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Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome |
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| Authors: | Sarah Tehseen Louis Rapkin Erwin Schemankewitz Joseph F Magliocca Rene Romero |
| Institution: | 1. Department of Pediatrics, Division of Pediatric Hematology Oncology (Aflac Cancer and Blood Disorders Center), Emory University, Atlanta, GA, USA;2. Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA;3. Department of Pathology, Children's Healthcare of Atlanta, Atlanta, GA, USA;4. Department of Surgery, Division of Liver Transplantation, Emory University, Atlanta, GA, USA |
| Abstract: | 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. |
| Keywords: | Beckwith– Wiedemann syndrome chemotherapy desmoplastic nested spindle cell tumors ectopic ACTH production hepatic transplant |