAC) abdominal, y se descubrió casualmente un tumor abdominal retroperitoneal que se introducía en el canal espinal en reloj de arena. Había tenido previamente un dolor lumbar irradiado en la cara anterior del abdomen, sin que hasta entonces se le diera ningún significado. Por la TAC y la resonancia magnética lumbares, se detectó un tumor sólido de grandes proporciones que emergía en forma de reloj de arena del canal vertebral por el agujero de conjunción entre L2 y L3. Se intervino quirúrgicamente en dos tiempos y se extirpó radicalmente. Por su gran vascularización, se practicó horas antes una embolización arterial del tumor para limitar la hemorragia quirúrgica.
Conclusiones Debido a la sintomatología inespecífica y tardía del ganglioneuroma, se ha mantenido el criterio terapéutico de realizar una punción biopsia así como un seguimiento y control si el estudio histológico no evidencia ninguna malignidad. Ante la posibilidad de malignización de estos tumores y la escasa garantía de una punción biopsia en el diagnóstico certero, se deberá realizar la extirpación lo más completa y precoz posible
Keywords
Hourglass vertebral tumours
Magnetic resonance imaging
Neuroblastic tumours
Retroperitoneal tumours
Palabras Claves
Ganglioneuroma
Resonancia magnética
Tumores neuroblásticos
Tumores retroperitoneales
Tumores vertebrales en reloj de arena