Recurrent Nested Stromal Epithelial Tumor of the Liver With Extrahepatic Metastasis (Case Report and Review of Literature)

brodsky, sergey; Sandoval, Claudio; Sharma, Ninu; Yusuf, Yasmin; Facciuto, Marcelo; Humphrey, Marissa; Yeh, y; Braun, Alex; Melamed, Myron R.; Finegold, Milton J.

doi:10.2350/07-12-0391

Cited by 5 publications

(11 citation statements)

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“…Since these tumors have a known metastatic potential, postoperative follow-up with PET can be helpful. [14,15] In our case, the patient had a relatively shorter recurrence-free interval compared to the previously reported cases.…”

Section: Discussionsupporting

confidence: 41%

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

Babu

Sreevalli

Hassan

et al. 2020

Indian Journal of Medical and Paediatric Oncology

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Section: Discussionsupporting

confidence: 41%

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

Babu

Sreevalli

Hassan

et al. 2020

Indian Journal of Medical and Paediatric Oncology

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“…No evidence of extra-hepatic disease is seen in either of the tumours. However, Brodsky et al (7) reported a case of a recurrent NSET in a 17-yearold female which presented with extra hepatic lymph node metastasis. One case was reported in a 22-month baby who had an incidental liver mass, which on histopathology was reported as NSET, with associated Beckwith-Weidmann syndrome (15).…”

Section: Presentationmentioning

confidence: 99%

Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver

Misra

Bihari

2016

APMIS

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Desmoplastic nested spindle cell tumour of liver (DNSTL), nested stromal-epithelial tumour (NSET) and calcifying nested stromal-epithelial tumour (CNSET) are recently described entities with similar morphology, immunohistochemistry and molecular genetics. These are rare entities with only three large case series described till date. These tumours commonly present in the paediatric age group. NSETs, in addition have been described to be associated with ectopic adrenocorticotropic hormone (ACTH) production and Cushingoid features. It is important to discuss this rare group of tumours with a low malignant potential as the most common radiological differential diagnosis is hepatoblastoma, which has a relatively poorer prognosis. Thus, a pathologist needs to keep this entity in mind, so as to offer a correct histological diagnosis.

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“…Clinically, the prognosis is good for tumors that are completely resected. However, 5 cases of locally recurrent NSET, 1 case with lymph node metastases in a young adult [19], and 1 case with pulmonary metastases following a liver transplantation [9] have been reported. Surgical excision appears to be the preferred treatment, as chemotherapy and radiotherapy have not been beneficial for those with unresectable tumors [11].…”

Section: Discussionmentioning

confidence: 99%

Nested Stromal Epithelial Tumor of the Liver in Beckwith-Wiedemann Syndrome

et al. 2013

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Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder characterized by overgrowth, congenital malformation, and tumor predisposition. Children with BWS have a higher incidence of tumors, commonly intra-abdominal tumors such as Wilms tumor, hepatoblastoma, and adrenal cortical carcinoma. Here, we describe the first case of a rare hepatic malignancy of nested stromal epithelial tumor (NSET) of the liver in a child with BWS. A 22-month old girl with BWS had a new incidental liver mass. Her alpha-fetoprotein levels were normal. She underwent a liver segmentectomy. Histopathologic features combined with immunohistochemistry results (positivity for pankeratin [AE1/3], CD56, CK19, CD117, CD99 [weak membranous pattern], β-catenin, and WT1-COOH [focal]), were diagnostic of NSET of the liver. This is the first case of NSET of the liver associated with BWS. Its occurrence at such an early age is consistent with the tumor predisposition of BWS.

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Recurrent Nested Stromal Epithelial Tumor of the Liver With Extrahepatic Metastasis (Case Report and Review of Literature)

Cited by 5 publications

References 0 publications

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

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

Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver

Nested Stromal Epithelial Tumor of the Liver in Beckwith-Wiedemann Syndrome

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