Primitive neuroectodermal tumor: Difference between revisions

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{{Short description|Cancer of the developing nervous system}}

{{hatnote|Not to be confused with [[pancreatic neuroendocrine tumor]], which is also abbreviated as PNET.}}

{{Infobox medical condition (new)

| name = Primitive neuroectodermal tumor

| image = PNET_Histopathology_HE_200x.jpg

| caption = Micrograph of an H&E stained section of a peripheral PNET.

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| field = Oncology

| synonyms = PNET

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'''Primitive neuroectodermal tumor''' is a malignant (cancerous) [[neural crest]] tumor.<ref>{{DorlandsDict|eight/000112249|primitive neuroectodermal tumor}}</ref> It is a rare [[tumor]], usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%.<ref name=Smoll20111>{{Cite journal | last1 = Smoll | first1 = N. R. | title = Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs) | doi = 10.1002/cncr.26387 | journal = Cancer | volume = 118 | issue = 5 | pages = 1313–1322 | year = 2012 | pmid = 21837678| pmcs2cid = 8490276 | s2ciddoi-access = 8490276free }}</ref>

It gets its name because the majority of the cells in the tumor are derived from [[neuroectoderm]], but have not developed and differentiated in the way a normal [[neuron]] would, and so the cells appear "primitive". PNET belongs to the [[Ewing family of tumors]].

PNET belongs to the [[Ewing family of tumors]].

==Genetics==

{{Medref|section|date=March 2018}}

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 name="pmid8129041">{{cite journal |vauthors=Eibl RH, Kleihues P, Jat PS, Wiestler OD |title=A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen |journal=Am. J. Pathol. |volume=144 |issue=3 |pages=556–64 |date=March 1994 |pmid=8129041 |pmc=1887088 |doi= |url=}}</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 name="pmid1933879">{{cite journal |vauthors=Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P |title=p53 mutations in nonastrocytic human brain tumors |journal=Cancer Res. |volume=51 |issue=22 |pages=6202–5 |date=November 1991 |pmid=1933879 |doi= |url=http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=1933879}}</ref>{{additional citation needed|date=July 2021}}

==Diagnosis==

[[File:Peripheral PNET CD99 200x.jpg|thumb|CD99 staining of tissue from peripheral PNET]]

===Classification===

It is classified into two types, based on ___location in the body: peripheral PNET and CNS PNET.{{citation needed|date=June 2020}}

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{{main|Central nervous system primitive neuroectodermal tumor}}

PNET of the CNS generally refer to supratentorial PNETs.

* In the past [[medulloblastoma]]s were considered PNETs; however, they are genetically, transcriptionally and clinically distinct. As such, "infratentorial" PNETs are now referred to as medulloblastoma {{Citation needed|date=March 2013}}.

* [[Pineoblastoma]]s are embryonal tumours originating in the [[pineal gland]] and are likely distinct from supratentorial PNETs.

==Treatment==

{{Empty section|date=March 2018}}

The approach to management of a CNS PNET is first to obtain detailed imaging through MRI, as well as additional scans of the patient's body (X-ray, CT, PET, even bone marrow biopsies) to look for metastasis or other associated malignancies. The tumor will then need to be biopsied to confirm the diagnosis. After the diagnosis of a CNS PNET is confirmed, management includes neoadjuvant chemotherapy and radiation (to reduce tumor size burden), complete surgical resection with confirmed negative margins, and/or additional adjuvant post-surgical chemotherapy. CNS PNET is aggressive and must be managed as so. Palliative care services should also become involved in the patient's care team when the diagnosis is made.

<ref>Honrado, Carlo P, and Augustine L Moscatello. Primitive Neuroectodermal Tumors: Background, Epidemiology, Clinical Features, Medscape, 21 Jan. 2021, emedicine.medscape.com/article/855644-overview#a7.</ref>

==See also==

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{{Reflist}}

{{Medical resources

| DiseasesDB = 31470

| ICD10 =

| ICD9 =

| ICDO = {{ICDO|9473|3}}

| OMIM =

| MedlinePlus =

| eMedicineSubj = ped

| eMedicineTopic = 2589

| eMedicine_mult = {{eMedicine2|neuro|326}}

| MeshID = D018242

}}

{{Nervous tissue tumors}}

{{Osseous and chondromatous tumors}}

{{Small blue round cell tumors}}

[[Category:Pediatric cancers]]