Dysembryoplastic neuroepithelial tumor
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2] Maria Fernanda Villarreal, M.D. [3]
Synonyms and keywords: DNT; DNET; Dysembryoplastic neuroepithelial tumors; Dysembryoplastic neuroepithelial tumour; Dysembryoplastic neuroepithelial tumours
Overview
Dysembryoplastic neuroepithelial tumor (also known as DNT or DNET) is a type of benign glioneuronal brain tumor that arises from the oligodendrocyte, which is normally involved in the production of myelin in the central nervous system. Dysembryoplastic neuroepithelial tumor is most commonly found in the temporal lobe (supratentorial cortex). These are glioneuronal tumours comprised of both glial and neuron cells and often have ties to focal cortical dysplasia.[1]
Historical Perspective
- Dysembryoplastic neuroepithelial tumor was first discovered by Dumas-Duport in 1988.
Classification
- According to the World Health Organization is classified as benign brain tumors (WHO Grade I)
- Dysembryoplastic neuroepithelial tumor may be classified according to the World Health Organization into 3 groups:
- Complex
- Specific glioneuronal element (SGNE)
- Glial nodules and a multinodular architecture
- Component of associated focal cortical dysplasia is common (Blumcke classification IIIb)
- Simple
- Specific glioneuronal element (SGNE) only
- Nonspecific
- Same clinical and neuroimaging features as complex DNE
- No specific glioneuronal element (SGNE)
Pathophysiology
- The pathogenesis of dysembryoplastic neuroepithelial tumor is characterized by the overgrowth of glioneuronal tissue, which is mainly conformed of oligodendrocytes.
- The IDH1 mutations have been associated with the development of dysembryoplastic neuroepithelial tumor.
- On gross pathology, characteristic findings of dysembryoplastic neuroepithelial tumor, include:
- Cortical mass
- On microscopic histopathological analysis, characteristic findings of dysembryoplastic neuroepithelial tumor, include:
- Axonal columns oriented to the surface
- Floating neurons in eosinophilic matrix
- Lined by cells similar to oligodendrocytes:
- Large central nuclei with indentations
- Multiple small nucleoli (common)
- Clear cytoplasm
- Few stellated astrocytes
- On inmunohistochemistry, characteristic findings of dysembryoplastic neuroepithelial tumor, include:
- Positive MAP2
- Positive CD34
- Positive calbindin
- Positive nestin
- Positive MIB-1 (Ki-67)
Causes
- There are no established causes for dysembryoplastic neuroepithelial tumor.
Differentiating Dysembryoplastic Neuroepithelial Tumor from Other Diseases
- Dysembryoplastic neuroepithelial tumor must be differentiated from other tumors that cause seizures, such as:
- Pleomorphic xanthoastrocytoma
- Ganglioglioma
- Oligodendroglioma
- Desmoplastic infantile ganglioglioma
Epidemiology and Demographics
- The prevalence of dysembryoplastic neuroepithelial tumor remains unknown.
Age
- Dysembryoplastic neuroepithelial tumor is more commonly observed among patients aged between 8 and 19 years old.
- Dysembryoplastic neuroepithelial tumor is more commonly observed among children and young patients.
Gender
- Dysembryoplastic neuroepithelial tumor affects men and women equally.
Race
- There is no racial predilection for dysembryoplastic neuroepithelial tumor.
Risk Factors
- Common risk factors in the development of dysembryoplastic neuroepithelial tumor are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- The majority of patients with dysembryoplastic neuroepithelial tumor remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with dysembryoplastic neuroepithelial tumor may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of dysembryoplastic neuroepithelial tumor include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with dysembryoplastic neuroepithelial tumor is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of dysembryoplastic neuroepithelial tumor is made when at least [number] of the following [number] diagnostic criteria are met:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- Dysembryoplastic neuroepithelial tumor is usually asymptomatic.
- Symptoms of dysembryoplastic neuroepithelial tumor may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with dysembryoplastic neuroepithelial tumor usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with dysembryoplastic neuroepithelial tumor.
- A [positive/negative] [test name] is diagnostic of dysembryoplastic neuroepithelial tumor.
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of dysembryoplastic neuroepithelial tumor.
- Other laboratory findings consistent with the diagnosis of dysembryoplastic neuroepithelial tumor include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with dysembryoplastic neuroepithelial tumor.
- [Imaging study 1] is the imaging modality of choice for dysembryoplastic neuroepithelial tumor.
- On [imaging study 1], dysembryoplastic neuroepithelial tumor is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- Dysembryoplastic neuroepithelial tumor may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for dysembryoplastic neuroepithelial tumor; the mainstay of therapy is supportive care.
- The mainstay of therapy for dysembryoplastic neuroepithelial tumor is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for dysembryoplastic neuroepithelial tumor.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of dysembryoplastic neuroepithelial tumor.
- [Surgical procedure] can only be performed for patients with [disease stage] dysembryoplastic neuroepithelial tumor.
Prevention
- There are no primary preventive measures available for dysembryoplastic neuroepithelial tumor.
- Effective measures for the primary prevention of dysembryoplastic neuroepithelial tumor include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with dysembryoplastic neuroepithelial tumor are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
References
- ↑ Suh, Yeon-Lim (2015-11-01). "Dysembryoplastic Neuroepithelial Tumors". Journal of Pathology and Translational Medicine. 49 (6): 438–449. doi:10.4132/jptm.2015.10.05. ISSN 2383-7837. PMC 4696533. PMID 26493957.