Presacral ganglioneuroma in an adult. Report of a clinical case
Abstract: Ganglioneuromas are rare tumors of the sympathetic nervous system, developed from cells of the neural crest. They are the most differentiated within the group of peripheral neuroblastic tumors. They occur more frequently in children, and their most common locations are the poster...
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| Formato: | Artículo revista |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2021
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/34994 |
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I10-R327-article-34994 |
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Universidad Nacional de Córdoba |
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I-10 |
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R-327 |
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Revista de la Facultad de Ciencias Médicas de Córdoba |
| format |
Artículo revista |
| topic |
ganglioneuroma sympathetic nervous system S100 proteins ganglioneuroma sistema nervioso simpático proteínas S100 |
| spellingShingle |
ganglioneuroma sympathetic nervous system S100 proteins ganglioneuroma sistema nervioso simpático proteínas S100 Masciarelli , L Falco , F Pérez , C Aguado , G Ferraris , L Storniolo , A Presacral ganglioneuroma in an adult. Report of a clinical case |
| topic_facet |
ganglioneuroma sympathetic nervous system S100 proteins ganglioneuroma sistema nervioso simpático proteínas S100 |
| author |
Masciarelli , L Falco , F Pérez , C Aguado , G Ferraris , L Storniolo , A |
| author_facet |
Masciarelli , L Falco , F Pérez , C Aguado , G Ferraris , L Storniolo , A |
| author_sort |
Masciarelli , L |
| title |
Presacral ganglioneuroma in an adult. Report of a clinical case |
| title_short |
Presacral ganglioneuroma in an adult. Report of a clinical case |
| title_full |
Presacral ganglioneuroma in an adult. Report of a clinical case |
| title_fullStr |
Presacral ganglioneuroma in an adult. Report of a clinical case |
| title_full_unstemmed |
Presacral ganglioneuroma in an adult. Report of a clinical case |
| title_sort |
presacral ganglioneuroma in an adult. report of a clinical case |
| description |
Abstract:
Ganglioneuromas are rare tumors of the sympathetic nervous system, developed from cells of the neural crest. They are the most differentiated within the group of peripheral neuroblastic tumors. They occur more frequently in children, and their most common locations are the posterior mediastinum, retroperitoneum, and adrenal gland. Pelvic ganglioneuromas are very rare, only a few cases have been reported in the world (less than 30). Our objective is to present the case of an adult man with a presacral ganglioneuroma, as this location is extremely infrequent.
We report the case of a 41-year-old patient who consulted at Elpidio Torres Hospital for weight loss of unknown cause over the prior two years, without other accompanying symptoms. Multiple studies were performed, showing a soft tissue mass in front of the sacrum on the abdominal CT scan. These images were complemented with an MRI, which reported a space-occupying lesion adjacent to the sciatic notch, with multilobed edges, heterogeneous and with intense enhancement.
A multidisciplinary surgery was performed, including the General Surgery, Coloproctology and Neurosurgery services. They requested an intraoperative pathological study of the tumor, which was reported as a spindle cell lesion of probable neural lineage. Then, they proceeded to excise approximately 70% of the tumor, with referral of the surgical piece to Pathology for its study.
Macroscopically, the tissue fragments measured overall 7x6x0.6 cm, were whitish and elastic in consistency. Microscopically, the neoplasm consisted of a proliferation of fused cells, with elongated nuclei and ill-defined eosinophilic cytoplasm, arranged in interlocking bundles and fascicles. In addition, the presence of mature ganglion cells was highlighted. Immunohistochemical techniques showed diffuse positivity for S100.
The patient evolved favorably after surgery, without recurrence of the lesion so far.
