Presentation of an Extremely Rare Tumor: Nested Stromal-epithelial Tumor of the Liver
Nested stromal-epithelial tumor (NSET) is a very rare mesenchymal liver tumor, which is originated from non-hepatocyte cells in the liver. NSET has been defined as a non-hepatocytic and non-biliary tumor of the liver with epithelial and spindled cells with associated myofibroblastic stroma and variable intralesional calcification and ossification. It is often seen in young women and can be represented as large masses. This article is aimed to present the clinical and imaging findings of a 77-year-old male patient with a massive NSET including coarse calcification, with literature comparison.
Ishak KG, Goodman ZD, Stocker JT. Miscellaneous malignant tumors. Tumors of the Liver and Intrahepatic Bile Ducts. Ch. 11. Washington, DC: Armed Forces Institute of Pathology; 2001. p. 276–78.
2. Heywood G, Burgart LJ, Nagorney DM. Ossifying malignant mixed epithelial and stromal tumor of the liver: A case report of a previously undescribed tumor. Cancer 2002;94:1018–22.
3. Makhlouf HR, Abdul-Al HM, Wang G, Goodman ZD. Calcifying nested stromal-epithelial tumors of the liver: A clinico-pathologic, immunohistochemical, and molecular genetic study of 9 cases with a long-term follow-up. Am J Surg Pathol 2009;33:976–83. [CrossRef]
4. Heerema-McKenney A, Leuschner I, Smith N, Sennesh J, Finegold MJ. Nested stromal epithelial tumor of the liver: Six cases of a distinctive pediatric neoplasm with frequent calcifications and association with cushing syndrome. Am J Surg Pathol 2005;29:10–20. [CrossRef]
5. Wang Y, Zhou J, Huang WB, Rao Q, Ma HH, Zhou XJ, et al. Calcifying nested stroma-epithelial tumor of the liver: A case report and review of literature. Int J Surg Pathol 2011;19:268–72.
6. Schaffer LR, Shehata BM, Yin J, Schemankewitz E, Alazraki A. Calcifying nested stromal-epithelial tumor (CNSET) of the liver: A newly recognized entity to be considered in the radiologist’s differential diagnosis. Clin Imaging 2016;40:137–9.
7. Meletani T, Cantini L, Lanese A, Nicolini D, Cimadamore A, Agostini A, et al. Are liver nested stromal epithelial tumors always low aggressive? World J Gastroenterol 2017;23:8248–55.
8. Khoshnam N, Robinson H, Clay MR, Schaffer LR, Gillespie SE, Shehata BM, et al. Calcifying nested stromal-epithelial tumor (CNSET) of the liver in beckwith-wiedemann syndrome. Eur J Med Genet 2017;60:136–9. [CrossRef]
9. Procopio F, Di Tommaso L, Armenia S, Quagliuolo V, Roncalli M, Torzilli G, et al. Nested stromal-epithelial tumour of the liver: An unusual liver entity. World J Hepatol 2014;6:155–9.
10. Wu XR, Cong WM. Hepatic tumors in children. Surgical Pathology of Hepatobiliary Tumors. Ch. 8. Singapore: Springer Nature and People’s Medical Publishing House; 2017. p. 299–300.
11. Brodsky SV, Sandoval C, Sharma N, Yusuf Y, Facciuto ME, Humphrey M, et al. Recurrent nested stromal epithelial tumor of the liver with extrahepatic metastasis: case report and review of literature. Pediatr Dev Pathol 2008;11:469–73. [CrossRef]
12. Grazi GL, Vetrone G, d'Errico A, Caprara G, Ercolani G, Cescon M, et al. Nested stromal-epithelial tumor (NSET) of the liver: A case report of an extremely rare tumor. Pathol Res Pract 2010;206:282–6. [CrossRef]