Epstein‐Barr virus‐associated post‐transplant primary smooth muscle tumor of the liver: Report of an autopsy case

Abstract

Epstein-Barr (EB) virus-associated primary smooth muscle tumors have been reported in immunosuppressed young patients with acquired immunodeficiency syndrome (AIDS) and young people who have undergone liver transplantation. An autopsy case of EB virus-associated smooth muscle cell tumor in a 21 year old female who received immunosuppressive therapy following renal transplantation is reported. Multiple tumor nodules were present in the liver, but no primary lesion was found in any other organ. Histologically, the nodules were composed of spindle cells, positive for alpha-smooth muscle actin, which were arranged in fascicles and closely associated with vascular channels, thereby suggesting a vascular smooth muscle cell origin. EB virus infection of the tumor cells was clearly demonstrated by in situ hybridization with an EB virus-encoded RNA 1 (EBER-1) probe. The present case illustrates that EB virus infection may play some role in the development of smooth muscle tumors not only in immunocompromised young patients with liver allografts, but also in those with renal allografts.

Molecular and Clinicopathological Analysis of Epstein-Barr Virus–Associated Posttransplant Smooth Muscle Tumors

“…As could be expected from the known risk profiles of PTLD (1), most of these patients were children and the PTLD manifested significantly earlier than the PTSMT. PTSMT with associated PTLD manifest predominantly in kidney transplant patients (2,4,6,(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26), and although, PTSMT generally developed later in kidney transplanted patients, the specific type of graft was not associated with incidence of PTSMT. In contrast, PTSMT manifest more often in adults, but earlier in younger patients.…”

“…The femaleto-male ratio was 1:1.2 (no statistical significance) (2-41). The majority of PTSMT developed after kidney transplantation (n = 38/68, 60%) (2,4,6,(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26) and the site of tumor manifestation was mainly the liver/transplant liver (n = 38/68, 56%) (2,(5)(6)(7)10,11,15,(17)(18)(19)(20)(21)24,25,(27)(28)(29)(30)(31)(33)(34)(35)(36)38,41). PTSMT occurred in a total of 11 transplant Liver (n = 10/68, 15%) Heart (n = 9/68, 13%) Lung (n = 4/68, 6%) Heart + lung (n = 1/68, 2%) Bone marrow (n = 3/68, 4%)…”

“…Remarkably, the main site of tumor manifestation was the liver and the transplant liver (2,(5)(6)(7)10,11,15,(17)(18)(19)(20)(21)24,25,(27)(28)(29)(30)(31)(33)(34)(35)(36)38,41). Other solid grafts were less frequently affected; kidney transplants in particular rarely developed PTSMT, although kidneys were the most frequently transplanted organ in all patients with subsequent PTSMT (2,4,(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). We could demonstrate that the tumor cells originate from the donor in hepatic PTSMT (#1).…”

Caveolin Expression is Common among Benign and Malignant Smooth Muscle and Adipocyte Neoplasms

“…An established role for the Epstein-Barr virus (EBV) in the pathogenesis of leiomyosarcoma has been shown among immunodeficient patients (6). To the contrary, latent EBV infection is not thought to play a pathogenic role in leiomyosarcoma among immunocompetent individuals, nor is it thought to contribute to the pathogenesis of leiomyoma (7).…”

Multiple Smooth-Muscle Neoplasm and Thyroid Carcinoma in An Adult With AIDS

“…At present, new diseases associated with human immunodeficiency virus (HIV) infection continue to be recognized (1)(2)(3)(4). The tumor of smooth-muscle origin has recently been introduced to this entity.…”