Skip to main content
Log in

Mikulicz syndrome associated with a malignant large cell gastric lymphoma

A case report and review of the literature

  • Case Report
  • Published:
International Journal of Gastrointestinal Cancer Aims and scope Submit manuscript

Abstract

There is much confusion in the literature regarding the differences between Mikulicz disease and Mikulicz syndrome. This may be because there seems to be a connection between the disease and disease processes associated with the syndrome. This article provides historical data discussing the emergence of Mikulicz disease, confusions regarding its definition, and finally offers an explanation to the interrelationships of these two entities. This case report hypothesizes that the non-Hodgkin’s lymphoma (NHL), which in association with Sjögren’s syndrome and Mikulicz disease in this patient comprised the Mikulicz syndrome, may have transformed into his malignant large-cell gastric lymphoma. It supports conclusions in the literature that Mikulicz disease, benign lymphoepithelial lesion, and Sjögren’s syndrome may all be a part of the same disease process as graduated variants of one another, with malignant lymphoma being a recognized complication of these entities.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Mikulicz J. Ueber eine eigenartige symmetrische. Erkrankung der Thanen and Mundspeicheldrusen, Beitr 2. Chir Festscher Theodor Billroth. 1892, p. 610–630.

  2. Howard CP. Mikulicz disease and allied conditions. Int Clin 1909;1:3–18.

    Google Scholar 

  3. Jacobsen AW, Schaffer JT. Mikulicz syndrome: the report of ten cases. Am J Dis Child 1927;34:327–332.

    Google Scholar 

  4. Godwin JT. Benign lymphoepithelial lesions of parotid gland: report of 11 cases. Cancer 1952;5:1089–1093.

    Article  PubMed  CAS  Google Scholar 

  5. Morgan W, Cattleman B. A clinicopathologic study of Mikulicz’s diseass. Am J Pathol 1953;29:471–503.

    PubMed  CAS  Google Scholar 

  6. Sjögren HS. Zur Kenntnis der keratoconjuctivitis sicca (Keratitis folliformis bei hypofunktion der tranen drusen). Acta Ophthlalmolog 1933;2:1–151.

    Google Scholar 

  7. Penfold CN. Mikulicz Syndrome. J Oral Maxillofacial Surg 1985;43(11):900–905.

    Article  CAS  Google Scholar 

  8. Chometre G, Guilbert F, Auriol M, Vaillant JM. Lymphoma of the salivary glands. New nosologic frontiers. Rev Stomatol Chir Maxillo-Faciale 1991;1:32–35.

    Google Scholar 

  9. Eck M, Schmauber B, Haas R, Greiner A, et al. MALT-type lymphoma of the stomach is associated with Helicobacter pylori strains expressing the CagA protein. Gastroenterology 1997;112:1482–1486.

    Article  PubMed  CAS  Google Scholar 

  10. Blaser MJ, Perez-Perez GI, Klenthous H, et al. Infection with Helicobacter pylori strains possessing CagA is associated with an increased risk of developing adenocarcinoma of the stomach. Cancer Res 1995;55:211–2115.

    Google Scholar 

  11. Parsonnet J, Hansen S, Rodriguez L, et al. Helicobacter pylori infection and gastric lymphoma. N Engl J Med 1994;330:1267–1271.

    Article  PubMed  CAS  Google Scholar 

  12. Tursi A, Cammarota G, Papa A, et al. Long-term follow-up of disappearance of gastric mucosa-associated lymphoid tissue after anti-Helicobacter pylori therapy. Am J Gastroenterol 1997;92:1849–1852.

    PubMed  CAS  Google Scholar 

  13. Weber DM, Dimopoulos MA, Anandu DP, et al. Regression of gastric lymphoma of mucosa-associated lymphoid tissue with antibiotic therapy for Helicobacter pylori. Gastroenterology 1994;107:1835–1838.

    PubMed  CAS  Google Scholar 

  14. Wotherspoon AC, Doglioni C, Diss TC, et al. Regression of primary low-grade B gastric lymphoma of mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori infection. Lancet 1993;342:575–577.

    Article  PubMed  CAS  Google Scholar 

  15. Isaacson PG. Recent developments in our understanding of gastric lymphoma. Am J Surg Pathol 1996;20(suppl 1):S1-S7.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Anastasios A. Mihas MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mihas, A.A., Lawson, P.B., Dreiling, B.J. et al. Mikulicz syndrome associated with a malignant large cell gastric lymphoma. Int J Gastrointest Canc 33, 123–127 (2003). https://doi.org/10.1385/IJGC:33:2-3:123

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1385/IJGC:33:2-3:123

Key Words

Navigation