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. Dysembryoplastic neuroepithelial tumors 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 and are undiagnosed until they become 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.[2]

Classification

Dysembryonic neuroepithelial tumors are classified as benign brain tumors (WHO Grade I). Dysembryoplastic neuroepithelial tumors may be classified into 3 groups:[2]

  • Simple
    • Specific glioneuronal element (SGNE) only
  • Nonspecific
  • Complex
    • Specific glioneuronal element (SGNE)
    • Glial nodules and a multinodular architecture
    • Component of associated focal cortical dysplasia is common (Blumcke classification IIIb)
    • Same clinical and neuroimaging features as complex DNE
    • No specific glioneuronal element (SGNE)

Pathophysiology

Pathogenesis

The pathogenesis of dysembryoplastic neuroepithelial tumor is characterized by the overgrowth of glioneuronal tissue, which primarily consists of oligodendrocytes.

Genetics

The IDH1 gene mutations have been associated with the development of dysembryoplastic neuroepithelial tumor.

Gross Pathology

On gross pathology, characteristic findings of dysembryoplastic neuroepithelial tumor may appear as a cortical mass.[2]

Microscopic Pathology

  • On microscopic histopathological analysis, characteristic findings of dysembryoplastic neuroepithelial tumor may include:[2]
  • On immunohistochemical analysis, characteristic findings of dysembryoplastic neuroepithelial tumor may include:[2]

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:[2]

Epidemiology and Demographics

Prevalence

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.[2]

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

Natural History

  • The majority of patients with dysembryoplastic neuroepithelial tumor remain asymptomatic and only become 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.

Complications

Common complications of dysembryoplastic neuroepithelial tumor include:[2]

Prognosis

The prognosis of dysembryoplastic neuroepithelial tumor is generally good; the 5-year survival rate of patients with dysembryoplastic neuroepithelial tumor is approximately 80%.

Diagnosis

Symptoms

The most common symptom of dysembryoplastic neuroepithelial tumors are seizures. Other common symptoms of dysembryoplastic neuroepithelial tumor may include:[2]

Physical Examination

Patients with dysembryoplastic neuroepithelial tumor are commonly 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.

MRI Findings

On MRI, findings of dysembryoplastic neuroepithelial tumor may include:[3][2]

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 quantity proteinaceous material or presence of blood products
  • Peri-tumoral FLAIR/T2 edema is distinctly uncommon
T2 (GE/SWI)
  • Calcified areas (common) will show blooming signal loss

CT Findings

On CT scan, findings of dysembryoplastic neuroepithelial tumor may include:

  • Tumors with cortical location may scallop the inner table of the skull vault (44-60%) but no erosion
  • The cranial fossa can be minimally enlarged at times
  • Calcification in approximately 30% (more common histologically)
  • Low density
  • No enhancement

Other Diagnostic Studies

Dysembryoplastic neuroepithelial tumor may also be diagnosed using EEG.[2] Findings on EEG may include:

  • Repetitive spikes
  • Burst of polyspikes

Treatment

Medical Therapy

There is no treatment for dysembryoplastic neuroepithelial tumor; the mainstay of therapy is surgery.[2]

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

Primary Prevention

There are no known primary preventive measures for dysembryoplastic neuroepithelial tumor.[2]

Secondary Prevention

Secondary prevention strategies following diagnosis and treatment of dysembryoplastic neuroepithelial tumor include regular follow-up 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. ISSN 2383-7837. PMC 4696533Freely accessible. PMID 26493957. doi:10.4132/jptm.2015.10.05. 
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 Dysembryoplastic neuroepithelial tumour. Wikipedia. https://en.wikipedia.org/wiki/Dysembryoplastic_neuroepithelial_tumour Accessed on May 2, 2016
  3. Dysembryoplastic neuroepithelial tumour. Libre Pathology https://librepathology.org/wiki/Dysembryoplastic_neuroepithelial_tumour Accessed on May 2, 2016

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