Epstein-Barr Virus-Associated Smooth Muscle Tumours after Transplantation, Infection with Human Immunodeficiency Virus and Congenital Immunodeficiency Syndromes

作者: Kais Hussein , Britta Maecker-Kolhoff , Frank Donnerstag , Florian Laenger , Hans Kreipe

DOI: 10.1159/000351326

关键词:

摘要: Smooth muscle tumours (SMT) after transplantation (PTSMT) or associated with congenital immunodeficiency syndromes (CI-SMT) and human virus (HIV-SMT) are rare. The majority of PTSMT CI-SMT Epstein-Barr (EBV), while some HIV-SMT can be EBV-negative. SMT in immunodeficient states may present unspecific symptoms which mainly related to tumour localisation. In PTSMT, >50% manifest the liver/transplant liver, but general occur at any site as single multiple tumours. Multiple manifestations do not define metastatic disease synchronously and/or metachronously. originate from recipient well donor. Morphologically, most tumours, particular lack marked histological atypia necrosis, sarcoma-like variants, histomorphology does predict clinical aggressiveness tumourbiological behaviour. surgery reduced immunosuppression show comparable overall survival rates, poor prognosis is intracranial manifestation non-resectable CI-SMT, should performed. all 3 types, adverse comorbidities extent size.

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