Journal
PEDIATRIC TRANSPLANTATION
Volume 21, Issue 6, Pages -Publisher
WILEY
DOI: 10.1111/petr.13000
Keywords
Beckwith-Wiedemann syndrome; chemotherapy; desmoplastic nested spindle cell tumors; ectopic ACTH production; hepatic transplant
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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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