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Primitive neuroectodermal tumor of the corpus uteri

MedGen UID:
1640822
Concept ID:
C4707724
Neoplastic Process
Synonyms: body of uterus primitive neuroectodermal tumor; body of uterus primitive neuroectodermal tumour; Malignant peripheral neuroectodermal tumor of the corpus uteri; malignant peripheral neuroectodermal tumor of the corpus uteri; malignant peripheral neuroectodermal tumour of the corpus uteri; Peripheral neuroectodermal cancer of the corpus uteri; peripheral neuroectodermal cancer of the corpus uteri; primitive neuroectodermal tumor of body of uterus; Primitive neuroectodermal tumor of corpus uteri; primitive neuroectodermal tumor of the corpus uteri; primitive neuroectodermal tumour of body of uterus
SNOMED CT: Primitive neuroectodermal tumor of corpus uteri (766247009)
 
Monarch Initiative: MONDO:0016263
Orphanet: ORPHA213630

Definition

Primitive neuroectodermal tumor of the corpus uteri is a rare cancer of corpus uteri derived from neural crest cells, characterized by small, round neoplastic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, poorly circumscribed polypoid mass with necrotic areas and hemorrhage. It usually presents with lower abdominal or pelvic pain, irregular vaginal bleeding or discharge, pelvic mass and uterine enlargement. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimitive neuroectodermal tumor of the corpus uteri

Recent clinical studies

Diagnosis

Akazawa M, Saito T, Ariyoshi K, Okadome M, Yokoyama R, Taguchi K
J Obstet Gynaecol Res 2018 Oct;44(10):2008-2015. Epub 2018 Jul 26 doi: 10.1111/jog.13753. PMID: 30051552
Cate F, Bridge JA, Crispens MA, Keedy VL, Troutman A, Coffin CM, Fadare O
Hum Pathol 2013 Apr;44(4):656-63. Epub 2012 Dec 21 doi: 10.1016/j.humpath.2012.09.008. PMID: 23266445
Akbayir O, Güngördük K, Rafioğlu G, Gülkilik A, Yavuz E, Tekirdağ AI, Odabaş E
Arch Gynecol Obstet 2008 Apr;277(4):345-8. Epub 2007 Sep 6 doi: 10.1007/s00404-007-0451-0. PMID: 17823807
Varghese L, Arnesen M, Boente M
Int J Gynecol Pathol 2006 Oct;25(4):373-7. doi: 10.1097/01.pgp.0000215300.39900.4e. PMID: 16990715

Therapy

Akazawa M, Saito T, Ariyoshi K, Okadome M, Yokoyama R, Taguchi K
J Obstet Gynaecol Res 2018 Oct;44(10):2008-2015. Epub 2018 Jul 26 doi: 10.1111/jog.13753. PMID: 30051552
Akbayir O, Güngördük K, Rafioğlu G, Gülkilik A, Yavuz E, Tekirdağ AI, Odabaş E
Arch Gynecol Obstet 2008 Apr;277(4):345-8. Epub 2007 Sep 6 doi: 10.1007/s00404-007-0451-0. PMID: 17823807

Prognosis

Daya D, Lukka H, Clement PB
Hum Pathol 1992 Oct;23(10):1120-9. doi: 10.1016/0046-8177(92)90030-7. PMID: 1328031

Supplemental Content

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