Disseminated non-Hodgkin's lymphoma presenting as bilateral salivary gland enlargement: a case report
A 73-year-old Indian female patient presented with rapidly progressive painless swelling of face, mainly localized in the parotid and submandibular region bilaterally for the duration of one year. The patient had a history of fever for the most recent two months, with unexplained weight loss (weight at the time of presentation was 35 kg). She had also noticed a recent change in her voice.\nPhysical examination revealed the patient to be moderately built and poorly nourished with signs of anemia. Ocular examination showed bilateral subconjuctival hemorrhage and puffiness of the eyelids. The spleen was also palpable 10 cm below the costal margin, whereas the liver was just barely palpable. Further systemic examination including that of the respiratory, cardiac, and central nervous systems was normal.\nExtraorally, she had a diffuse firm swelling of both the parotid and submandibular glands (), and a rubbery consistency of the cervical lymph nodes along with those of the submental, submandibular, and jugulodiagastric groups.\nIntraorally, two soft, non-tender swellings of about 2×3 cm were seen in the buccal vestibule in the region of the left upper and lower second molar. Multiple nodules were also palpable in the buccal mucosa bilaterally (). A routine hemogram showed pancytopenia with a hemoglobin level of 8.8 gm/dL, a total leukocyte count of 2.5×103/mm3 and platelet count of 1.03×103/µL. A lactate dehydrogenase level of 621 IU/L was noted and tests for human immunodeficiency virus (HIV) I and II were found to be non-reactive. Bone marrow cytology revealed an interstitial pattern of marrow involvement.\nPanoramic and chest radiographs were unremarkable. High resolution ultrasonography and color Doppler sonography studies confirmed the enlargement of the bilateral parotid and submandibular glands, and also the buccal and lingual lymph nodes (). The cervical group of lymph nodes revealed similar enlargement of multiple nodes belonging to IA, IB, IIA, and IIB levels along with the pre- and post-auricular and supraclavicular group of lymph nodes. Ultrasonography of the abdomen confirmed the enlargement of the spleen, which measured 17 cm. The porta hepatis lymph nodes were noted for enlargement and a hypoechogenic appearance.\nContrast-enhanced computed tomography confirmed the diffuse symmetrical enlargement of the parotid and submandibular glands (), while asymmetric thickening of the subglottic larynx and right aryepiglottic fold were evident with narrowing of the subglottic lumen. Prominent pharyngeal tonsils confirmed the involvement of Waldeyer's ring. The ocular adnexa of the left orbit were involved, presenting as a bulky lacrimal gland.\nFine needle aspiration cytology (FNAC) from the right parotid and submandibular glands, buccal vestibule, and tongue nodule showed a monomorphic population of lymphoid cells with the absence of Reed-Sternberg cells, which was suggestive of non-Hodgkin's lymphoma. Incisional biopsy of the right submandibular lymph node was performed, which revealed diffuse infiltration by sheets of a monomorphic population of moderately large cells, with moderate cytoplasm and large round vesicular nuclei having prominent nucleoli. Mitotic figures were frequently identified ().\nImmunohistochemistry was performed using standard techniques. The tumor cells expressed CD20 () and were immunonegative for CD10, CD3, CD5, CD23, and Cyclin D1. The Mib-1 labeling index (for proliferation marker Ki-67) was approximately 10-15%.\nDiagnosis of NHL-diffuse large B-cell type was concluded based on FNAC and biopsies of the submandibular glands, parotid glands, and lymph nodes, supported by immunohistochemical positivity for CD20 marker. The patient was referred to the oncology department regarding chemotherapy and radiotherapy.