Gastrointestinal Stromal Tumor in a Patient with Neurofibromatosis Type 1 That Was Successfully Treated with Regorafenib

An unconscious 55-year-old man with a history of neurofibromatosis type 1 (NF1) was transported to the emergency department and was diagnosed with acute renal failure owing to a large bladder tumor. A submucosal tumor was also identified in the duodenum. The patient was diagnosed with a primary gast...

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Detalles Bibliográficos
Autores principales: Fujimi, Akihito, Nagamachi, Yasuhiro, Yamauchi, Naofumi, Tamura, Fumito, Kimura, Tomohiro, Miyajima, Naoya, Inomata, Hidetoshi, Nishisato, Takuji, Yoshida, Makoto, Takada, Kohichi, Kobayashi, Ko, Kato, Junji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6663552/
https://www.ncbi.nlm.nih.gov/pubmed/30918185
http://dx.doi.org/10.2169/internalmedicine.2321-18
Descripción
Sumario:An unconscious 55-year-old man with a history of neurofibromatosis type 1 (NF1) was transported to the emergency department and was diagnosed with acute renal failure owing to a large bladder tumor. A submucosal tumor was also identified in the duodenum. The patient was diagnosed with a primary gastrointestinal stromal tumor (GIST) of the bladder and duodenum. After six cycles of regorafenib therapy, (18)F-fluorodeoxyglucose accumulation in the duodenal GIST on positron emission tomography-computed tomography (PET-CT) showed a partial metabolic response. Currently, no standard drug therapy for unresectable or relapsed NF1-associated GIST has been established. Regorafenib may thus be considered as and appropriate initial therapy.

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