title: Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver.
abstract: 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.
authors.affiliation: Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India.
authors.forename: Sunayana
authors.initials: S
authors.lastname: Misra
authors.affiliation: Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India.
authors.forename: Chhagan
authors.initials: C
authors.lastname: Bihari
mesh_terms: D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000235:genetics / Q000378:metabolism; D002277:Carcinoma / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D004847:Epithelial Cells / Q000378:metabolism / Q000473:pathology; D015870:Gene Expression; D018197:Hepatoblastoma / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology*; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D017154:Stromal Cells / Q000378:metabolism / Q000473:pathology; D055815:Young Adult
chemical_list: D000324:Adrenocorticotropic Hormone
keywords: Desmoplastic nested spindle cell tumour; liver; nested stromal-epithelial tumour