CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2020; 41(02): 235-237
DOI: 10.4103/ijmpo.ijmpo_58_20
Grand Round

Nested Stromal and Epithelial Tumor of the Liver: An Unusual Nonhepatocytic Entity

MC Suresh Babu
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
A Sreevalli
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Syed Adil Hassan
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
G Champaka
Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
D Lokanatha
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Linu Abraham Jacob
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
KN Lokesh
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
AH Rudresha
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
LK Rajeev
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Saldanha Smitha
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
› Author Affiliations
Financial support and sponsorship Nil.

Introduction

Nested stromal-epithelial tumor (NSET) of the liver is an extremely rare, nonhepatocytic, nonbiliary tumor of the liver. To the best of our knowledge, 41 cases have been reported in literature. We report the 42nd case. The reported tumors have been found predominantly in females and more commonly in children, and most arose from the right lobe of the liver. In a number of cases, an association between these tumors and Cushing syndrome has also been described. Here, we report NSET of the liver in a 39-year-old female patient. To the best of our knowledge, this case is the first to be reported from India to have undergone transarterial chemoembolization (TACE).



Publication History

Received: 12 February 2020

Accepted: 09 April 2020

Article published online:
23 May 2021

© 2020. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 
  • References

  • 1 Benedict M, Zhang X. Calcifying nested stromal-epithelial tumor of the liver: An update and literature review. Arch Pathol Lab Med 2019; 143: 264-8
  • 2 Ishak KG, Goodman ZD, Stocker JT. Tumors of the Liver and Intrahepatic Bile Ducts. Ch. 11. Washington, DC: Armed Forces Institute of Pathology; 2001: 276-78
  • 3 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
  • 4 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
  • 5 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
  • 6 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: 976-83
  • 7 Wang Y, Zhou J, Huang WB, Rao Q, Ma HH, Zhou XJ. Calcifying nested stroma-epithelial tumor of the liver: A case report and review of literature. Int J Surg Pathol 2011; 19: 268-72
  • 8 Venus A, Mehta SS, Natarajan S. A rare nonhepatocytic primary liver neoplasm with an unusual presentation: Ossifying malignant mixed epithelial and stromal tumor. Indian J Med Paediatric Oncol 2018; 39: 237-40
  • 9 Rod A, Voicu M, Chiche L, Bazille C, Mittre H, Louiset E. et al. Cushing’s syndrome associated with a nested stromal epithelial tumor of the liver: Hormonal, immunohistochemical, and molecular studies. Eur J Endocrinol 2009; 161: 805-10
  • 10 Khoshnam N, Robinson H, Clay MR, Schaffer LR, Gillespie SE, Shehata BM. Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome. Eur J Med Genet 2017; 60: 136-9
  • 11 Assmann G, Kappler R, Zeindl-Eberhart E, Schmid I, Häberle B, Graeb C. 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: 1815-27
  • 12 Remes-Troche JM, Montaño-Loza A, Meza-Junco J, García-Leiva J, Torre-Delgadillo A. Hepatoblastoma in adult age. A case report and literature review. Ann Hepatol 2006; 5: 179-81
  • 13 Tsuruta S, Kimura N, Ishido K, Kudo D, Sato K, Endo T. et al. Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: A case report and review of the literature. World J Surg Oncol 2018; 16: 227
  • 14 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
  • 15 Hommann M, Kaemmerer D, Daffner W, Prasad V, Baum RP, Petrovitch A. et al. Nested stromal epithelial tumor of the liver – Liver transplantation and follow-up. J Gastrointest Cancer 2011; 42: 292-5