Benign lymphoepithelial lesion
- Mikulicz syndrome redirects here.
|Benign lymphoepithelial lesion|
|Classification and external resources|
Historically, bilateral parotid and lacrimal gland enlargement was characterized by the term Mikulicz's disease if the enlargement appeared apart from other diseases. If it was secondary to another disease, such as tuberculosis, sarcoidosis, lymphoma, and Sjögren's syndrome, the term used was Mikulicz's syndrome. Both names derive from Jan Mikulicz-Radecki, the Polish surgeon best known for describing these conditions.
Today, the terms "Mikulicz's disease" and "Mikulicz's syndrome" are viewed as ambiguous and outdated by some sources.
In 80% of cases, the parotid gland is affected. Lacrimal glands are also affected.
Benign lymphoepithelial lesion is most likely to occur in adults around 50 years of age. There is a predilection for gender with 60% - 80% being female. The gland affected has a diffuse swelling. The swelling can be asymptomatic, but mild pain can also be associated. There is a preponderance of this disease in those who suffer from HIV infection.
Most cases of benign lymphoepithelial lesions appear in conjunction with Sjögren's syndrome. When Sjögren's syndrome is present, the swelling is usually bilateral. Otherwise, the affected glands are usually only on one side of the body.
There is a marked lymphoplasmacytic infiltration. Lymphoid follicles surround solid epithelial nests, giving rise to the 'epimyoepithelial islands', that are mainly composed of ductal cells with occasional myoepithelial cells. Excess hyaline basement membrane material is deposited between cells, and there is also acinar atrophy and destruction.
Treatment usually consists of observation unless the patient has concern, there is pain, drainage, or other symptoms related to the lesion. Surgical removal of the affected gland would be recommended in those cases. Another treatment option would be aspiration, which can be repeated multiple times. This is commonly performed in those who are debilitated or in those whose benefit from surgery would be outweighed by the risks. Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset.
- synd/2087 at Who Named It? - "Mikulicz's disease"
- synd/2088 at Who Named It? - "Mikulicz's syndrome"
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- Azzopardi, JG; Evans, DJ (Nov 1971). "Malignant lymphoma of parotid associated with Mikulicz disease (benign lymphoepithelial lesion)". J Clin Pathol. 24 (8): 744–752. PMC 477147.
- Yamamoto, M; Harada, S; Ohara, M; Suzuki, C; Naishiro, Y; Yamamoto, H; Takahashi, H; Imai, K (Feb 2005). "Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome.". Rheumatology (Oxford, England) 44 (2): 227–34. doi:10.1093/rheumatology/keh447. PMID 15509627.
- Delaney, William E.; Balogh, Károly. "Carcinoma of the parotid gland associated with benign lymphoepithelial lesion (Mikulicz's disease) in Sjögren's syndrome". Cancer 19 (6): 853–860. doi:10.1002/1097-0142(196606)19:6<853::AID-CNCR2820190617>3.0.CO;2-N.
- Mikulicz syndrome on The National Organization for Rare Disorders (NORD)
- Benign lymphoepithelial lesions on Radiopedia