Premium
Molecular and Clinicopathological Analysis of Epstein‐Barr Virus–Associated Posttransplant Smooth Muscle Tumors
Author(s) -
Jonigk D.,
Laenger F.,
Maegel L.,
Izykowski N.,
Rische J.,
Tiede C.,
Klein C.,
MaeckerKolhoff B.,
Kreipe H.,
Hussein K.
Publication year - 2012
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/j.1600-6143.2012.04011.x
Subject(s) - medicine , pi3k/akt/mtor pathway , epstein–barr virus , lung , transcription factor , leiomyosarcoma , microrna , virus , cancer research , pathology , signal transduction , immunology , biology , gene , genetics
Epstein‐Barr virus (EBV)–associated posttransplant smooth muscle tumors (PTSMT) are very rare complications. We aimed to provide a clinicopathological characterization which is based on our own case series (n = 5) as well as previously reported PTSMT cases (n = 63). Meta‐analysis of PTSMT and molecular analysis of tumor cells from our cohort was performed. Most PTSMT developed in kidney‐transplanted patients (n = 41/68, 60%). Liver/transplant liver was the main site of manifestation (n = 38/68, 56%). Tumors occurred after a median interval of 48 months (range 5–348) and developed earlier in children than in adults. Most tumors showed no marked cellular atypia, low mitosis rate and no tumor necrosis. Gene expression analysis of 20 EBV‐related genes, including two microRNAs, revealed overexpression of MYC (p = 0.0357). Therapy was mainly based on surgical resection or reduced immunosuppression but no significant differences in overall survival were evident. Lower overall survival was associated with multiorgan involvement (n = 33/68, 48.5%) and particularly with intracranial PTSMT manifestation (n = 7/68, 10%; p < 0.02), but not transplant involvement (n = 11/68, 16%). In summary, PTSMT differ from conventional leiomyosarcomas by their lack of marked atypia, unusual sites of involvement and defining EBV association. Surgery and reduced immunosuppression show comparable clinical results and prognosis is associated with intracranial manifestation.