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

Abstract

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.

Liver Transplantation for Unresectable Calcifying Nested Stromal Epithelial Tumor: Case Report With a 1-Year Follow-Up and Review of Literature

“…Tehseen et al report using Basiliximab, steroids, and Tacrolimus, while Gard et al reports using Tacrolimus alone, we do not know whether in this case immunosuppression was altered after recurrence ( 3 , 25 ).…”

“…It is worth noticing that this patient had suspected lymph node metastases in PET/CT prior to LT. The authors do not provide post-transplant follow-up of this patient ( 3 ). Four patients received neoadjuvant chemotherapy.…”

“…To the best of our knowledge, there were eight cases of patients diagnosed with CNSET that underwent liver transplantation. Six of them as a primary method of treatment ( 1 , 3 , 11 , 12 , 24 , 25 ), and two underwent transplantation for recurrence ( 2 , 10 ) ( Table 2 ).…”

“…In the available reports that provide reasons for qualifying CNSET patients for LT, among the most common indications is the exhaustion of possibilities for radical surgical resection, whether it be tumor size, vascular involvement, significant mass effect, or risk of progressive liver failure ( 1 , 3 , 10 , 11 , 24 , 25 ). In particular cases, patients were qualified for LT due to poor response to systemic treatment ( 1 , 11 , 24 ).…”

“…There were only four reported cases of recurrence among 35 patients that underwent liver resection as the sole surgical intervention ( 7 , 8 , 16 , 26 ). To the best of our knowledge, only six patients underwent liver transplantation (LT) for primarily unresectable CNSET ( 1 , 3 , 11 , 12 , 24 , 25 ), and two patients were transplanted due to recurrence ( 2 , 10 ). Due to the low occurence of this tumor, data in the literature is still limited.…”