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{{Infobox_Disease |
{{SI}}                                                                 
  Name          = Primitive neuroectodermal tumor |
{{CMG}} {{AE}} {{MV}}
  Image          = |
  Caption        = |
  DiseasesDB    = 31470 |
  ICD10          = |
  ICD9          = |
  ICDO          = M9473/3 |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  eMedicine_mult = |
  MeshID        = D018242 |
}}
{{SI}}
{{CMG}}


{{SK}} PNET; Askin tumor; peripheral neuroepithelioma; ependymoblastoma
{{SK}} Primitive neuroectodermal tumors; PNET; CNS PNET; PNET; Askin tumor; Peripheral neuroepithelioma; Ependymoblastoma
==Overview==


== 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 is 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%
'''Primitive Neuroectodermal Tumors''', or '''PNET''', refers to two different tumor types. These include Peripheral PNET and CNS PNET. This can be a source of confusion<ref>[http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=9323 Ependymoblastoma] at US National Institutes of Health Office of Rare Diseases</ref>.
==Historical Perspective==
*Primitive neuroectodermal tumor was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].


==Classification==
==Classification==
===Peripheral PNET===
*Primitive neuroectodermal tumor may be classified according to [classification method] into [number] subtypes/groups:
The Peripheral PNET is now thought to be virually identical to [[Ewing's sarcoma|Ewing sarcoma]]:
:*[group1]
:*[group2]
:*[group3]
*Other variants of primitive neuroectodermal tumor include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
*The pathogenesis of primitive neuroectodermal tumor is characterized by [feature1], [feature2], and [feature3].
*The XXX has been associated with the development of primitive neuroectodermal tumor.
*On gross pathology, characteristic findings of primitive neuroectodermal tumor, include:
*On microscopic histopathological analysis,  characteristic findings of primitive neuroectodermal tumor, include:
==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 [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
==Epidemiology and Demographics==
* The prevalence of primitive neuroectodermal tumor is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of primitive neuroectodermal tumor was estimated to be [number or range] cases per 100,000 individuals in [location].
===Age===
*Patients of all age groups may develop primitive neuroectodermal tumor.


"Current evidence indicates that both Ewing's sarcoma and PNET have a similar neural phenotype and, because they share an identical chromosome translocation, they should be viewed as the same tumor, differing only in their degree of neural differentiation. Tumors that demonstrate neural differentiation by light microscopy, immunohistochemistry, or electron microscopy have been traditionally labeled PNETs, and those that are undifferentiated by these analyses have been diagnosed as Ewing's sarcoma." <ref name="robbins">Robbins Pathologic Basis of Disease, 7th edition</ref>
===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 [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, patients with primitive neuroectodermal tumor may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of primitive neuroectodermal tumor, include:


===PNET of the CNS===
*Prognosis is generally poor, and the 5-survival rate of patients with primitive neuroectodermal tumor is approximately 53%
== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of primitive neuroectodermal tumor is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
*Primitive neuroectodermal tumor is usually asymptomatic.
*Symptoms of primitive neuroectodermal tumor may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]


PNET of the CNS are grossly divided into [[Supratentorial|supratentorial]] PNET and [[Infratentorial neoplasms|infratentorial]] PNET, the latter being more common. <ref name="emed-pnet">Subrata Ghosh, MD, MBBS, MS, [http://www.emedicine.com/neuro/topic326.htm Primitive Neuroectodermal Tumors of the Central Nervous System], eMedicine 2007.</ref>
=== Physical Examination ===
*Patients with primitive neuroectodermal tumor usually are well-appearing.
*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 primitive neuroectodermal tumor.


An example of infratentorial PNET includes [[Medulloblastoma|Medulloblastomas]], which occurs in the cerebellum.  
===Imaging Findings===
*MRI is the imaging modality of choice for primitive neuroectodermal tumor.
*On [imaging study 1], findings of primitive neuroectodermal tumor, include:
:*
:*
:*
   
=== Other Diagnostic Studies ===
*Primitive neuroectodermal tumor may also be diagnosed using [diagnostic study name].


An example of supratentorial PNET includes  Pinealoblastomas, which occurs in the pineal region.
== Treatment ==
 
=== Medical Therapy ===
==Model==
*There is no treatment for primitive neuroectodermal tumor; the mainstay of therapy is supportive care.
Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis. <ref>Eibl RH, Kleihues P, Jat PS, Wiestler OD (1994) A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen. Am J Pathol. 1994 Mar;144(3):556-64.</ref>. The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10 % of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 Large T- antigen, other than p53. <ref>Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P: p53 mutations in nonastrocytic human brain tumors. Cancer Res (1991) 51:6202-5</ref>
 
==Related Chapters==
*[[Ependymoma]]


=== 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==
==References==
{{Reflist|2}}
{{Reflist|2}}
 
== Acknowledgements ==
[[Category: Oncology]]
The content on this page was first contributed by: C. Michael Gibson, M.S., M.D.
 
[[de:Primitiv neuroektodermaler Tumor]]
[[pl:Obwodowy niedojrzały guz neuroektodermalny]]
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Revision as of 19:36, 20 May 2016

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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; 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 is 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 [scientist name], a [nationality + occupation], in [year] during/following [event].


Classification

  • Primitive neuroectodermal tumor may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of primitive neuroectodermal tumor include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of primitive neuroectodermal tumor is characterized by [feature1], [feature2], and [feature3].
  • The XXX has been associated with the development of primitive neuroectodermal tumor.
  • On gross pathology, characteristic findings of primitive neuroectodermal tumor, include:
  • On microscopic histopathological analysis, characteristic findings of primitive neuroectodermal tumor, include:

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 [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of primitive neuroectodermal tumor is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of primitive neuroectodermal tumor was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop primitive neuroectodermal tumor.


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 [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, patients with primitive neuroectodermal tumor may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of primitive neuroectodermal tumor, include:
  • Prognosis is generally poor, and the 5-survival rate of patients with primitive neuroectodermal tumor is approximately 53%

Diagnosis

Diagnostic Criteria

  • The diagnosis of primitive neuroectodermal tumor is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Primitive neuroectodermal tumor is usually asymptomatic.
  • Symptoms of primitive neuroectodermal tumor may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]


Physical Examination

  • Patients with primitive neuroectodermal tumor usually are well-appearing.
  • 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 primitive neuroectodermal tumor.

Imaging Findings

  • MRI is the imaging modality of choice for primitive neuroectodermal tumor.
  • On [imaging study 1], findings of primitive neuroectodermal tumor, include:

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

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