Calcifying nested stromal–epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis

Schaffer, Lauren R.; Shehata, Bahig M.; Yin, Julie; Schemankewitz, Erwin; Alazraki, Adina

doi:10.1016/j.clinimag.2015.10.003

Cited by 17 publications

(22 citation statements)

References 12 publications

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“…3 CNSET has been reported to be associated with cortisol-related syndrome and rarely, Beckwith-Wiedemann syndrome (BWS). [2][3][4][5][6][7][8][9][10] CLINICAL CHARACTERISTICS AND IMAGING Calcifying nested stromal-epithelial tumors are rare liver tumors with nonspecific clinical features ( Table 1). The age range in which these tumors occur is reported to be between 2 and 34 years.…”

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confidence: 99%

“…12 The radiologic findings that characterize CNSET are described as large, well-circumscribed, macrolobulated masses, with enhancement and calcification. 7,13 Areas of calcification appear hyperdense, whereas cystic or myxoid components within the tumor appear hypodense. 12 On magnetic resonance imaging examination, there is a predominant T1 hypointensity and T2 hyperintensity.…”

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confidence: 99%

“…12 On magnetic resonance imaging examination, there is a predominant T1 hypointensity and T2 hyperintensity. 7 Based on the imaging characteristics of CNSET, the radiologic interpretation is likely to be a hepatoblastoma or a calcified hemangioma. 12…”

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

Benedict

Zhang

2018

Archives of Pathology &Amp; Laboratory Medicine

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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.

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

Benedict

Zhang

2018

Archives of Pathology &Amp; Laboratory Medicine

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“…Nested stromal-epithelial tumor (NSET) of the liver is a very rare type of cancer[ 1 ], with few cases diagnosed worldwide. It usually affects young people with age range from 1 to 34 years[ 2 ] and no sex preference.…”

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Are liver nested stromal epithelial tumors always low aggressive?

Meletani

Cantini

Lanese

et al. 2017

WJG

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Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. The histological exam confirmed the diagnosis of NSET. After 6 mo from surgery, a liver recurrence was described and a chemo-embolization was performed. After a further disease progression, based on the correlation between the histological features of the disease and those of the hepatoblastoma, a similar chemotherapy regimen (with cisplatin and ifosfamide/mesna chemotherapy, omitting doxorubicin due to liver impairment) was administered. However, infection of the biliary catheter required a dose modification of the treatment. No benefit was noted and a progression of disease was radiologically assessed after only four cycles. The worsening of the clinical status prevented further treatments, and the patient died a few months later. This case report documents how the NSET might have an aggressive and non-preventable behavior. No chemotherapy schedules with a proved efficacy are available, and new data are needed to shed light on this rare neoplasm.

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“…[ 3 ] Most of these cases have been reported in young females, in the right lobe of the liver. [ 4 ] Its pathogenesis remains unclear, however, based on some immunohistochemical studies these neoplasms are suspected to originate from hepatic mesenchymal precursor cell with primitive differentiation along the bile duct lineage. [ 1 ] These tumors are often detected incidentally on imaging.…”

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Nested stromal-epithelial tumor of liver with recurrent extrahepatic metastasis: Role of fluorodeoxyglucose positron emission tomography/computed tomography

2017

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Nested stromal-epithelial tumor (NSET) is a very rare nonhepatocytic and nonbiliary primary tumor of the liver. An 8-year-old boy was incidentally detected with hepatic lesions, involving both lobes of the liver for which he later underwent orthotopic liver transplant. The hepatic lesions were confirmed to be NSET following histopathological examination of explant liver specimen. He later developed recurrence with multiple metastatic lesions, including multi-station nodal and right talar bone involvement. We here present the case highlighting the importance of 18F-fludeoxyglucose positron emission tomography/computed tomography in the management of this rare tumor, in particular for monitoring disease progression and/or recurrence.

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Calcifying nested stromal–epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis

Cited by 17 publications

References 12 publications

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

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

Are liver nested stromal epithelial tumors always low aggressive?

Nested stromal-epithelial tumor of liver with recurrent extrahepatic metastasis: Role of fluorodeoxyglucose positron emission tomography/computed tomography

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