Dysembryoplastic neuroepithelial tumor

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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). Dysembryoplastic neuroepithelial tumor was first discovered by Dumas-Duport in 1988. These are glioneuronal tumours comprised of both glial and neuron cells and often have ties to focal cortical dysplasia.[1] According to the World Health Organization is classified as benign brain tumors (WHO Grade I). Dysembryoplastic neuroepithelial tumors may be classified according to the World Health Organization into 3 groups: complex, simple, and unspecific. Dysembryoplastic neuroepithelial tumor is more commonly observed among patients aged between 8 and 19 years old. The majority of patients with dysembryoplastic neuroepithelial tumor remain asymptomatic are symptomatic at the time of diagnosis. Early clinical features include seizures, headaches, and personality changes. Common complications of dysembryoplastic neuroepithelial tumor, include: status epilepticus and severe memory loss. Surgical excision is the most common approach to the treatment of dysembryoplastic neuroepithelial tumor.

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:<ref name=
  • 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:

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

  • There are no associated risk factors in the development of dysembryoplastic neuroepithelial tumor.

Natural History, Complications and Prognosis

  • The majority of patients with dysembryoplastic neuroepithelial tumor remain asymptomatic are symptomatic at the time of diagnosis.
  • Early clinical features include seizures, headaches, and personality changes.
  • If left untreated, the majority of patients with dysembryoplastic neuroepithelial tumor may progress to develop severe cognitive dysfunction.
  • Common complications of dysembryoplastic neuroepithelial tumor, include:
  • Prognosis is generally good, and the 5-year survival rate of patients with dysembryoplastic neuroepithelial tumor is approximately 80%.

Diagnosis

Symptoms

  • The most important symptom of dysembryoplastic neuroepithelial tumor are seizures.
  • Symptoms of dysembryoplastic neuroepithelial tumor may include the following:

Physical Examination

  • Patients with dysembryoplastic neuroepithelial tumor usually are well-appearing.
  • Physical examination may be remarkable for:

Laboratory Findings

  • There are no specific laboratory findings associated with dysembryoplastic neuroepithelial tumor.

Imaging Findings

  • MRI is the imaging modality of choice for dysembryoplastic neuroepithelial tumor.
  • On MRI, findings of dysembryoplastic neuroepithelial tumor, include:

T1

  • Solid component iso to hypointense

T1 C+ (Gd)

  • Solid component variable contrast enhancement

T2

  • Hyperintense solid component
  • Variable signal in the cystic component depending on amount of proteinaceous material or presence of blood products
  • Peritumoral FLAIR/T2 oedema is distinctly uncommon

T2 (GE/SWI)

  • Calcified areas (common) will show blooming signal loss

Other Diagnostic Studies

  • Dysembryoplastic neuroepithelial tumor may also be diagnosed using EEG and markers
  • Findings on EEG, include:
  • Findings on markers, include:
  • Positive synaptophysin
  • Positive neuronal specific enolase

Treatment

Medical Therapy

  • There is no treatment for dysembryoplastic neuroepithelial tumor; the mainstay of therapy is surgery.

Surgery

  • Surgery is the mainstay of therapy for dysembryoplastic neuroepithelial tumor.
  • Surgical excision is the most common approach to the treatment of dysembryoplastic neuroepithelial tumor.

Prevention

  • There are no primary preventive measures available for dysembryoplastic neuroepithelial tumor.
  • Once diagnosed and successfully treated, patients with dysembryoplastic neuroepithelial tumor are followed-up periodically.
  • Follow-up testing will include MRI evaluation, EEG, and neurological exam

References

  1. 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.