Neurofibromatosis Type 1 with a Giant Diffuse Plexiform Neurofibroma Invading the Liver

Plexiform neurofibromas (PNs) occur in approximately 50% of patients with neurofibromatosis type 1 (NF1). PNs are rare in the abdominal cavity and especially rare in hepatobiliary lesions. A 31-year-old man with NF1 had a tumor extending along the celiac artery, superior mesenteric artery, and intra...

Descripción completa

Detalles Bibliográficos
Autores principales: Yokogawa, Yuko, Suzuki, Tomohiro, Suzuki, Hiroyuki, Nemoto, Rena, Shimizu, Hiroshi, Ueda, Tsuyoshi, Uchihara, Daiki, Okubo, Yoshinori, Ichii, Osamu, Tai, Mayumi, Ejiri, Yutaka, Harada, Masaru
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2023
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10641209/
https://www.ncbi.nlm.nih.gov/pubmed/36792186
http://dx.doi.org/10.2169/internalmedicine.1372-22
Descripción
Sumario:Plexiform neurofibromas (PNs) occur in approximately 50% of patients with neurofibromatosis type 1 (NF1). PNs are rare in the abdominal cavity and especially rare in hepatobiliary lesions. A 31-year-old man with NF1 had a tumor extending along the celiac artery, superior mesenteric artery, and intrahepatic portal vein. We diagnosed him with diffuse PN based on liver tumor biopsy findings and the tumor form. Because the tumor had invaded along the intrahepatic portal vein, surgical resection was deemed difficult, and the patient was followed up with imaging studies. The patient remained asymptomatic without tumor growth.

Ejemplares similares