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  Table of Contents  
Year : 2011  |  Volume : 48  |  Issue : 3  |  Page : 381-383

Mucoepidermoid carcinoma of the sublingual gland associated with Sjögren's syndrome

1 Department of Reconstructive and Diagnostic Surgical Sciences, Unit of Oral Pathology and Medicine, University of Milan, Milan, Italy
2 Italian Association for Metals and Biocompatibility Research- A.I.R.M.E.B., Milan, Italy
3 Unit of Anatomical Pathology, IRCCS Maggiore Hospital, Mangiagalli and R. Elena Foundation, Milan, Italy

Date of Web Publication14-Sep-2011

Correspondence Address:
G P Bombeccari
Department of Reconstructive and Diagnostic Surgical Sciences, Unit of Oral Pathology and Medicine, University of Milan, Milan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.84933

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How to cite this article:
Bombeccari G P, Guzzi G P, Pallotti F, Spadari F. Mucoepidermoid carcinoma of the sublingual gland associated with Sjögren's syndrome. Indian J Cancer 2011;48:381-3

How to cite this URL:
Bombeccari G P, Guzzi G P, Pallotti F, Spadari F. Mucoepidermoid carcinoma of the sublingual gland associated with Sjögren's syndrome. Indian J Cancer [serial online] 2011 [cited 2021 Jul 29];48:381-3. Available from: https://www.indianjcancer.com/text.asp?2011/48/3/381/84933


Patients with primary Sjögren's syndrome (pSS) have an increased risk for development of non-Hodgkin's lymphoma. [1] Associations between pSS and other malignant tumors such as myeloid myeloma, oral cancer, breast cancer, and thymoma have been reported in literature. [2] Here, we describe an unusual case of low-grade mucoepidermoid carcinoma (MEC) of the sublingual gland in a subject, with coexisting pSS. In May 2004, a 70-year-old man presented to our department showing clinical evidence of a lump in the floor of the mouth. On oral examination, the raised area measured 2.0 cm per 1.5 cm, and he had no symptoms. Both the Wharton's ducts were not impaired, and no teeth mobility was observed. He had persistent hypertension, for which he was taking calcium antagonists. The patient was an ex-smoker. His medical history reported diagnostic positivity for pSS since the onset of the disease nine years ago, with marked signs and symptoms of oral as well as ocular dryness, associated with serum for autoantibodies Ro / La and hypergammaglobulinemia. He displayed mild normochromic-normocytic anemia, likely secondary to the pSS-related chronic inflammation condition. No extra-glandular manifestations of pSS were reported. He received palliative treatment consisting of saliva substitutes for the management of xerostomia, and topical antimycotic agents were used as fungal prophylaxis. Thus, we performed an incisional biopsy, including healthy tissue in the biopsy specimens. Histological analysis of the lesions revealed a low-grade, well-differentiated MEC, involving the right sublingual salivary gland [Figure 1]. In order to improve the accuracy of the conventional histological grading, we performed an immunohistochemical stain with an MIB-1 monoclonal antibody, which detected Ki-67 cell-cycle antigen in formalin-fixed paraffin-embedded tissue sections, to study the cell proliferation grade. The percentage of the positive neoplastic nuclei was estimated after scanning the entire tumor surface at low power (×10 objective), including areas of the highest and lowest positivity [Figure 2]. The percentage of MIB 1-positive proliferating cells turned out to be lower than 10%. At the same time, on other paraffin sections of the sample, we analyzed the HER-2 / neu expression, whose intensity of immunostaining appeared negative [Figure 3]. The patient underwent surgical excision of the lesion with no lateral lymph node removal. On analyzing the five histological factors of the Auclair grading system, neural invasion, necrosis, and anaplasia were absent, whereas, the mitotic index resulted in one mitosis for 10 high-power-fields and the intracystic component appeared to be > 20%. Interestingly, predictor risk factors for lymphoma development, such as, hypocomplementemia, skin vasculitis, and CD4+ T-cell depletion were no detectable. [3] It seems conceivable that a longstanding deficiency in immune surveillance finally allows malignant transformation in antigen-stimulated proliferating B cells. [4] We observed a mild decline over time of IgM and a slightly most marked decline of IgG serum concentrations at the development of MEC, with respect to those displayed at diagnosis. At present, it is not clear whether such an outcome reflects a risk of developing malignancies or is more linked to the diminishing inflammatory activity of pSS. [5] In addition, abnormal serum levels of immunoglobulin free light chains were not present. At the time of the last follow-up, five years after the initial successful surgical excision, the patient showed no clinical evidence of a relapse. The incidence of major salivary glands MEC is about 45%, mainly in the parotid gland, whereas, it is uncommon in the sublingual gland, accounting for approximately <1% of all MEC manifestations. [6] To the best of our knowledge, this is the first case of sublingual gland MEC in the pSS environment, longitudinally monitored. Our report adds strength to the hypothesis that there may be a subgroup of pSS patients who are at a greater risk of developing a non-lymphoid cancer in the oral cavity. [2] A long-term evaluation of the above-mentioned predictor variables for cancer development is mandatory, given the significant risk that increases over time. With regard to this, a strict surveillance would be advocated for patients affected by Sjögren's syndrome, treated with potentially cancerogenic disease-modifying medications.
Figure 1: Tumoral nest composed of a mixture of well-differentiated squamous and mucinous cells (H and E, ×40)

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Figure 2: Ki-67 immunostain (×10) showing low (8%) proliferative index

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Figure 3: Her-2 / neu immunostain (×10) completely negative

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  References Top

1.Lazarus MN, Robinson D, Mak V, Moller H, Isenberg DA. Incidence of cancer in a cohort of patients with primary Sjögren's syndrome. Rheumatology 2006;45:1012-5.  Back to cited text no. 1
2.Zhang W, Feng S, Yan S, Zhao Y, Li M, Sun J, et al. Incidence of malignancy in primary Sjogren's syndrome in a Chinese cohort. Rheumatology 2010;49:571-7.  Back to cited text no. 2
3.Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren's syndrome: A cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 2006;65:796-803.  Back to cited text no. 3
4.Zeher M, Szodoray P, Gyimesi E, Szondy Z. Correlation of increased susceptibility to apoptosis of CD4+ T cells with lymphocyte activation and activity of disease in patients with primary Sjögren's syndrome. Arthritis Rheum 1999;42:1673-81.  Back to cited text no. 4
5.Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternak A. A longitudinal cohort study of Finish patients with primary Sjogren's syndrome: Clinical, immunological, and epidemiological aspects. Ann Rheum Dis 2001;60:467-72.  Back to cited text no. 5
6.Regezi JA, Sciubba JJ, Jordan RC. Salivary glands diseases. Oral Pathology and Medicine, Clinical Pathologic Correlations. 5th ed. Philadelphia: Saunders Press; 2008. p. 249-69.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]

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