小児と成人患者の比較解析による新たに発症したNF2頭蓋内髄膜腫の臨床的特徴

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Clin Neurol Neurosurg.2020 Jul;194:105799. S0303-8467(20)30142-6. doi: 10.1016/j.clineuro.2020.105799.Epub 2020-03-19.

小児と成人患者の比較解析による新たに発症したNF2頭蓋内髄膜腫の臨床的特徴

Clinical features of newly developed NF2 intracranial meningiomas through comparative analysis of pediatric and adult patients.

Peng Li
Tao Wu
Ying Wang
Fu Zhao
Zhenmin Wang
Xingchao Wang
Bo Wang
Zhijun Yang
Pinan Liu

PMID: 32229353 DOI: 10.1016/j.clineuro.2020.105799.

抄録

目的:

NF2患者は生涯を通じて新たに髄膜腫を発症する可能性がある。新たに発症したNF2髄膜腫の臨床的特徴についてはほとんど知られていない。この研究では、大規模な患者集団において新たに発症したNF2髄膜腫を解析した。

OBJECTIVE: NF2 patients can develop new meningiomas throughout their lifetime. Little is known about the clinical features of newly developed NF2 meningiomas. In this study, we analyzed newly developed NF2 meningiomas in a large patient population.

患者と方法:

NF2患者452人のうち、小児の81人と成人の939人のNF2髄膜腫の位置パターンを比較し、成人期に新たに発生した髄膜腫の優勢な位置を明らかにした。24人のNF2患者における新たに発症した39例の髄膜腫の臨床的特徴を要約した。NF2髄膜腫増殖率の臨床的危険因子を分析した。

PATIENTS AND METHODS: Among 452 NF2 patients, the location patterns of 81 pediatric and 939 adult NF2 meningiomas were compared to find the predominant locations of newly developed meningiomas in adulthood. The clinical features of 39 newly developed meningiomas in 24 NF2 patients were summarized. Clinical risk factors of NF2 meningioma growth rates were analyzed.

結果:

RESULTS: Pediatric patients had significantly more intracranial meningiomas than adult patients at the skull base (except for the petrosal region) (p < 0.0063). Adult patients had significantly more cranial meningiomas than pediatric patients at the parasagittal, parafalcine (middle & posterior), and frontal/parietal/cerebellar convex surfaces (p < 0.0063). Newly developed NF2 meningiomas in adults tended to occur at different locations than the locations of NF2 meningiomas in pediatric patients. New meningiomas could develop at various ages. Ninety-five NF2 patients were imaged and followed up for at least one year. Twenty-four patients (25.3 %) developed 39 new meningiomas during the follow-up period. They usually had initial meningiomas when new meningiomas occurred. The number of newly developed meningiomas per patient and the petrosal location were significantly associated with both the absolute and relative annual growth rates (p < 0.05).

結論:

新たに発症したNF2髄膜腫の数は、NF2疾患の重症度の臨床的マーカーとなるようである。成人では、新規NF2髄膜腫は初期髄膜腫を有する患者に発生する傾向がある。新たに発生したNF2髄膜腫の優勢な部位は、傍矢状面、傍鰭状面（中・後面）、および前頭・頭頂・小脳凸面であるように思われる。

CONCLUSIONS: The number of newly developed NF2 meningiomas seems to be a clinical marker of NF2 disease severity. In adults, new NF2 meningiomas tend to occur in patients with initial meningiomas. The predominant locations of newly developed NF2 meningiomas seem to be the parasagittal, parafalcine (middle/posterior), and frontal/parietal/cerebellar convex surfaces.