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Calcifying nested stromal-epithelial tumor is a rare hepatic malignancy with approximately 50 cases reported in the literature. Its clinical presentation is nonspecific, and the diagnosis is mainly based on histology which shows nests of spindle and epithelioid cells along with a desmoplastic myofibroblastic stroma containing variable calcification and ossification. In this report, we present a case of a 24-year-old woman with a history of abdominal pain, distension, and dyspepsia. She had a palpable liver with normal liver function test results. Serum alpha-fetoprotein levels were within normal range, and serologies for hepatitis B and C virus remained negative. Radiological investigations (magnetic resonance imaging and computed tomography) showed a large, right hepatic lobe mass with tumor invasion into the right posterior portal vein, but the 2 modalities could not characterize the lesion. Finally, an ultrasound-guided biopsy of the liver lesion provided the diagnosis of calcifying nested stromal-epithelial tumor. The tumor was resected successfully.
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http://dx.doi.org/10.14309/crj.0000000000001163 | DOI Listing |
Clin J Gastroenterol
August 2025
Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, Kita 15, Nishi 7, Kita-Ku, Sapporo, 060-8638, Japan.
Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant tumor that primarily affects young females. Multiple SPNs, particularly in older females, are extremely rare. While these tumors generally have a good prognosis, some may progress to malignancy.
View Article and Find Full Text PDFInt J Surg Pathol
May 2025
Department of Pathology, İstanbul Başakşehir Çam and Sakura City Hospital, İstanbul, Turkey.
Calcifying Nested Stromal-Epithelial Tumor (CNSET) of the liver is a hepatic neoplasm of uncertain lineage, primarily affecting young individuals. This case report aims to elucidate the clinical, histopathological, and molecular characteristics of CNSET, contributing to the sparse knowledge regarding this unusual entity. We present here a 17-year-old female patient diagnosed with CNSET, highlighting the tumor's radiologic appearance, surgical management, and postoperative findings, alongside a comprehensive review of existing literature on the subject.
View Article and Find Full Text PDFBiomedicines
February 2025
Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, 1468 Madison Avenue, New York, NY 10029, USA.
Hepatic mesenchymal tumors (HMTs) are non-epithelial benign and malignant tumors with or without specific mesenchymal cell differentiation. They are relatively uncommon. Except for mesenchymal hamartoma, calcified nested stromal-epithelial tumor, and embryonal sarcoma, most mesenchymal lesions are not specific to the liver.
View Article and Find Full Text PDFCurr Med Imaging
August 2024
Department of Radiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Medical Imaging, Shanghai 200032, China.
Background: A calcifying nested stromal-epithelial tumour (CNSET) is an erratic primary liver malignant tumour frequently discovered in young girls and females. Neither its pathogenesis nor its nosogenesis is clearly known. While principally indolent, infrequent tumours with aggressive clinical progression have been defined.
View Article and Find Full Text PDFCancers (Basel)
December 2023
Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, 80-210 Gdansk, Poland.
Unlabelled: a single-centre experience in bi- and uni-segmentectomies for primary liver tumours in children.
Methods: This study included 23 patients that underwent (bi)segmentectomy. There were 15 malignant tumours (hepatoblastoma-13 patients), 7 benign tumours, and 1 calcifying nested stromal epithelial tumour.