A 62-year-old male patient presented with the chief complaints of swelling on the left side of face and neck region for 2 months, which was of sudden onset. Swelling started as a discomfort on the left side of the neck followed by the appearance of swelling, which gradually increased in size and associated pain for 1 month. Had multiple medical consultations and multiple courses of antibiotics and a course of antifilarial over the past 1 month, with no reduction in symptoms. There were no other associated signs or symptoms.
He was under medication for hypothyroidism and diabetes mellitus. History of filariasis 10 years back followed by 3-4 episodes of lymphadenitis and had antifilarial therapy during these episodes. He was a cigarette smoker for 20 years, smoking two cigarettes per day which he quit 3 years back.
Extraoral examination revealed facial asymmetry of the left side. Multiple lymph nodes were palpable, including left submandibular and level II cervical lymph node with the largest one measuring approximately 4 cm x 5 cm in size. Lymph nodes were non-tender, firm in consistency, mobile, and some were matted together. Skin over the lymph nodes appeared normal without any signs of inflammation or infection. Patient consent was obtained for taking photograph and using it for study and publication purposes [Figure 1a and b].
Intraoral examination revealed poor oral hygiene with the presence of local factors and signs of periodontitis. On hard tissue examination, tooth number 36 had a crown with tenderness on vertical percussion. Thus by history and clinical examination, a differential diagnosis of cervical lymphadenopathy due to locoregional infection, filariasis, tuberculosis, NHL was given. Patient advised to discontinue all antibiotics and antifilarial drugs.
Panoramic radiograph revealed generalized horizontal bone loss and endodontically treated 36 with crown and periapical bone density [Figure 2a]. Posterior-anterior chest radiograph was noncontributory [Figure 2b]. All hematological investigations were within the normal limit except for lactate dehydrogenase (307.8 U/L, normal range 0-248 U/L). Blood smear was negative for filarial microbes, and Mantoux test was negative. Computed tomography (CT) of the neck with contrast showed multiple discrete and conglomerate lymph nodes in left level Ib, II, III, and V. Largest node measured 3.5 cm x 4.4 cm [Figure 2c]. The nodes did not show any enhancement/necrosis and were pushing the submandibular gland to one side. However, the gland appeared normal and was separated from the nodal mass [Figure 2d]. The adjacent bones did not show any erosion/lytic change. The right side also showed level Ib, II, and V nodes, but they were discrete and showed no evidence of necrosis or any other mass lesion [Figure 2e]. A tru-cut nodal biopsy taken from cervical lymph node, microscopic features were suggestive of lymphoproliferative disorder. Immunohistochemistry (IHC) was performed to categories the lesion; cells were positive for leukocyte common antigen, CD20, some for CD30 and Epstein-Barr virus latent membrane protein-1 (EBV-LMP-1) and were negative for CD3, CD10, cyclin D1, B-cell lymphoma-6 (BCL-6), CD23, anaplastic lymphoma kinase-1 and terminal deoxynucleotidyl transferase. IHC confirmed NHL, that is, suggestive of EBV-positive diffuse large B-cell lymphoma (DLBL) of elderly. Bone aspiration from pelvic bone ruled out the bone marrow involvement. Fluoro-2-deoxyD-glucose (FDG) positron emission tomography/CT (PET/CT), showed extensive FDG avid supra and infra-diaphragmatic lymph nodal lesions indicating metabolically active NHL. Focal FDG avid within spleen were present [Figure 3a]. Thus, according to Ann Arbor staging of primary lymphoma, he had Stage III S.
A final diagnosis of NHL of DLBL was made based on the clinical presentations and investigations. The patient received six cycles of standard R-CHOP chemotherapy regimen which included rituximab 375 mg/m2, cyclophosphamide 750 mg/m2, doxorubicin 50 mg/m2, vincristine 1.4 mg/m2, and prednisone 50 mg/m2. On the 1st day of each cycle, the patient received rituximab, doxorubicin, vincristine, and cyclophosphamide and a 5-day course of prednisolone. At the end of the 21 days, patient started with second cycle of the R-CHOP chemotherapy, he completed six cycles of chemotherapy regimen. Whole-body PET/CT was done after six cycles of chemotherapy to assess the response to treatment, which showed complete metabolic and near complete anatomical resolution of supra/infra-diaphragmatic lymph nodes and splenic deposits [Figure 3b]. No new FDG avid lymph nodal/extranodal lymphomatous deposits were evident. Thus, a Deauville Score I was made suggesting an overall good response to chemotherapy and indicating no need of further chemotherapy at that level.