Calcifying nested stromal-epithelial tumor: A rare hepatic neoplasm

Reyes, María Pérez; Pérez, Belinda Sánchez; Díaz, F.; Villalba, Julio Santoyo; Santoyo, Julio Santoyo

doi:10.1016/j.cireng.2021.06.008

Cited by 1 publication

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“…We presented a case of a patient with unresectable CNSET, an extremely rare primary hepatic tumor. Our patient being a young female, having little to no symptoms at the initial presentation was consistent with previously reported cases ( 19 , 31 ). One of the hypotheses is that CNSET might develop from benign calcified hepatic lesions.…”

Section: Discussionsupporting

confidence: 92%

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

et al. 2022

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IntroductionCalcifying nested stromal epithelial tumor (CNSET) is an extremely rare diagnosis among patients treated for primary hepatic neoplasms. There are only 45 cases reported worldwide. Histopathological characteristics are well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma with variable calcification and ossification. It is mostly diagnosed in children and young females. Treatment strategies implemented for the management of CNSET include radiofrequency ablation, transarterial chemoembolization, surgical resection, adjuvant and neoadjuvant chemotherapy, and liver transplantation. Given the small number of available cases, there are still no established standards of treatment for this neoplasm.Case PresentationA 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient's clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment.ConclusionMultiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.

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Section: Discussionsupporting

confidence: 92%