Primitive neuroectodermal tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Primitive neuroectodermal tumors; PNET; CNS PNET; Askin tumor; Peripheral neuroepithelioma; Ependymoblastoma

Overview

Primitive neuroectodermal tumor (also known as "PNET") is a rare type of malignant neural crest tumor. Primitive neuroectodermal tumor arises from the neuroectoderm, which is normally involved in the development of the nervous system. Primitive neuroectodermal tumor was first discovered by James Ewing, an American pathologist, in 1921.[1] Primitive neuroectodermal tumors are more commonly seen among children and young adults . The median age at diagnosis is 25 years of age. Prognosis is generally poor, and the 5-survival rate of patients with primitive neuroectodermal tumor is approximately 53%

Historical Perspective

  • Primitive neuroectodermal tumor was first discovered by James Ewing, an American pathologist, in 1921.[1]

Classification

  • Primitive neuroectodermal tumor may be classified according to World Health Organization into 3 subtypes:
  • CNS primitive neuroectodermal tumors (PNETs)
  • Neuroblastomas
  • Peripheral primitive neuroectodermal tumors (pPNETs)

Pathophysiology

  • Primitive neuroectodermal tumor is composed of primitive undifferentiated neuroepithelial cells.
  • Primitive neuroectodermal tumors are intracranial and usually located in the cerebral hemisphere.
  • The EWS-like gene has been associated with the development of primitive neuroectodermal tumor.
  • On gross pathology, characteristic findings of primitive neuroectodermal tumor, include:
  • Cystic components
  • On microscopic histopathological analysis, characteristic findings of primitive neuroectodermal tumor, include:
  • Small blue cell tumor
  • Round hyperchromatic cells
  • Abundant mitotic figures
  • Homer Wright rosettes
  • Fibrosis

Causes

  • There are no established causes for primitive neuroectodermal tumor.

Differentiating Primitive Neuroectodermal Tumor from Other Diseases

  • Primitive neuroectodermal tumor must be differentiated from other diseases that cause seizures, or increase on intracranial pressure, such as:
  • Astrocytoma
  • Ependymoma
  • Oligodendroglioma
  • Intracranial teratoma

Epidemiology and Demographics

  • The prevalence of primitive neuroectodermal tumor remains unknown.
  • Primitive neuroectodermal tumor account for 4-17% of all soft tissue paediatric tumors.

Age

  • The median age at diagnosis is 25 years old.
  • Primitive neuroectodermal tumor is more commonly observed among children and young adults.

Gender

  • Primitive neuroectodermal tumor affects men and women equally.

Race

  • There is no racial predilection for primitive neuroectodermal tumor.

Risk Factors

  • Common risk factors in the development of primitive neuroectodermal tumor, include:

Natural History, Complications and Prognosis

  • The majority of patients with primitive neuroectodermal tumor remain asymptomatic for years.
  • Early clinical features include morning headache,
  • If left untreated, patients with primitive neuroectodermal tumor may progress to develop
  • Common complications of primitive neuroectodermal tumor, include:
  • Increased intracranial pressure
  • Cranial nerve palsy
  • Seizures
  • Prognosis is generally poor, and the 5-survival rate of patients with primitive neuroectodermal tumor is approximately 53%

Diagnosis

Symptoms

  • Clinical presentation of primitive neuroectodermal tumors is often non-specific.
  • Symptoms of primitive neuroectodermal tumor may include the following:
  • Obtain medical history, on the following syndromes:
  • Gorlin-Goltz syndrome
  • Turcot syndrome

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with primitive neuroectodermal tumor.

Imaging Findings

  • MRI is the imaging modality of choice for primitive neuroectodermal tumor.
  • On MRI, findings of primitive neuroectodermal tumor, include:
  • T1: highly variable and can be hypointense to isointense
  • T2: generally high signal solid components
  • Cystic components are common
  • Low signal portions due to calcific compoments
  • T1 C+ (Gd): shows markedly heterogenous enhancement and leptomeningeal seeding is common
  • DWI: often shows rescricted diffusion
  • MR spectroscopy: elevated choline, decreased NAA, elevated taurine (Tau) peak (relatively specific for PNET).

Other Diagnostic Studies

  • Primitive neuroectodermal tumor may also be diagnosed using [diagnostic study name].

Treatment

Medical Therapy

  • There is no treatment for primitive neuroectodermal tumor; the mainstay of therapy is supportive care.

Surgery

  • Surgery is the mainstay of therapy for primitive neuroectodermal tumor.
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of primitive neuroectodermal tumor.
  • [Surgical procedure] can only be performed for patients with [disease stage] primitive neuroectodermal tumor.

Prevention

  • There are no primary preventive measures available for primitive neuroectodermal tumor.

References

  1. 1.0 1.1 James Ewing. https://en.wikipedia.org/wiki/James_Ewing_(pathologist) Accessed on May 20, 2016