title: Recurrent nested stromal epithelial tumor of the liver with extrahepatic metastasis: case report and review of literature.
abstract: Nested stromal epithelial tumor is a recently described primary neoplasm of the liver. This tumor is characterized by well-demarcated nests of spindle and epithelioid cells with occasional calcification and bone formation. An association between these tumors and Cushing syndrome has been described. Herein we report a case of a recurrent nested stromal epithelial tumor of the liver in a 17-year-old female with aggressive clinical behavior and an extrahepatic lymph node metastasis. Also, we provide the first detailed clinical, histologic, immunohistochemical, and cytogenetic comparison of the original and recurrent tumors. Initially, the patient presented with Cushingoid symptoms and epigastric pain, radiating to her back. A computed tomographic (CT) scan revealed a large lesion in the liver. After a partial hepatectomy, the Cushingoid features were resolved. A year later, a CT scan revealed multiple lesions within the liver, and positron emission tomographic/CT imaging showed a hypermetabolic lymph node. The patient underwent a cadaveric liver transplant. Histologically, both the original and recurrent tumors had similar characteristics, with different immunoreactivity, correlating with the absence of systemic hormonal symptoms. Electron microscopy of the original neoplasm revealed an abundance of rough cytoplasmic reticulum and mitochondria. No evidence of endocrine differentiation was found. Cytogenetics of the primary tumor was complex with an abnormal hypotriploid karyotype. Our data indicate that patients with nested stromal epithelial tumor of the liver must be carefully followed with imaging to detect hepatic recurrence and extrahepatic metastases.
authors.forename: Sergey V
authors.initials: SV
authors.lastname: Brodsky
authors.forename: Claudio
authors.initials: C
authors.lastname: Sandoval
authors.forename: Ninu
authors.initials: N
authors.lastname: Sharma
authors.forename: Yasmin
authors.initials: Y
authors.lastname: Yusuf
authors.forename: Marcelo E
authors.initials: ME
authors.lastname: Facciuto
authors.forename: Marissa
authors.initials: M
authors.lastname: Humphrey
authors.forename: Y Albert
authors.initials: YA
authors.lastname: Yeh
authors.forename: Alex
authors.initials: A
authors.lastname: Braun
authors.forename: Myron
authors.initials: M
authors.lastname: Melamed
authors.forename: Milton J
authors.initials: MJ
authors.lastname: Finegold
mesh_terms: D000293:Adolescent; D000782:Aneuploidy; D014408:Biomarkers, Tumor / Q000378:metabolism; D004847:Epithelial Cells / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation; D008198:Lymph Nodes / Q000473:pathology; D009364:Neoplasm Recurrence, Local; D018193:Neoplasms, Complex and Mixed / Q000378:metabolism / Q000556:secondary* / Q000601:surgery; D049268:Positron-Emission Tomography; D017154:Stromal Cells / Q000378:metabolism / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor