Recurrence of Dysembryoplastic Neuroepithelial Tumor following Resection
ABSTRACT Dysembryoplastic neuroepithelial tumor (DNT) is an increasingly recognized surgically curable tumor that characteristically presents with seizures in children. We report a case of a patient who underwent resection of a histologically proven DNT at 6 years of age. The resection was thought to be total at the time of surgery, and this impression was confirmed on postoperative imaging. Following the initial resection, the patient underwent surveillance imaging at regular intervals. Six years following the initial surgery, surveillance imaging demonstrated an enlarging area of signal abnormality at the site of the prior resection. The patient underwent a second resection with pathological confirmation of DNT recurrence. Although recurrence of DNT following resection is rare, this case suggests that surveillance imaging may have a role in patients with DNT, even following resections that are thought to be complete.
Article: Neuronal Tumors[Show abstract] [Hide abstract]
ABSTRACT: Gangliogliomas and gangliocytomas belong to a family of rare, slow-growing, neuronal tumors. Courville fi rst introduced the term “ganglioglioma” in 1930 to describe the mixed neuronal and glial elements typically seen in this tumor (Courville 1930). Although there is a pathologic difference between ganglioglioma and gangliocytoma, the natural history and biology of these two subtypes appear to be the same.
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ABSTRACT: Pediatric low-grade gliomas are the most common tumors of the central nervous system in children, accounting for almost 50% of all childhood brain tumors. They are a heterogeneous group of tumors with different histologic subtypes. Most treatment studies address low-grade gliomas as a single entity, depriving us of histology-specific treatment outcomes. This is mostly due to a lack of understanding of tumor biology at the molecular level. Pediatric low-grade gliomas are not benign, and most incompletely resected tumors will progress and negatively affect quality of life. The advancements made in understanding sporadic pilocytic astrocytoma and neurofibromatosis 1-associated pilocytic astrocytoma in particular have paved the way for potential targeted therapy and biological stratification. Such progress in pilocytic astrocytoma needs to be consolidated and expanded to other histologic varieties of pediatric low-grade gliomas.Cancer biology & therapy 02/2009; 8(1):4-10. DOI:10.4161/cbt.8.1.7237 · 3.07 Impact Factor
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ABSTRACT: Dysembryoplastic neuroepithelial tumors (DNETs) have traditionally been viewed as benign "quasihamartomatous" tumors widely considered curable with surgery alone. More recently, case reports have described malignant gliomas arising after irradiation and recurrences following subtotal or even gross total resection. Herein, we describe five cases of DNET with recurrences 2-7 years after resection. Although the radiology was often alarming (e.g., new ring enhancing mass), the pathology remained benign in most cases. Nonetheless, a probably radiation induced anaplastic astrocytoma was encountered in one case 7 years after therapy. These findings suggest that these patients may need closer follow-up than initially suggested, lending further support to the notion that this tumor behaves more like a benign neoplasm, rather than a dysplastic or hamartomatous lesion.Journal of Neuro-Oncology 04/2009; 94(2):283-92. DOI:10.1007/s11060-009-9849-9 · 3.07 Impact Factor