multiple myeloma is also known as kahler 's disease , myelomatosis , and medullary plasmacytoma . it is considered to be the most common hematological malignancy after lymphoma , constituting about 10% of blood cancers . the incidence of this disease is quite variable . in the usa , it varies from 1/100,000/year among asians to 810/100,000/year among afro  americans . bone involvement secondary to bone marrow infiltration is the most common manifestation and results in osteolytic lesions , bone pains , and pathological fractures . the resorption of bone is attributed to increased osteoclastic activity with increased proliferation and survival of osteoclasts . it is now known that myeloma cells also cause inhibition of osteoblastic differentiation through the production of the dickkopf-1 protein . frequently affected sites include the vertebrae , ribs , skull , femur , clavicles , pelvis , and scapulae . jaw lesions are rarely the first sign of the disease and the incidence varies from 8 to 15% . we present here the case of a patient whose initial presentation of multiple myeloma was a mandibular swelling . a 45 year - old female presented with swelling of the left jaw of two months duration that was associated with difficulty in swallowing . local examination revealed a swelling in the left mandibular region , 2  3 cm in size , firm , fixed , and nontender with normal overlying skin . the margins were well defined and the surface was smooth ; local temperature was not raised . radiographic investigation revealed a destructive , radiolucent lesion in the posterior part of the body and ascending ramus of the left mandible . the patient 's hemoglobin was 5.1 g / dl and erythrocyte sedimentation rate was markedly raised ( 75 mm in the first two hours ) . radiological examination revealed the presence of punched - out lesions in the skull with generalized osteopenia . fine needle aspiration of the jaw swelling yielded a highly cellular aspirate with uniformly dispersed populations of abnormal plasma cells having abundant basophilic cytoplasm , eccentric , hyperchromatic nuclei with prominent nucleoli , and reticular chromatin [ figures 1 and 2 ] . the cells could be easily identified as plasma cells with their characteristic morphological appearance and therefore , the need for immunohistochemical analysis did not arise . based on the pathological picture and reports of ancillary investigations , a diagnosis of multiple myeloma was established . numerous plasma cells in varying stages of maturation ( pap , 500 ) dispersed population of myeloma cells with atypical nuclei , binucleation , prominent nucleoli , and loss of normal it is prudent to keep in mind that jaw involvement can also be asymptomatic and the jawbones are usually involved in patients with widespread skeletal involvement . such an examination can yield three different types of presentations : generalised rarefaction , multiple radiolucent areas or , on occasion , there may be absence of bony abnormality . fine needle aspiration is now a standard technique for the initial assessment of tumorous masses . aspiration of bone frequently results in hemorrhagic aspirates with poor cellularity , but , in general , sufficient diagnostic material can be obtained from a malignant lesion . the microscopic appearance of multiple myeloma is highly characteristic , with a monoclonal proliferation of plasma cells of variable maturity . other conditions that should be kept in mind are langerhans cell granulomatosis and metastatic disease . lymphoplasmacytic lymphoma is characterised by a neoplastic proliferation of malignant lymphoid cells with plasmacytoid features . however , the nuclei of the cells in lymphoplasmacytic lymphoma resemble the nuclei of lymphocytes rather than plasma cells . the cells in lymphoplasmacytic lymphoma show positivity for surface immunoglobulin , cd19 , and cd20 whereas myeloma cells are strongly positive for cd138 and negative for surface immunoglobulin and cd20 . other lymphomas that can show plasmacytoid features include b - cell chronic lymphocytic lymphoma and follicular lymphoma . langerhans cell granulomatosis can be identified without difficulty in presence of the characteristic langerhans cells with a polymorphous mixture of inflammatory cells in the background . metastasis to the mandible or the maxilla is rare but the possibility must not be overlooked . common sources of metastasis to the jawbone are the breast , lungs , kidney , and prostate . these findings have an impact on prognosis and may possibly alter current treatment protocols of this disease . multiple myeloma can be subclassified into four types : hyperdiploid , hypodiploid , pseudodiploid , and near - tetraploid . the latter three are collectively designated as non - hyperdiploid multiple myeloma. the non - hyperdiplioid ( nh - mm ) variant is characterised by primary translocation of the igh locus on chromosome 14q32 , whereas there is infrequent translocation of the igh locus in hyperdiploid multiple myeloma . it has thus been proposed that the evolution of multiple myeloma can proceed along two different or dichotomous paths . in addition , based on the pattern of igh translocation and expression of cyclin d , multiple myeloma has been divided into different categories , providing a format for a molecular classification . this has been designated as the translocation / cyclin d expression ( tc ) classification . the groups are identified as iiqb ( 16% ) , 6p21 ( 3% ) , maf , 4p16 , d1 ( 34% ) , d1 + d2 ( 6% ) d2 ( 17% ) , and none ( 2% ) . for example , patients with 4p16 have shortened survival , whereas d1 and 11q13 have better prognosis . again , lytic bone lesions are very prevalent in categories 6p21 , 11q13 , d1 , and d1 + d2 . the molecular classification of multiple myeloma may thus provide a model for predicting response to therapy and survival . however , a composite standardised format will have to be developed and brought into widespread use . novel therapeutic targets have also been identified , such as the dickkopf-1 protein and cs-1 , a cell surface glycoprotein which is expressed in multiple myeloma . exciting developments in myeloma research promise a better opportunity for survival for patients and may help to reduce morbidity and mortality . however , since the clinical presentation of the disease does not seem to be influenced to any major extent by the molecular changes , an in - depth knowledge of the varied forms of presentation is unquestionably necessary for the clinician .
multiple myeloma is a systemic b - cell lymphoproliferative disease with varied manifestations . 
 its diagnosis can therefore pose difficulties for both the clinicians and pathologists . 
 jaw lesions , though not uncommon , rarely present as the first sign in multiple myeloma . 
 we present here the case of a 45 year - old female who presented with a swelling of the jaw and on subsequent work - up , was diagnosed with multiple myeloma . 
 recent research regarding this disease has also been highlighted .