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SAITO Taiichi
Department Research Institutes and Facilities, Research Institutes and Facilities Position |
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| Article types | Original article |
| Language | English |
| Peer review | Peer reviewed |
| Title | Familial occurrence of dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: case report. |
| Journal | Formal name：Neurosurgery Abbreviation：Neurosurgery ISSN code：1524-4040(Electronic)0148-396X(Linking) |
| Domestic / Foregin | Foregin |
| Volume, Issue, Page | 63(2),pp.E370-2; discussion E372 |
| Author and coauthor | Saito Taiichi, Sugiyama Kazuhiko, Yamasaki Fumiyuki, Tominaga Atsushi, Kurisu Kaoru, Takeshima Yukio, Hirose Takanori |
| Publication date | 2008/08 |
| Summary | OBJECTIVE:Dysembryoplastic neuroepithelial tumor (DNT)-like neoplasms of the septum pellucidum are extremely rare. In this article, we report the familial occurrence of these neoplasms.CLINICAL PRESENTATION:We report two cases of such neoplasms: Patient 1, a 42-year-old woman, and Patient 2, the 20-year-old nephew of Patient 1. Patient 1 experienced headache and worsening dizziness; Patient 2 experienced headache and worsening dizziness and also had partial seizures. In both cases, magnetic resonanceCLINICAL PRESENTATION:imaging (MRI) revealed an intraventricular tumor adjacent to the septum pellucidum. Both tumors appeared as a hypointense region on T1-weighted MRI, and both appeared as a hyperintense region on T2-weighted MRI without gadolinium enhancement. Interestingly, both tumors had a high apparent diffusion coefficient.INTERVENTION:Both tumors were subtotally removed and had common histological findings, such as alveolar structures with oligodendroglia-like cells andINTERVENTION:"INTERVENTION:specific glioneuronal element.INTERVENTION:"INTERVENTION:These findings are consistent with a dysembryoplastic neuroepithelial tumor-like neoplasm. After tumor removal, the symptoms disappeared. The postoperative course was uneventful, and the patients did not require adjuvant therapy. MRI showed no regrowth of residual tumors at 4 years (Patient 1) and 2 years (Patient 2) postoperatively.CONCLUSION:The familial occurrence of this rare tumor suggests that both of these cases arose from a common germline mutation. Identification of this rare tumor in this rare location is important to avoid unnecessary adjuvant therapy. A markedly high apparent diffusion coefficient and histological findings of specific glioneuronal element can facilitate the differential diagnosis of dysembryoplastic neuroepithelial tumor-like neoplasms. Genetic study of affected patients in this family may provide clues to its molecular pathogenesis. |
| DOI | 10.1227/01.NEU.0000320421.82255.63 |
| Document No. | 18797318 |