Glioblastoma in adults with neurofibromatosis type I: A report of two cases

Abstract

Neurofibromatosis type I (NF1) is a familial tumor syndrome with an autosomal‐dominant inheritance. NF1‐associated tumors often include neurofibromas, malignant peripheral nerve sheath tumors and pilocytic astrocytomas of the optic nerve. The presentation of NF1 patients with glioblastoma is a rare occurrence, with only a handful of cases reported in the literature. We report two cases of glioblastomas occurring in adults with NF1 and briefly review the relevant literature.

Ten-year survival in glioblastoma patient with neurofibromatosis type 1: illustrative case

“…Few cases have been reported in the literature. 8,16 In the few reported cases (Table 1), 5,7,[16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] it is noted that the mean age of patients with NF1 with glioblastoma at diagnosis is much younger (mean age 34 years) than that of patients with sporadic glioblastoma (mean age 55 years). 31 In a systematic review of patients without NF1 with glioblastoma and >10-year survival, there was an inverse relationship between age at diagnosis and years of survival, where younger age at diagnosis by 4.7 years resulted in 1 year longer overall survival after 10 years of survival.…”

An Update on Neurofibromatosis Type 1-Associated Gliomas

“…There are limited data available on glioblastomas in individuals with NF1, with few cases reported in the literature [82][83][84][85]. Although it is challenging to draw a conclusion based on few case reports, the observed age of occurrence of NF1-associated glioblastoma in adults, mean of 38.3 years, is much younger than the mean age for patients with sporadic glioblastoma (Figure 2) [82]. Indeed, there is growing evidence that NF1-associated glioblastoma may comprise a unique subset of glioblastoma IDH wild type.…”