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

Desmoplastic spindle cell tumors of liver are rare tumors of low malignant potential characterized by well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma. While surgery remains the definitive treatment, there have been reports of tumor recurrence locally and metastasis which respond poorly to chemotherapy. Hepatic transplant has been attempted in cases of recurrence or large size of primary tumor. Long-term follow-up and imaging surveillance are required as these tumors have shown a tendency for recurrence many years after initial therapy.

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“…Association with fragile X syndrome has also been reported. In both cases, the tumors were completely resected with no recurrence upon follow‐up …”

Section: Discussionmentioning

confidence: 99%

Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome

Tehseen

Rapkin

Schemankewitz

et al. 2017

Pediatric Transplantation

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“…A thorough computerized PUBMED search for Desmoplastic nested spindle cell tumour (DNSTL), Nested stromal epithelial tumour (NSET) and Calcifying nested stromal epithelial tumour CNSET revealed three case series by Hill et al, Heerema‐McKinney et al and Makhlouf et al as discussed above and thirteen case reports. The cases are summarized in Table .…”

Section: Clinical Features Of Tumours Reported In the English Literatmentioning

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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“…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%

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

Cited by 23 publications

References 20 publications

Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome

Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome

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

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

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