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Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver
Author(s) -
Misra Sunayana,
Bihari Chhagan
Publication year - 2016
Publication title -
apmis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0903-4641
DOI - 10.1111/apm.12502
Subject(s) - pathology , cushingoid , hepatoblastoma , stromal cell , immunohistochemistry , differential diagnosis , medicine , biology
Desmoplastic nested spindle cell tumour of liver (DNSTL), nested stromal‐epithelial tumour (NSET) and calcifying nested stromal‐epithelial tumour (CNSET) are recently described entities with similar morphology, immunohistochemistry and molecular genetics. These are rare entities with only three large case series described till date. These tumours commonly present in the paediatric age group. NSETs, in addition have been described to be associated with ectopic adrenocorticotropic hormone (ACTH) production and Cushingoid features. It is important to discuss this rare group of tumours with a low malignant potential as the most common radiological differential diagnosis is hepatoblastoma, which has a relatively poorer prognosis. Thus, a pathologist needs to keep this entity in mind, so as to offer a correct histological diagnosis.