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  • Primary lymphoma of bone is an uncommon

    2019-05-07

    Primary lymphoma of bone is an uncommon clinical entity and accounts for less than 5% of malignant bone tumors, 4–5% of extra nodal lymphoma and less than 1% of all non-Hodgkin\'s lymphoma. Diffuse large-B-cell lymphoma (DLBCL) accounts for the majority of cases of PBL [2]. The varied clinical and histopathological profiles of PLB are still to be explored entirely.
    Methods A 5 year study (2006–2011) of all primary lymphomas of bone were undertaken retrospectively and prospectively in our department. Cases with unusual presentation were studied in detail along with their immunohistochemical profile. In all patients, staging evaluation included hematological and chemical survey, in addition to chest X-rays, abdominal ultrasonography, computed tomography of the chest and abdomen, and bone marrow biopsy. Cases were staged according to the Ann Arbor staging system [3]. Complete data on followup was not available due to either inability of patient to complete followup or referral of patient to some higher/cancer speciality center. The patients were treated with combined radiochemotherapy. The dose of radiation therapy was between 35 and 45Gy, and CHOP chemotherapy regimen was employed. Complete Response (CR) was defined as disappearance of all evidence of lymphoma, as documented by a normal physical examination, blood tests, and radiologic imaging. When residual radiographic abnormalities were consistent with normal bone reformation, patients were considered in CR if no other possible signs of disease were present. Partial Response (PR) was defined as >50% reduction in tumor burden without CR after completion of treatment, and no response as anything else. Local failure was defined as failure in the initial bone site and/or in adjacent lymphnodes. Failure anywhere outside these confines was considered to be distant [4].
    Observations and results Out of 20,000 bone lesions received in our department over 5 years from 2006 to 2011, 360 (1.8%) were neoplastic out of which 11 were bone lymphomas. Of the 11 cases of bone lymphomas, 6 cases were secondary involvement by non-Hodgkin\'s lymphoma and 5 cases were primary bone lymphoma. All the 5 cases of primary bone lymphoma (PBL) were Diffuse large B-cell lymphoma (DBCL) type, 3 of which presented in pelvis(60%), 1 each in RVX-208 (20%) and mandible (20%). 3 cases were of usual and 2 had unusual presentation or morphology. Table 1 summarizes the clinic-pathological characteristics of the patients described in this study. The first unusual presentation (case 4) was a 65-year-old man who presented with the chief complaints of intermittent fever, pain and swelling of the jaw for the past 6 months. On examination, multiple draining sinuses were present on right side of jaw. A CT scan of the jaw revealed a breach in the cortex of the mandible on the right side [Fig. 1]. A presumptive diagnosis of tubercular osteomyelitis was made and draining fluid was sent for AFB stain, results came were negative. A biopsy was sent for histopathological examination, which revealed diffuse infiltration of the skin, subcutaneous tissue and muscle with lymphoid cells [Fig. 2]. Immunohistochemistry was positive for LCA, CD20, Ki 67 (50–60%) and negative for CD3 and CD4 [Fig. 3]. Hence a diagnosis of diffuse large B cell lymphoma of the mandible was made and the patient was started on CHOP chemotherapy regimen followed by radiotherapy to which he is responding well. Additional investigations did not show any other bony lesions or lymphnode involvement. The second peculiar presentation (case 5) was a 54-year-male who complained of pain and swelling of right hip since 5 months with increasing intensity. On examination the patient had restricted movements of left hip joint and a lump could be palpated. X-ray showed soft tissue and bony mass involving the right ileum [Fig. 4]. A presumptive clinicoradiological diagnosis of chondrosarcoma was made and biopsy was performed. Histopathological examination showed small round cell tumor cells in a sclerotic stroma [Fig. 4]. Immunohistochemistry of the lesion showed positivity for LCA, CD 20, CD10,Vimentin, Bcl-2, Bcl-6, PAX-5, MUM1, Ki-67 (70–80%) and negative for CD 99, Pancytokeratin, Desmin, CD-3, S-100, CD-5, HMB-45 [Fig. 5]. The morphology and immunophenotype was compatible with the diagnosis of diffuse large B cell lymphoma and suggest a follicle center cell origin. So a final diagnosis of diffuse large B cell lymphoma of right ileum was made. No additional bony or lymphnode involvement was found. The patient was advised CHOP chemotherapy followed by radiotherapy.