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WHO Classification of Tumours
Leiomyosarcoma, NOS
Liver and intrahepatic bile ducts


Definition

An uncommon, aggressive malignant smooth muscle neoplasm, usually occurring in post-menopausal women. It is characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid, granular cell, inflammatory and myxoid leiomyosarcomas.

Hepatic leiomyosarcoma
Conventional leiomyosarcomas are almost always metastatic when involving the liver, and search for a primary tumour in locations such as the retroperitoneum is always necessary. EBV-associated leiomyosarcomas/smooth-muscle tumours can form primary hepatic masses. These tumours occur in patients with immunosuppression, either acquired (HIV/AIDS-associated) or iatrogenic (usually transplant-associated). In liver-transplant patients, occurrence from donor cells has been reported in allografts, and occurrence from recipient cells outside the graft.

Histologically, these tumours are typically less differentiated than typical leiomyosarcomas, composed of oval to spindled mesenchymal cells with immunoreactivity for α smooth muscle actin, but usually not for desmin. Demonstration of nuclear EBER is diagnostic.

Tumour behaviour is unpredictable, and multifocal lesions do not necessarily indicate metastasis, but are a feature of this tumour

Click to access Pubmed
Timmons CF, Dawson DB, Richards CS, Andrews WS, Katz JA (1995)
Epstein-Barr virus-associated leiomyosarcomas in liver transplantation recipients. Origin from either donor or recipient tissue.
Cancer 76: 1481-9



. In some cases, antiviral treatment and restoration of immune response have resulted in tumour regression and long-term survival
Click to access Pubmed
Bonatti H, Hoefer D, Rogatsch H, Margreiter R, Larcher C, Antretter H (2005)
Successful management of recurrent Epstein-Barr virus-associated multilocular leiomyosarcoma after cardiac transplantation.
Transplant Proc 37: 1839-44




Click for details
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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