Sjögren Syndrome Complicated by Mucosa-Associated Lymphoid Tissue Lymphoma and Lymphocytic Interstitial Pneumonia.
Bottom Line: Sjögren syndrome (SS) is an autoimmune disease with exocrine glands dysfunction and multiorgan involvement.We present a case of chronic SS in which F-18 FDG PET/CT demonstrated FDG avid intraparotid and cervical lymph nodes pathologically proven to be mucosa-associated lymphoid tissue lymphoma.In addition, the patient had bibasilar cystic changes consistent with lymphocytic interstitial pneumonia.
Affiliation: Saint Louis Univeristy , Saint Louis, MO , USA.
Sjögren syndrome (SS) is an autoimmune disease with exocrine glands dysfunction and multiorgan involvement. It is associated with increased risk of lymphoproliferative disorders, especially B-cell marginal zone lymphoma. While the role of F-18 Fluorodeoxyglucose position emission tomography/computed tomography (F-18 FDG PET/CT) for evaluation of lymphoma has been established, its use in patients with a chronic history of SS to evaluate for possible lymphoproliferative disorders or multiorgan involvement is limited. We present a case of chronic SS in which F-18 FDG PET/CT demonstrated FDG avid intraparotid and cervical lymph nodes pathologically proven to be mucosa-associated lymphoid tissue lymphoma. In addition, the patient had bibasilar cystic changes consistent with lymphocytic interstitial pneumonia.
No MeSH data available.
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Mentions: A 51-year-old female with a history of SS diagnosed 15 years ago presented with a mass on her left face. Contrast-enhanced computed tomography (CCT) demonstrated fatty atrophy of the parotid glands (Figure 1, yellow arrow), predominately enhancing left parotid nodule (Figure 1, green arrow), and enlarged left cervical lymph nodes (Figure 1, red arrows). Diagnostic considerations included chronic sialadenitis but lymphoma or lymphoproliferative disorder was not excluded. F-18 FDG PET/CT demonstrated an FDG avid left parotid nodule with SUV Max 8.6 (Figure 2, green arrows) and cervical lymph nodes (Figure 2, red arrows), which were suspicious for lymphoma. Other considerations of FDG lesions in the salivary glands may include salivary gland tumors or lymph node metastases from squamous cell carcinoma although these would be less likely in this particular patient. In addition, CT lung window of the F-18 FDG PET/CT showed non-FDG avid bibasilar thin-walled lung cysts, consistent with LIP (Figure 3, blue arrowheads). CT guided fine needle aspiration of the left parotid gland nodule demonstrated medium to intermediate size atypical lymphocytes. The atypical lymphocytes were found to be positive for PAX5 and negative for CD3, CD10, and CD5 with kappa restriction on flow cytometric analysis. On histological evaluation, a lymphoepithelial lesion with atypical lymphocytes of above mentioned immunophenotype is noted and a diagnosis of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) was rendered (Figure 4). This case demonstrates SS-associated LIP complicated by MALT lymphoma. The patient would be followed monthly and treatment options included single agent Rituxan or an immunochemotherapy regimen, such as bendamustine/rituximab. The patient is currently receiving hydroxychloroquine therapy for SS and monitoring of lymphoma, also follow-up F-18 FDG PET/CT was recommended per the oncologist.
No MeSH data available.