The genetic profile of a neurofibromatosis type 1 Vietnamese patient with complicated pheochromocytomas and colorectal tumors

Neurofibromatosis type 1 (NF1) is a hereditary, autosomal dominant condition marked by the development of tumors along the nervous system due to uncontrolled cell proliferation. The current case reports a 31-year-old male patient diagnosed with NF1 with the involvement of bilateral pheochromocytomas...

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Bibliographic Details
Main Authors: Ha Thanh Tran Le, Hai Thi Tran, Linh Khanh Nguyen, Lap Tien Doan, Quyet Ngoc Kieu La, Thu Hong Nguyen-Huu, Ha Hai Nguyen
Format: Article
Language:English
Published: Open Exploration Publishing Inc. 2025-07-01
Series:Exploration of Neuroscience
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Online Access:https://www.explorationpub.com/uploads/Article/A1006102/1006102.pdf
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Summary:Neurofibromatosis type 1 (NF1) is a hereditary, autosomal dominant condition marked by the development of tumors along the nervous system due to uncontrolled cell proliferation. The current case reports a 31-year-old male patient diagnosed with NF1 with the involvement of bilateral pheochromocytomas and colonic inflammatory polyps/leiomyoma. A genetic profile was explored through whole-exome sequencing to identify pathogenic variants, and segregation analysis was subsequently performed in the patient’s family. Sequencing analysis revealed a novel heterozygous frameshift variant, NF1 c.7301dupA (p.S2435Efs*11), which was identified as the pathogenic variant in the patient. Additionally, two identified variants, PMS2 c.2T>C (p.M1T) and MUTYH c.850-2A>G, may be associated with colonic tumor conditions in the patient. These findings provide insights into the molecular etiology underlying this rare presentation of multiple tumors in a Vietnamese male and may contribute to improved treatment planning and patient management.
ISSN:2834-5347