Calcifying nested stromal-epithelial tumor (CNSET) of the liver

Victor Ianole, Loredana Beatrice Ungureanu, Eugenia Moroșan, Cristian Dumitru Lupașcu

Abstract


Calcifying nested stromal-epithelial tumor (CNSET) of the liver is an extremely rare primary tumor of non-hepatic and non-biliary origins which generally affects children and young adults, mainly women. We report a case of 28-year-old woman with a history of oral contraceptive consumption for 9 years, who underwent surgery for a large right hepatic mass that proved to be a CNSET, according to the pathology report. Postoperatively the patient had a slow recovery and after discharge the patient was sent to the oncology department where she underwent several treatment cycles with Imatinib. The case report documented all the clinical and histopathological features necessary for diagnosis of CNSET. CNSET of liver should always be considered and included in the differential diagnosis of hepatic tumors especially if the patient is a young woman with a long history of oral contraceptive consumption and an abdominal palpable mass.


Keywords


calcifying nested stromal-epithelial tumor; hepatic tumor; hepatoblastoma; liver; mixed tumor

Full Text:

PDF

References


Khoshnam N, Robinson H, Clay MR, et al. Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome. Eur J Med Genet 2017; 60(2):136-139.

Hamilton SR, Aaltonen LA (Eds): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. IARC Press: Lyon 2000.

Weeda VB, de Reuver PR, Bras H et al. Cushing syndrome as presenting symptom of calcifying nested stromal-epithelial tumor of the liver in an adolescent boy: a case report. J Med Case Rep 2016; 10:160.

Makhlouf HR, Abdul-Al HM, Wang G, Goodman ZD. Calcifying nested stromal-epithelial tumors of the liver: a clinicopathologic, immunohistochemical, and molecular genetic study of 9 cases with a long-term follow-up. Am J Surg Pathol 2009; 33(7):976-983.

Schaffer LR, Shehata BM, Yin J, et al. 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(1):137-139.

Fryer E, Chetty R. Unusual and rare tumours of the liver. Diagn Histopathol 2012; 18(10):449-456.

Procopio F, Di Tommaso L, Armenia S, et al. Nested stromal-epithelial tumour of the liver: An unusual liver entity. World J Hepatol 2014; 6(3):155-159.

Grazi GL, Vetrone G, d'Errico A, et al. Nested stromal-epithelial tumor(NSET) of the liver: a case report of an extremely rare tumor. Pathol Res Pract 2010;206(4):282-286.

Meletani T, Cantini L, Lanese A, et al. Are liver nested stromal epithelial tumors always low aggressive? World J Gastroenterol 2017; 23(46):8248-8255.

Assmann G, Kappler R, Zeindl-Eberhart E, et al. β-Catenin mutations in 2 nested stromal epithelial tumors of the liver--a neoplasia with defective mesenchymal-epithelial transition. Hum Pathol 2012; 43(11):1815-1827.

Oviedo Ramírez MI, Bas Bernal A, Ortiz Ruiz E, et al. Desmoplastic nested spindle cell tumor of the liver in an adult. Ann Diagn Pathol 2010; 14(1):44-49.




DOI: http://dx.doi.org/10.22551/2018.18.0501.10120

Copyright (c) 2018 Victor Ianole, Loredana Beatrice Ungureanu, Eugenia Moroșan, Cristian Dumitru Lupașcu

Creative Commons License
Archive of Clinical Cases is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

 

ISSN: 2360-6975