Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review

Abstract

Calcifying nested stromal-epithelial tumor is a rare entity that has gone by a variety of names in the literature: ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor of the liver. To our knowledge, approximately 38 cases have been reported in the literature. The histogenesis is still largely unknown but histopathologically is characterized by nests of spindle and epithelioid cells in an organoid arrangement surrounded by a prominent dense myofibroblastic stroma with occasional psammomatous calcification and focal heterotopic ossification. Vascular invasion is rare and tumoral recurrence is uncommon with only a single reported case of metastasis leading to death. Treatment is mainly by surgical intervention with the role of chemotherapy seeming limited, but lack of data hinders a true recommendation. It is important to rule out other processes such as hepatoblastoma, calcified hemangioma, synovial sarcoma, metastatic gastrointestinal stromal tumor, desmoplastic small round cell tumor, among others, which appear similar radiographically and histopathologically.

Pre-clinical validation of an RNA-based precision oncology platform for patient-therapy alignment in a diverse set of human malignancies resistant to standard treatments

“…The proposed PCM framework discussed in this study is uniquely suited to identify therapeutic alternatives even for rare cancers lacking actionable mutations and standard of care options. Calcifying Nested Stromal Epithelial Tumor (CNSET) is an exceptionally uncommon primary hepatic tumor that occurs in children and young adults, with only about 40 cases reported in the literature [36]. While localized disease is often effectively cured with surgical resection, recurrent and de novo metastatic disease demonstrates chemotherapy resistance and there are no proven therapeutic options [37][38][39][40].…”

Surgical resection of calcifying nested stromal-epithelial tumor in an adolescent female: A case report

“…There have been several cases of patients presenting with abdominal pain, mass, or Cushing syndrome. Liver function tests are most often normal without elevation of aminotransferases, bilirubin, or alkaline phosphatase [1]. Levels of tumor markers are similarly normal.…”

“…CNSET affects females to males in a ratio of 2.5:1 and most frequently involves the right hepatic lobe (65 % of cases) [1]. This tumor, as evidenced by review of the cases reported, appears to have a low malignant potential overall [9].…”

“…It is characterized by nests of epithelioid and spindle cells with a desmoplastic myofibroblastic stroma and variable calcification and ossification. There have been 38 cases reported in the literature, with the tumor occurring most frequently in women (male to female ratio 1:2.5) [1]. In the cases reported, CNSET was often incidentally found in asymptomatic patients [2].…”

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

“…NSET of the liver is an extremely rare, recently described nonhepatocytic, nonbiliary tumor of the liver. [1] In 2001, it was first described by Ishak et al [2] It is also called as desmoplastic nested spindle cell tumor of the liver and ossifying malignant mixed epithelial and stromal tumor. It consists of nests of the epithelial and spindle cells with associated myofibroblastic stroma and variable intralesional calcification and ossification.…”