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

Keywords:
Smooth muscle tumours, Epstein-Barr virus, Immunosuppression
1.
Shroff R, Rees L: The post-transplant lymphoprolipherative disorder - a literature review. Pediatr Nephrol 2004;19:369-377.
2.
Jonigk D, Laenger F, Maegel L, Izykowski N, Rische J, Tiede C, Klein C, Maecker-Kolhoff B, Kreipe H, Hussein K: Molecular and clinicopathological analysis of Epstein-Barr virus-associated posttransplant smooth muscle tumors. Am J Transplant 2012;12:1908-1917.
3.
Ramdial PK, Sing Y, Deonarain J, Vaubell JI, Naicker S, Sydney C, Hadley LG, Singh B, Kiratu E, Gundry B, Sewram V: Extra-uterine myoid tumours in patients with acquired immunodeficiency syndrome: a clinicopathological reappraisal. Histopathology 2011;59:1122-1134.
4.
McClain KL, Leach CT, Jenson HB, Joshi VV, Pollock BH, Parmley RT, DiCarlo FJ, Chadwick EG, Murphy SB: Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. N Engl J Med 1995;332:12-18.
5.
Suankratay C, Shuangshoti S, Mutirangura A, Prasanthai V, Lerdlum S, Shuangshoti S, Pintong J, Wilde H: Epstein-Barr virus infection-associated smooth-muscle tumors in patients with AIDS. Clin Infect Dis 2005;40:1521-1528.
6.
Reyes C, Abuzaitoun O, De Jong A, Hanson C, Langston C: Epstein-Barr virus-associated smooth muscle tumors in ataxia-telangiectasia: a case report and review. Hum Pathol 2002;33:133-136.
7.
Hatano M, Takada H, Nomura A, Ohga S, Ohshima K, Saeki I, Tajiri T, Taguchi T, Suita S, Hara T: Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency. Pediatr Pulmonol 2006;41:371-373.
8.
Petrilli G, Lorenzi L, Paracchini R, Ubiali A, Schumacher RF, Cabassa P, Facchetti F: Epstein-Barr virus-associated adrenal smooth muscle tumors and disseminated diffuse large B-cell lymphoma in a child with common variable immunodeficiency: a case report and review of the literature. Int J Surg Pathol 2011, DOI: 10.1177/1066896911399901.
9.
Mierau GW, Greffe BS, Weeks DA: Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome. Ultrastruct Pathol 1997;21:301-305.
10.
Tulbah A, Al-Dayel F, Fawaz I, Rosai J: Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report. Am J Surg Pathol 1999;23:473-476.
11.
Shaw RK, Issekutz AC, Fraser R, Schmit P, Morash B, Monaco-Shawver L, Orange JS, Fernandez CV: Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency. Blood 2012;119:4009-4012.
12.
Atluri S, Neville K, Davis M, Robertson KA, Marshalleck FE, O'Malley DP, Buckley RH, Nelson RP Jr: Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency disease. J Pediatr Hematol Oncol 2007;29:166-172.
13.
Monforte-Muñoz H, Kapoor N, Saavedra JA: Epstein-Barr virus-associated leiomyomatosis and posttransplant lymphoproliferative disorder in a child with severe combined immunodeficiency: case report and review of the literature. Pediatr Dev Pathol 2003;6:449-457.
14.
Chaves NJ, Kotsimbos TC, Warren MA, McLean CA, Spelman DW, Williams TJ, Snell GI, Westall GP: Cranial leiomyosarcoma in an Epstein-Barr virus (EBV)-mismatched lung transplant recipient. J Heart Lung Transplant 2007;26:753-755.
15.
Rougemont AL, Alfieri C, Fabre M, Gorska-Flipot I, Papp E, Champagne J, Phan V, Fournet JC, Sartelet H: Atypical Epstein-Barr virus (EBV) latent protein expression in EBV-associated smooth muscle tumours occurring in paediatric transplant recipients. Histopathology 2008;53:363-367.
16.
Purgina B, Rao UN, Miettinen M, Pantanowitz L: AIDS-related EBV-associated smooth muscle tumors: a review of 64 published cases. Patholog Res Int 2011;2011:561548.
17.
Kingma DW, Shad A, Tsokos M, Fest T, Otsuki T, Frekko K, Werner E, Werner A, Magrath I, Raffeld M, Jaffe ES: Epstein-Barr virus (EBV)-associated smooth-muscle tumor arising in a post-transplant patient treated successfully for two PT-EBV-associated large-cell lymphomas. Case report. Am J Surg Pathol 1996;20:1511-1519.
18.
Ong KW, Teo M, Lee V, Ong D, Lee A, Tan CS, Vathsala A, Toh HC: Expression of EBV latent antigens, mammalian target of rapamycin, and tumor suppression genes in EBV-positive smooth muscle tumors: clinical and therapeutic implications. Clin Cancer Res 2009;15:5350-5358.
19.
Shen Q, Feng W, Long MS, Duan X, Jaijakul S, Arias CA, Brown RE, Zhao B: Multicentric hepatic EBV-associated smooth muscle tumors in an AIDS patient: a case report, investigation of mTOR activation and review of the literature. Int J Clin Exp Pathol 2011;4:421-429.
20.
Jimenez-Heffernan JA, Hardisson D, Palacios J, Garcia-Viera M, Gamallo C, Nistal M: Adrenal gland leiomyoma in a child with acquired immunodeficiency syndrome. Pediatr Pathol Lab Med 1995;15:923-929.
21.
Somers GR, Tesoriero AA, Hartland E, Robertson CF, Robinson PJ, Venter DJ, Chow CW: Multiple leiomyosarcomas of both donor and recipient origin arising in a heart-lung transplant patient. Am J Surg Pathol 1998;22:1423-1428.
22.
Sunde J, Chetty-John S, Shlobin OA, Boice CR: Epstein-Barr virus-associated uterine leiomyosarcoma in an adult lung transplant patient. Obstet Gynecol 2010;115:434-436.
23.
Micheletti AR, Macedo AC, Silva GB, Silva AC, Silva-Vergara ML, Murta EF, Adad SJ: Benign and malignant neoplasias in 261 necropsies for HIV-positive patients in the period of 1989 to 2008. Rev Inst Med Trop Sao Paulo 2011;53:309-314.
24.
LeBoit PE, Burg G, Weedon D, Sarasin A (ed): Pathology and Genetics of Tumours of the Skin. WHO Classification of Tumours. Lyon, IARC, 2005.
25.
Fletcher CDM, Unni KK, Mertens F (eds): Pathology and Genetics of Tumours of Soft Tissue and Bone. WHO Classification of Tumours. Lyon, IARC, 2002.
26.
Tavassoéli FA, Devilee P (eds): Pathology and Genetics of Tumours of the Breast and Female Genital Organs. WHO Classification of Tumours. Lyon, IARC, 2003.
27.
Toh HC, Teo M, Ong KW, Lee V, Chan E, Lee AS, Vathsala A: Use of sirolimus for Epstein-Barr virus-positive smooth-muscle tumour. Lancet Oncol 2006;7:955-957.
28.
Belingheri M, Comoli P, Locatelli F, Baldanti F, Martina V, Giani M, Ferraresso M, Cro L, Edefonti A, Ghio L: Successful medical treatment of EBV smooth muscle tumor in a renal transplant recipient. Pediatr Transplant 2010;14:E101-E104.
© 2013 S. Karger AG, Basel
2013
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.