Nörofibromatozis Tip 2 ile İlişkili Multipl Menenjiyomların Zamansal, Mekansal ve Histopatolojik Heterojenitesi: 11 Yıllık Takipli Bir Olgu Sunumu

Abstract

Nörofibromatozis tip 2 (NF2), sıklıkla çoklu meningiomalarla ilişkili kalıtsal bir tümör predispozisyon sendromudur. Bu olgu sunumunda, 12 yıl boyunca izlenen ve yedi cerrahi girişim gerektiren çoklu kraniyal ve spinal meningiomalar geliştiren genç bir kadın hasta sunulmaktadır. Tümörler serebellum, temporal lob, serebellopontin köşe, korpus kallozum posterioru, servikal omurga ve fronto-orbital bölge dahil olmak üzere farklı anatomik lokalizasyonlarda ortaya çıkmıştır. Histopatolojik inceleme, Dünya Sağlık Örgütü (DSÖ) Grade 1 meningioma, transisyonel meningioma, psammomatöz meningioma, fibröz meningioma ve atipik meningioma (DSÖ Grade 2) varlığını göstererek belirgin bir heterojenite ortaya koymuştur. İzlem sürecinde hastaya ayrıca temozolomid tedavisi uygulanmış, geçici radyolojik stabilizasyon sağlanmasına rağmen sonraki dönemde ek tedavi gerektiren progresyon gelişmiştir. Bu olgu, NF2 ile ilişkili meningiomaların zamansal, mekânsal ve histopatolojik heterojenitesini ortaya koymakta ve multifokal hastalığa sahip bireylerde kişiselleştirilmiş tedavi planlaması, multidisipliner takip ve uzun dönem izlemin önemini vurgulamaktadır.

Keywords

• Nörofibromatozis tip 2
• Meningioma
• Histopatolojik heterojenite
• Çoklu meningiomalar
• Uzun dönem takip

Supporting Institution

Yok.

Ethical Statement

Bu olgu sunumunun yayımlanması için hastadan yazılı bilgilendirilmiş onam alınmıştır. Ayrıca hastanın aile üyelerinden de yazılı onam alınmıştır. Kurum politikaları doğrultusunda bu olgu sunumu için etik kurul onayı gerekmemektedir.

Temporal, spatial, and histopathological heterogeneity of multiple meningiomas associated with neurofibromatosis type 2: An 11-year follow-up case report

Abstract

Neurofibromatosis type 2 (NF2) is a hereditary tumor predisposition syndrome frequently associated with multiple meningiomas. We report a young woman with NF2 who was followed for more than 11 years and developed multiple cranial and spinal meningiomas requiring seven surgical interventions. Tumors arose in distinct anatomical locations, including the cerebellum, temporal lobe, cerebellopontine angle, posterior corpus callosum region, cervical spine, and fronto-orbital region. Histopathological examination demonstrated marked heterogeneity, including transitional, psammomatous, and fibrous meningioma subtypes (CNS WHO grade 1), as well as atypical meningioma (CNS WHO grade 2). During follow-up, the patient also received temozolomide therapy, during which temporary radiological stabilization was observed before subsequent progression necessitated additional treatment. This case highlights the temporal, spatial, and histopathological heterogeneity of NF2-associated meningiomas and underscores the importance of individualized treatment planning, multidisciplinary follow-up, and long-term surveillance in patients with multifocal disease.

Keywords

• Neurofibromatosis type 2
• Meningioma
• Histopathological heterogeneity
• Multiple meningiomas
• Long-term follow-up

Supporting Institution

None

Ethical Statement

Written informed consent for publication was obtained from the patient. Additional written consent was also obtained from her family members. Ethics committee approval was not required for this case report in accordance with institutional policies.

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