Abdominal plexiform neurofibroma. Case report
Introduction: Plexiform neurofibroma is a subtype of type 1 neurofibromatosis that is characterized by affecting multiple bundles of a nerve, being recurrent, and having a high risk of malignant transformation. Case Report: A 2-year-old female patient, her mother noticed increase in volume in the lo...
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| Format: | Article |
| Language: | Spanish |
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Editorial Ciencias Médicas
2025-01-01
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| Series: | Acta Médica del Centro |
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| Online Access: | https://revactamedicacentro.sld.cu/index.php/amc/article/view/1959 |
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| author | José Rolando Rodríguez Núñez Fernando Karel Fonseca Sosa Juan Guillermo Bauzá López Pedro Rafael Casado Mendez |
| author_facet | José Rolando Rodríguez Núñez Fernando Karel Fonseca Sosa Juan Guillermo Bauzá López Pedro Rafael Casado Mendez |
| author_sort | José Rolando Rodríguez Núñez |
| collection | DOAJ |
| description | Introduction: Plexiform neurofibroma is a subtype of type 1 neurofibromatosis that is characterized by affecting multiple bundles of a nerve, being recurrent, and having a high risk of malignant transformation.
Case Report: A 2-year-old female patient, her mother noticed increase in volume in the lower abdominal region. On physical examination an increase in volume was palpated in the right iliac fossa. The abdominal computed tomography revealed a tumor image of variable density, predominantly hypodense. Ultrasound-guided fine-needle aspiration cytology revealed plexiform neurofibroma. During the intraoperative period, an abdominal tumor adhered to the posterior face of the bladder and the sacrum was identified. The tumor was completely resected. Histopathological examination confirmed the diagnosis. In the postoperative period, the magnetic resonance imaging concluded as a tumor recurrence of possible neurogenic etiology with malignant behavior. It was evaluated in a multidisciplinary consultation and pharmacological treatment was decided.
Conclusions: Abdominal plexiform neurofibroma is a rare condition with a high risk of tumor recurrence and malignant transformation. Although there is no curative treatment for this disease, surgical resection of the tumor together with the use of drugs that are still in the clinical trial phase have managed to reduce tumor volume and improve quality and life expectancy. |
| format | Article |
| id | doaj-art-0908f4bf9ff642378874aa37cd0b04ed |
| institution | Kabale University |
| issn | 2709-7927 |
| language | Spanish |
| publishDate | 2025-01-01 |
| publisher | Editorial Ciencias Médicas |
| record_format | Article |
| series | Acta Médica del Centro |
| spelling | doaj-art-0908f4bf9ff642378874aa37cd0b04ed2025-02-05T02:11:03ZspaEditorial Ciencias MédicasActa Médica del Centro2709-79272025-01-01191e1959e19591280Abdominal plexiform neurofibroma. Case reportJosé Rolando Rodríguez Núñez0Fernando Karel Fonseca Sosa1Juan Guillermo Bauzá López2Pedro Rafael Casado Mendez3Hospital Provincial Pediátrico “Hermanos Cordové”. Universidad de Ciencias Médicas de Granma. Manzanillo. Granma. Cuba.Hospital Provincial Clínico-Quirúrgico Docente “Celia Sánchez Manduley”. Universidad de Ciencias Médicas de Granma. Manzanillo. Granma. Cuba.Hospital Provincial Pediátrico “Hermanos Cordové”. Universidad de Ciencias Médicas de Granma. Manzanillo. Granma. Cuba.Hospital General “Mariano Pérez Bali”. Universidad de Ciencias Médicas de Granma. Manzanillo. Granma. Cuba.Introduction: Plexiform neurofibroma is a subtype of type 1 neurofibromatosis that is characterized by affecting multiple bundles of a nerve, being recurrent, and having a high risk of malignant transformation. Case Report: A 2-year-old female patient, her mother noticed increase in volume in the lower abdominal region. On physical examination an increase in volume was palpated in the right iliac fossa. The abdominal computed tomography revealed a tumor image of variable density, predominantly hypodense. Ultrasound-guided fine-needle aspiration cytology revealed plexiform neurofibroma. During the intraoperative period, an abdominal tumor adhered to the posterior face of the bladder and the sacrum was identified. The tumor was completely resected. Histopathological examination confirmed the diagnosis. In the postoperative period, the magnetic resonance imaging concluded as a tumor recurrence of possible neurogenic etiology with malignant behavior. It was evaluated in a multidisciplinary consultation and pharmacological treatment was decided. Conclusions: Abdominal plexiform neurofibroma is a rare condition with a high risk of tumor recurrence and malignant transformation. Although there is no curative treatment for this disease, surgical resection of the tumor together with the use of drugs that are still in the clinical trial phase have managed to reduce tumor volume and improve quality and life expectancy.https://revactamedicacentro.sld.cu/index.php/amc/article/view/1959neurofibromatosisenfermedad de von recklinghausenneurofibroma plexiforme abdominal. |
| spellingShingle | José Rolando Rodríguez Núñez Fernando Karel Fonseca Sosa Juan Guillermo Bauzá López Pedro Rafael Casado Mendez Abdominal plexiform neurofibroma. Case report Acta Médica del Centro neurofibromatosis enfermedad de von recklinghausen neurofibroma plexiforme abdominal. |
| title | Abdominal plexiform neurofibroma. Case report |
| title_full | Abdominal plexiform neurofibroma. Case report |
| title_fullStr | Abdominal plexiform neurofibroma. Case report |
| title_full_unstemmed | Abdominal plexiform neurofibroma. Case report |
| title_short | Abdominal plexiform neurofibroma. Case report |
| title_sort | abdominal plexiform neurofibroma case report |
| topic | neurofibromatosis enfermedad de von recklinghausen neurofibroma plexiforme abdominal. |
| url | https://revactamedicacentro.sld.cu/index.php/amc/article/view/1959 |
| work_keys_str_mv | AT joserolandorodrigueznunez abdominalplexiformneurofibromacasereport AT fernandokarelfonsecasosa abdominalplexiformneurofibromacasereport AT juanguillermobauzalopez abdominalplexiformneurofibromacasereport AT pedrorafaelcasadomendez abdominalplexiformneurofibromacasereport |