BAP1 tumour predisposition syndrome: a case report of two siblings with WHO Grade II‐III meningiomas

2021 ◽  
Vol 79 (S1) ◽  
pp. 27-28
Keyword(s):  
2019 ◽  
Vol 7 (2) ◽  
pp. 141
Author(s):  
Moowan Park ◽  
Eun Kyeong Hong ◽  
Sang Hyen Lee ◽  
Ho-Shin Gwak

Author(s):  
Lakshmipriya U. ◽  
Umasankar P. ◽  
Asiq Sideeque ◽  
Aparna Govindan

Chordoid meningioma is a rare tumour accounting for less than 0.5% of all meningiomas. It is a WHO grade II tumour with an aggressive behavior. It is a rare variant characterized by cords or trabeculae of eosinophilic or vacuolated cells set in an abundant mucoid matrix. It can be associated with systemic or hematologic manifestations like Castleman disease. The tumor has a propensity for aggressive behavior and increased likelihood of recurrence We report a case of an adult patient with chordoid meningioma who presented with headache and seizures and did not have any hematological/ systemic manifestations. She underwent total excision of the lesion and is doing well.


2006 ◽  
Vol 52 (1) ◽  
pp. 68-69
Author(s):  
M.O. Pinsker ◽  
R. Buhl ◽  
H.-H. Hugo ◽  
H.M. Mehdorn

2021 ◽  
Vol 11 ◽  
Author(s):  
Weiping Hong ◽  
Changguo Shan ◽  
Minting Ye ◽  
Yanying Yang ◽  
Hui Wang ◽  
...  

BackgroundAlthough surgical resection can cure the majority of meningiomas, there are still approximately 20% of patients suffering from an aggressive course with recurrence or progression. In this study, we reported a novel GNAS mutation and 1p/22q co-deletion responding to sunitinib in a patient with multiple recurrent meningiomas.Case PresentationA 53-year-old woman with meningioma was hospitalized due to postoperative tumor progression for 3 weeks. WHO grade I meningioma was pathologically diagnosed after the first three surgeries, but the second recurrence occurred approximately 3 years following the third surgery. Next-generation sequencing was performed on the first two recurrent samples. GNAS mutations and 1p/22q co-deletion were both identified, and amplification at 17q and chromosome 19 was also found in the second recurrent sample, based on which WHO grade II/III meningioma was diagnosed. The lesion in the left cerebellopontine angle area enlarged after use of radiotherapy combined with temozolomide chemotherapy for 2 months. When sunitinib was added, the residual lesions began to lessen and continuously reduced.ConclusionThis typical case suggested that timely molecular diagnosis for refractory meningiomas contributed to guiding the molecular classification and clinicians to make more reasonable individualized therapeutic regimens, consequently benefiting the patients. This case report also highlighted the potential role of sunitinib in the treatment of refractory meningiomas.


2018 ◽  
Vol 38 (5) ◽  
pp. 535-538 ◽  
Author(s):  
Michael A. Paolini ◽  
Mai-Lan Ho ◽  
Hannah R. Monahan ◽  
Aditya Raghunathan

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