Cerebellar glioblastoma in an NF1 patient. Is it surgical debulking really necessary?

Flower, Hannah; Gallo, Pasquale

doi:10.1080/02688697.2019.1690127

Cited by 6 publications

(5 citation statements)

References 22 publications

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“…[ 4 ] Flower et al reviewed a summary of cerebellar glioblastoma patients with NF1 and reported a case received chemotherapy and radiotherapy only without surgery. [ 5 ] The presented case survived 18 months which was ranked third in the group. In addition, the longest survival was achieved by the other patient who received biopsy instead of surgery.…”

Section: Discussionmentioning

confidence: 94%

“…[ 4 ] Based on the current studies, number of NF1 patients with infratentorial GBM was similar from those patients with supratentorial GBMs. [ 5 , 6 ]…”

Section: Discussionmentioning

confidence: 99%

“…In addition, the longest survival was achieved by the other patient who received biopsy instead of surgery. [ 5 , 7 ] However, for NF1 patients with supratentorial GBM, surgery with concurrent chemoradiotherapy may still be the best treatment option. Most of them who received the treatment option had a longer survival time than the median time of glioblastoma.…”

Section: Discussionmentioning

confidence: 99%

“…Globally, glioblastoma with neurofibromatosis type 1 were a rare disease as the publicly reported cases was approximately 30. [ 5 , 6 , 11 , 14 , 17 – 20 ] After excluding children patients and infratentorial GBM patients, there was about only half cases left. The reported cases presented different characteristics with incomplete data.…”

Section: Discussionmentioning

confidence: 99%

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Glioblastoma in a female neurofibromatosis 1 patient without IDH1, BRAF V600E, and TERT promoter mutations

Cai¹,

Chen

Wu³

et al. 2021

Medicine

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Rationale: Glioblastoma is the most lethal and common malignant brain tumor but rare in patients with neurofibromatosis type 1. The clinical findings and pathological findings with gene signatures in female patients have not been well clarified. Patient concerns: A 51-year-old female patient complained of headache and left limb weakness lasting for 20 days. The patient underwent a cesarean section 20 years ago and hysterectomy 1 year ago because of uterine leiomyomas. Multiple café-au-lait spots and neurofibromas were found over patient's chest, neck, back, and arms. The myodynamia of left distant and proximate epipodite were grade 0 and grade 1 respectively. The myodynamia of lower left limb was grade 3. Diagnoses: Magnetic resonance imaging revealed a malignant lesion which was most likely a glioblastoma in the right temporo-parietal lobe, approximately 5.6 × 5.9 × 6.9 cm in size with a rounded boundary. Interventions: A right temporo-parietal craniotomy was performed to resect the space-occupying lesion for gross total removal. Then, the patient received concurrent chemoradiotherapy. Histological examination confirmed a glioblastoma without v-RAF murine sarcoma viral oncogene homolog B1 gene, isocitrate dehydrogenase 1 gene, and telomerase reverse transcriptase gene promoter mutations. Outcomes: After surgery, the headache was relieved and the muscular strength of left limbs did improve. After receiving the standard treatment regimen, the patient was alive at 13 months follow-up. Lessons: This is the first reported glioblastoma in female neurofibromatosis type 1 patient without v-RAF murine sarcoma viral oncogene homolog B1 gene, isocitrate dehydrogenase 1 gene, and telomerase reverse transcriptase gene promoter mutations. Tumors in adult patients with these signatures were less aggressive with well-circumscribed border and had long-term survivals which strengthened the evidence that these patients may comprise a unique subset in glioblastoma.

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Section: Discussionmentioning

confidence: 94%

“…[ 4 ] Based on the current studies, number of NF1 patients with infratentorial GBM was similar from those patients with supratentorial GBMs. [ 5 , 6 ]…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Glioblastoma in a female neurofibromatosis 1 patient without IDH1, BRAF V600E, and TERT promoter mutations

Cai¹,

Chen

Wu³

et al. 2021

Medicine

View full text Add to dashboard Cite

show abstract

“…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.…”

Section: Nf1-associated Gliomasmentioning

confidence: 99%

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

Basindwah¹,

Alkhalidi²,

Abdel-Warith

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Gliomas are commonly detected in patients with neurofibromatosis type 1 (NF1) at an early age. Few patients with NF1 are diagnosed with glioblastoma. The course of management, response to therapy, and prognosis of such patients are unknown. Few reports have shown longer-than-average survival rates for patients with NF1 with glioblastoma. OBSERVATIONS A 27-year-old man with NF1 presented with symptoms of high intracranial pressure. Imaging and pathology showed left frontotemporal glioblastoma. Gross total resection was achieved, and concurrent chemoradiotherapy was administered. Recurrence of tumor was detected 48 months later, and the patient underwent tumor debulking and concurrent chemoradiotherapy. The patient received first-, second-, and third-line chemotherapy (temozolomide, bevacizumab, bevacizumab/irinotecan) with good tolerance and has survived >10 years since then with good functional status. LESSONS This case demonstrates >10 years overall survival of glioblastoma in a patient with NF1. Reports of patients with NF1 with longer survival may be attributed to the young age at diagnosis and relatively better tolerance for therapy. It might also support the growing evidence of a unique subset of glioblastoma associated with NF1 and opens the door for a more molecular targeted therapy in the future.

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Management, functional outcomes and survival in a French multicentric series of 118 adult patients with cerebellar glioblastoma

Picart

Meyronet

Pallud

et al. 2021

J Cancer Res Clin Oncol

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Cerebellar glioblastoma in an NF1 patient. Is it surgical debulking really necessary?

Cited by 6 publications

References 22 publications

Glioblastoma in a female neurofibromatosis 1 patient without IDH1, BRAF V600E, and TERT promoter mutations

Glioblastoma in a female neurofibromatosis 1 patient without IDH1, BRAF V600E, and TERT promoter mutations

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

Management, functional outcomes and survival in a French multicentric series of 118 adult patients with cerebellar glioblastoma

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