We conclude that ganglioneuromas should be considered as a differential diagnosis in patients presenting a pelvic mass. Its behavior is benign, being surgical removal the solution in most cases. |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2021 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/34994 |
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I10-R327-article-349942024-04-15T16:19:09Z Presacral ganglioneuroma in an adult. Report of a clinical case Ganglioneuroma presacro en un adulto. Reporte de un caso clínico Masciarelli , L Falco , F Pérez , C Aguado , G Ferraris , L Storniolo , A ganglioneuroma sympathetic nervous system S100 proteins ganglioneuroma sistema nervioso simpático proteínas S100 Abstract: Ganglioneuromas are rare tumors of the sympathetic nervous system, developed from cells of the neural crest. They are the most differentiated within the group of peripheral neuroblastic tumors. They occur more frequently in children, and their most common locations are the posterior mediastinum, retroperitoneum, and adrenal gland. Pelvic ganglioneuromas are very rare, only a few cases have been reported in the world (less than 30). Our objective is to present the case of an adult man with a presacral ganglioneuroma, as this location is extremely infrequent. We report the case of a 41-year-old patient who consulted at Elpidio Torres Hospital for weight loss of unknown cause over the prior two years, without other accompanying symptoms. Multiple studies were performed, showing a soft tissue mass in front of the sacrum on the abdominal CT scan. These images were complemented with an MRI, which reported a space-occupying lesion adjacent to the sciatic notch, with multilobed edges, heterogeneous and with intense enhancement. A multidisciplinary surgery was performed, including the General Surgery, Coloproctology and Neurosurgery services. They requested an intraoperative pathological study of the tumor, which was reported as a spindle cell lesion of probable neural lineage. Then, they proceeded to excise approximately 70% of the tumor, with referral of the surgical piece to Pathology for its study. Macroscopically, the tissue fragments measured overall 7x6x0.6 cm, were whitish and elastic in consistency. Microscopically, the neoplasm consisted of a proliferation of fused cells, with elongated nuclei and ill-defined eosinophilic cytoplasm, arranged in interlocking bundles and fascicles. In addition, the presence of mature ganglion cells was highlighted. Immunohistochemical techniques showed diffuse positivity for S100. The patient evolved favorably after surgery, without recurrence of the lesion so far. We conclude that ganglioneuromas should be considered as a differential diagnosis in patients presenting a pelvic mass. Its behavior is benign, being surgical removal the solution in most cases. Resumen: Los ganglioneuromas son tumores raros del sistema nervioso simpático, que se originan a partir de células de la cresta neural. Son los más diferenciados dentro del grupo de tumores periféricos neuroblásticos. Se presentan con mayor frecuencia en niños, y sus localizaciones más comunes son mediastino posterior, retroperitoneo y glándula suprarrenal. Los ganglioneuromas de localización pélvica son inhabituales, sólo se han reportado pocos casos en el mundo (menos de 30). Nuestro objetivo es presentar el caso de un hombre adulto con un ganglioneuroma presacro, por ser esta localización extremadamente infrecuente. Reportamos el caso de un paciente de 41 años que consultó en el Hospital Elpidio Torres por pérdida de peso de causa desconocida de dos años de evolución, sin otros síntomas acompañantes. Se le realizaron múltiples estudios, evidenciándose en la TAC de abdomen, una masa de partes blandas por delante del sacro. Estas imágenes fueron complementadas con una RMN, que informó una lesión ocupante de espacio adyacente a la escotadura ciática, de bordes multilobulados, heterogénea y con intenso realce. Se le realizó una cirugía multidisciplinaria, incluyendo a los servicios de Cirugía General, Coloproctología y Neurocirugía. Solicitaron un estudio anatomopatológico intraoperatorio del tumor, el cual se informó como lesión fusocelular de probable estirpe neural. Luego, procedieron a la exéresis de aproximadamente el 70% del tumor, con remisión de la pieza quirúrgica a Anatomía Patológica para su estudio. Macroscópicamente, los fragmentos de tejido medían en conjunto 7x6x0, 6 cm, eran blanquecinos y de consistencia elástica. Microscópicamente, la neoplasia estaba conformada por una proliferación de células fusadas, con núcleos elongados y citoplasma eosinófilo mal definido, dispuestas en fascículos y haces entrelazados. Además, se destacaba la presencia de células de tipo ganglionar maduras. Las técnicas de inmunohistoquímica mostraron positividad difusa para S100. El paciente evolucionó favorablemente luego de la cirugía, sin secuelas ni recidiva de la lesión hasta el momento. Concluimos que los ganglioneuromas deben tenerse en cuenta como diagnóstico diferencial en los pacientes que se presentan con una masa pelviana. Su comportamiento es benigno, siendo la extirpación quirúrgica curativa en la mayoría de los casos. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2021-10-12 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion texto texto texto https://revistas.unc.edu.ar/index.php/med/article/view/34994 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 78 No. Suplemento (2021): Suplemento JIC XXII Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 78 Núm. Suplemento (2021): Suplemento JIC XXII Revista da Faculdade de Ciências Médicas de Córdoba; v. 78 n. Suplemento (2021): Suplemento JIC XXII 1853-0605 0014-6722 http://creativecommons.org/licenses/by-nc/4.0 |