Skip to main content

Advertisement

SpringerLink
Log in

Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature

  • Brief Report
  • Published:
Virchows Archiv Aims and scope Submit manuscript

Abstract

Thymoma-associated multiorgan autoimmunity (TAMA) is a recently delineated and rare paraneoplastic syndrome reported in patients with thymoma. The disorder is characterized by graft-versus-host disease-like pathology affecting the skin, gastrointestinal tract (GIT), and liver, and is usually associated with a poor outcome. We document a case of TAMA with exclusive GIT involvement which included the stomach, small and large bowel, presenting in a 66-year-old male patient 5 years after complete resection of a type B2 thymoma. A brief review is provided of this scarce syndrome, the GIT pathology described in the 21 TAMA cases reported to date, and the unique characteristics of patients with exclusive GIT involvement by this acquired autoimmune disorder.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Wadhera A, Maverakis E, Mitsiades N et al (2007) Thymoma-associated multiorgan autoimmunity: a graft-versus-host-like disease. J Am Acad Dermatol 57(4):683–689. https://doi.org/10.1016/j.jaad.2007.02.027

    Article  PubMed  Google Scholar 

  2. Kornacki S, Hansen FC III, Lazenby A (1995) Graft-versus-host-like colitis associated with malignant thymoma. Am J Surg Pathol 19(2):224–228. https://doi.org/10.1097/00000478-199502000-00011

    Article  PubMed  CAS  Google Scholar 

  3. Sader C, Sharma S, Edwards MG (2002) Graft-versus-host disease-type colitis: an unusual association of malignant thymoma. Ann Thorac Surg 73(6):1947–1948. https://doi.org/10.1016/S0003-4975(01)03505-6

    Article  PubMed  Google Scholar 

  4. Offerhaus GJ, Schipper ME, Lazenby AJ et al (2007) Graft-versus-host-like disease complicating thymoma: lack of AIRE expression as a cause of non-hereditary autoimmunity? Immunol Lett 114(1):31–37. https://doi.org/10.1016/j.imlet.2007.08.010

    Article  PubMed  CAS  Google Scholar 

  5. Fukushima A, Ichimura Y, Obata S et al (2017) Thymoma-associated multi-organ autoimmunity: a case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy. J Dermatol 44(7):830–835. https://doi.org/10.1111/1346-8138.13777

    Article  PubMed  CAS  Google Scholar 

  6. Holder J, North J, Bourke J et al (1997) Thymoma-associated cutaneous graft-versus-host-like reaction. Clin Exp Dermatol 22(6):287–290

    Article  PubMed  CAS  Google Scholar 

  7. Wang MH, Wong JM, Wang CY et al (2000) Graft-versus-host disease-like syndrome in malignant thymoma. Scand J Gastroenterol 35(6):667–670. https://doi.org/10.1080/003655200750023660

    Article  PubMed  CAS  Google Scholar 

  8. Hanafusa T, Azukizawa H, Kitaba S et al (2011) Diminished regulatory T-cells in cutaneous lesions of thymoma-associated multi-organ autoimmunity: a newly described paraneoplastic autoimmune disorder with fatal clinical course. Clin Exp Immunol 166(2):164–170. https://doi.org/10.1111/j.1365-2249.2011.04472.x

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  9. Umetsu SE, Brown I, Langner C, Lauwers GY (2017) Autoimmune enteropathies. Virchows Arch. https://doi.org/10.1007/s00428-017-2243-7

  10. Warren S, Nehal K, Querfeld C, Wong R, Huang J, Pulitzer M (2015) Graft-versus-host disease-like erythroderma: a manifestation of thymoma-associated multiorgan autoimmunity. J Cutan Pathol 42(10):663–668. https://doi.org/10.1111/cup.12642

    Article  PubMed  PubMed Central  Google Scholar 

  11. Weber A, Marques-Maggio E (2013) Apoptotic colonopathy under immunosuppression: mycophenolate-related effects and beyond. Pathobiology 80(6):282–288. https://doi.org/10.1159/000350758

    Article  PubMed  CAS  Google Scholar 

  12. Lee FD (1993) Importance of apoptosis in the histopathology of drug related lesions in the large intestine. J Clin Pathol 46(2):118–122. https://doi.org/10.1136/jcp.46.2.118

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  13. Gonzalez RS, Salaria SN, Bohannon CD, Huber AR, Feely MM, Shi C (2017) PD-1 inhibitor gastroenterocolitis: case series and appraisal of “immunomodulatory gastroenterocolitis”. Histopathology 70(4):558–567. https://doi.org/10.1111/his.13118

    Article  PubMed  Google Scholar 

  14. Slavik T (2012) Human immunodeficiency-related gastrointestinal pathology: a Southern African perspective with review of the literature (part 2: neoplasms and noninfectious disorders). Arch Pathol Lab Med 136(3):316–323. https://doi.org/10.5858/arpa.2011-0336-RA

    Article  PubMed  Google Scholar 

  15. Maverakis E, Goodarzi H, Wehrli LN et al (2012) The etiology of paraneoplastic autoimmunity. Clin Rev Allergy Immunol 42(2):135–144. https://doi.org/10.1007/s12016-010-8248-5

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Anatomical Pathology, Ampath Pathology Laboratories, Pretoria, South Africa

    Tomas Slavik

  2. University of Pretoria, Pretoria, South Africa

    Tomas Slavik

  3. Midstream Mediclinic Hospital, Pretoria, South Africa

    Fritz M. Potgieter

  4. Pretoria East Netcare Hospital, Pretoria, South Africa

    David Brittain

Authors
  1. Tomas Slavik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Fritz M. Potgieter
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. David Brittain
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Tomas Slavik.

Ethics declarations

The information contained in, and preparation of, this manuscript complies with the journal’s ethical standards.

Conflict of interest

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Slavik, T., Potgieter, F.M. & Brittain, D. Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature. Virchows Arch 473, 121–125 (2018). https://doi.org/10.1007/s00428-018-2304-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00428-018-2304-6

Keywords

Search

Navigation