Primitive neuroectodermal tumor: Difference between revisions

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{{SI}}
{{Infobox medical condition
| name            = Primitive neuroectodermal tumor
| image          = [[File:PNET_Histopathology_HE_200x.jpg|left|thumb|Histopathology of a primitive neuroectodermal tumor]]
| caption        = Histopathology of a primitive neuroectodermal tumor
| field          = [[Oncology]]
| synonyms        = PNET
| symptoms        = [[Headache]], [[nausea]], [[vomiting]], [[seizures]], [[neurological deficits]]
| complications  = [[Metastasis]], [[neurological impairment]]
| onset          = Typically in [[children]] and [[young adults]]
| duration        = Variable
| causes          = [[Genetic mutations]]
| risks          = [[Genetic predisposition]], [[family history]]
| diagnosis      = [[Biopsy]], [[imaging studies]]
| differential    = [[Medulloblastoma]], [[Ewing sarcoma]], [[neuroblastoma]]
| treatment      = [[Surgery]], [[radiation therapy]], [[chemotherapy]]
| prognosis      = Variable, depends on stage and location
| frequency      = Rare
}}
[[File:Peripheral_PNET_CD99_200x.jpg|Peripheral Primitive Neuroectodermal Tumor stained with CD99 at 200x magnification|left|thumb]]
[[File:PNET.jpg|Primitive Neuroectodermal Tumor|left|thumb]]
'''Primitive neuroectodermal tumor''' ('''PNET''') is a term used to describe a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissues and bone. They are part of the Ewing family of tumors.
'''Primitive neuroectodermal tumor''' ('''PNET''') is a term used to describe a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissues and bone. They are part of the Ewing family of tumors.
== Classification ==
== Classification ==
PNETs are classified into two types: central PNETs (cPNETs) and peripheral PNETs (pPNETs). [[Central PNETs]] are intracranial and arise from the cells lining the ventricles or from the [[medulla]]. [[Peripheral PNETs]] are found outside the central nervous system and are most commonly found in the chest, abdomen, or pelvis.
PNETs are classified into two types: central PNETs (cPNETs) and peripheral PNETs (pPNETs). [[Central PNETs]] are intracranial and arise from the cells lining the ventricles or from the [[medulla]]. [[Peripheral PNETs]] are found outside the central nervous system and are most commonly found in the chest, abdomen, or pelvis.
== Symptoms ==
== Symptoms ==
The symptoms of PNETs depend on the location of the tumor. Symptoms may include pain, swelling, or a noticeable lump. If the tumor is in the brain, symptoms may include headache, nausea, vomiting, or changes in vision.
The symptoms of PNETs depend on the location of the tumor. Symptoms may include pain, swelling, or a noticeable lump. If the tumor is in the brain, symptoms may include headache, nausea, vomiting, or changes in vision.
== Diagnosis ==
== Diagnosis ==
Diagnosis of PNETs is based on the histological examination of the tumor. [[Immunohistochemistry]] is used to confirm the diagnosis. Other diagnostic tests may include [[Computed tomography|CT scan]], [[Magnetic resonance imaging|MRI]], and [[Positron emission tomography|PET scan]].
Diagnosis of PNETs is based on the histological examination of the tumor. [[Immunohistochemistry]] is used to confirm the diagnosis. Other diagnostic tests may include [[Computed tomography|CT scan]], [[Magnetic resonance imaging|MRI]], and [[Positron emission tomography|PET scan]].
== Treatment ==
== Treatment ==
Treatment for PNETs typically involves a combination of surgery, [[Chemotherapy|chemotherapy]], and [[Radiation therapy|radiation therapy]]. The choice of treatment depends on the size and location of the tumor, as well as the patient's overall health.
Treatment for PNETs typically involves a combination of surgery, [[Chemotherapy|chemotherapy]], and [[Radiation therapy|radiation therapy]]. The choice of treatment depends on the size and location of the tumor, as well as the patient's overall health.
== Prognosis ==
== Prognosis ==
The prognosis for patients with PNETs is generally poor, with a five-year survival rate of less than 50%. Factors that may affect the prognosis include the size and location of the tumor, the patient's age and overall health, and the response to treatment.
The prognosis for patients with PNETs is generally poor, with a five-year survival rate of less than 50%. Factors that may affect the prognosis include the size and location of the tumor, the patient's age and overall health, and the response to treatment.
== See also ==
== See also ==
* [[Ewing's sarcoma]]
* [[Ewing's sarcoma]]
* [[Neuroblastoma]]
* [[Neuroblastoma]]
* [[Medulloblastoma]]
* [[Medulloblastoma]]
[[Category:Neoplasms]]
[[Category:Neoplasms]]
[[Category:Rare diseases]]
[[Category:Rare diseases]]
[[Category:Pediatric cancers]]
[[Category:Pediatric cancers]]
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Latest revision as of 22:16, 9 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Primitive neuroectodermal tumor
Histopathology of a primitive neuroectodermal tumor
Synonyms PNET
Pronounce N/A
Specialty N/A
Symptoms Headache, nausea, vomiting, seizures, neurological deficits
Complications Metastasis, neurological impairment
Onset Typically in children and young adults
Duration Variable
Types N/A
Causes Genetic mutations
Risks Genetic predisposition, family history
Diagnosis Biopsy, imaging studies
Differential diagnosis Medulloblastoma, Ewing sarcoma, neuroblastoma
Prevention N/A
Treatment Surgery, radiation therapy, chemotherapy
Medication N/A
Prognosis Variable, depends on stage and location
Frequency Rare
Deaths N/A


Peripheral Primitive Neuroectodermal Tumor stained with CD99 at 200x magnification
Primitive Neuroectodermal Tumor

Primitive neuroectodermal tumor (PNET) is a term used to describe a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissues and bone. They are part of the Ewing family of tumors.

Classification[edit]

PNETs are classified into two types: central PNETs (cPNETs) and peripheral PNETs (pPNETs). Central PNETs are intracranial and arise from the cells lining the ventricles or from the medulla. Peripheral PNETs are found outside the central nervous system and are most commonly found in the chest, abdomen, or pelvis.

Symptoms[edit]

The symptoms of PNETs depend on the location of the tumor. Symptoms may include pain, swelling, or a noticeable lump. If the tumor is in the brain, symptoms may include headache, nausea, vomiting, or changes in vision.

Diagnosis[edit]

Diagnosis of PNETs is based on the histological examination of the tumor. Immunohistochemistry is used to confirm the diagnosis. Other diagnostic tests may include CT scan, MRI, and PET scan.

Treatment[edit]

Treatment for PNETs typically involves a combination of surgery, chemotherapy, and radiation therapy. The choice of treatment depends on the size and location of the tumor, as well as the patient's overall health.

Prognosis[edit]

The prognosis for patients with PNETs is generally poor, with a five-year survival rate of less than 50%. Factors that may affect the prognosis include the size and location of the tumor, the patient's age and overall health, and the response to treatment.

See also[edit]

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