Calcifying nested epithelial stromal tumor of the liver

MedGen UID:: 758637
•Concept ID:: C3472610
•: Neoplastic Process

Synonyms:	Calcifying nested epithelial stromal tumor; Calcifying Nested Epithelial Stromal Tumor of the Liver; calcifying nested epithelial stromal tumor of the liver; Calcifying Nested Epithelial-Stromal Tumor of the Liver; Calcifying Nested Stromal Epithelial Tumor of the Liver; Calcifying Nested Stromal-Epithelial Tumor; Calcifying Nested Stromal-Epithelial Tumor of the Liver
SNOMED CT:	Calcifying nested epithelial stromal tumor (450898007)

Monarch Initiative:	MONDO:0006122

Definition

A rare malignant tumor that arises from the liver and occurs in children. It is characterized by the presence of nested epithelioid and spindle cells. Desmoplasia, calcifications, and bone formation may also be present. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCalcifying nested epithelial stromal tumor of the liver

Disease
- Neoplasm
  - Neoplasm by Special Category
    - Childhood neoplasm
      - Childhood Liver Neoplasm
        Childhood Malignant Liver Neoplasm
        Calcifying nested epithelial stromal tumor of the liver

Recent clinical studies

Etiology

Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome.

Khoshnam N, Robinson H, Clay MR, Schaffer LR, Gillespie SE, Shehata BM
Eur J Med Genet 2017 Feb;60(2):136-139. Epub 2016 Dec 10 doi: 10.1016/j.ejmg.2016.12.001. PMID: 27965001

Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver.

Misra S, Bihari C
APMIS 2016 Apr;124(4):245-51. Epub 2016 Jan 14 doi: 10.1111/apm.12502. PMID: 26994733

Calcifying nested stroma-epithelial tumor of the liver: a case report and review of literature.

Wang Y, Zhou J, Huang WB, Rao Q, Ma HH, Zhou XJ
Int J Surg Pathol 2011 Apr;19(2):268-72. Epub 2011 Feb 13 doi: 10.1177/1066896910394841. PMID: 21320858

See all (3)

Diagnosis

Mesenchymal Neoplasms of the Liver.

Papke DJ Jr
Surg Pathol Clin 2023 Sep;16(3):609-634. Epub 2023 Jun 11 doi: 10.1016/j.path.2023.04.013. PMID: 37536892

Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature.

Tsuruta S, Kimura N, Ishido K, Kudo D, Sato K, Endo T, Yoshizawa T, Sukeda A, Hiraoka N, Kijima H, Hakamada K
World J Surg Oncol 2018 Nov 19;16(1):227. doi: 10.1186/s12957-018-1528-x. PMID: 30453967 Free PMC Article

Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver.

Misra S, Bihari C
APMIS 2016 Apr;124(4):245-51. Epub 2016 Jan 14 doi: 10.1111/apm.12502. PMID: 26994733

Calcifying nested stromal-epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis.

Schaffer LR, Shehata BM, Yin J, Schemankewitz E, Alazraki A
Clin Imaging 2016 Jan-Feb;40(1):137-9. Epub 2015 Oct 21 doi: 10.1016/j.clinimag.2015.10.003. PMID: 26589005

Advances in hepatobiliary pathology: update for 2010.

Lefkowitch JH
Clin Liver Dis 2010 Nov;14(4):747-62. doi: 10.1016/j.cld.2010.07.007. PMID: 21055694

See all (8)