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OSSIFYING FIBROMA Clinical Benign, true neoplasm Features Well demarcated(imp feature to diff.

f. from fibrous dysplasia), occasionally encapsulated Consist of : Fibrous tissue bony trabeculae rounded calcified bodies Demarcated with occasional encapsulation Clinically : Progressive swelling Slowly enlarging Mandible (premolar-molar region) Wide age range Sinonasal complex and orbit > female Juvenile Ossifying fibroma Children and adolescent Rapid growth Richly cellular , mitotically active fibrous tissue Trabeculae-immature looking woven bone d/d:osteosarcoma recurrence rate of 30-60% Histopath lesion is well ologic circumsribed Features Fibrous tissue containing trabeculae bone osteoblastic rimming spherical/rounded deposits of acellular calcified material may resemble cementum but similar tissue are found in

MYELOMA neoplasma of plasma cells Disseminated disease involving many bones (multiple myeloma or myelomatosis) Solitary (less common): solitary myeloma /plasmacytoma Jaw lesion in both (disseminated n solitary) Extramedullary plasmacytoma in oral soft tissue as diffuse/polypoid swellings Multiple myeloma : monoclonal proliferation of plasma cell s production of single type of Ig (most commonly igG) increased level in serum : paraprotein or M protein 50-70 years of age Any bone (skull, vertebrata, sternum, ribs, pelvicmost commonly affected) Diagnosis : Electrophoreses Bence John proteins : light chain in 50% of patients identified in urine Hypercalcimia amyloidoisis densely cellular sheets of myeloma cells resemblance of mature plasma cells / immediate precursor binucleated / multinucleated

LANGERHAN CELL HISTIOCYTOSIS Wide range clinical manifestation : Mosnostotic, solitary bone lesion (unifocal eosinophilic granuloma) Multifocal eosinophilic granuloma involving bone and other organs craniofacial, orbit, post pituitary gland (Hand schuller , Christian syndrome) skull defects, exophtalmus, diabetes insipidus in jaws: extensive destruction n loss/loosening teeth Disseminated multiorgan Letterer Siewe disease infant and children <2yrs high mortality Unifocal n multifocal in older children (adultless common) < 20years Male > Female (2x) Any bone involve (cranium n jaws-common sites) Mandible > maxilla Gingival ulceration/enlarge Poorly defined collection of histiocytes (large, pale staining, round indented nuclei) mixed with variable # of eosinophills Rod/raquet shaped granule (Birbeck granules) (EM) Express surface antigen: CD1-a

tumours arising in other craniofacial bones sometimes-predominant calcified tissue producedpsammatoid ossifying fibroma) d/d : fibrous dysplasia and cement-osseous dysplasia (clinical and radiographic features to distinguish) Immunohistochemistry kappa light chain (positive)

Birbeck granules

Immunohistochemistry lambda light chain(negative) Radiograp Appearance vary with the stage. Sharply demarcated hic Initial-Well demarcated radiolucent area Round /oval osteolytic lesion Mature-varying amount of calcified tissue are deposited Punched out radiolucent lesion Radiolucent, mixed Solitary / multiple osteolytic lesions Teeth floating in air in multiple eosinophilic granuloma Punched out radiolucency May simulate periapical lesion

Extra info Prognosis : Slowly growing / rapidly growing Maybe associated with hereditay hyperparathyroidism

Treatment : chemotherapy

Langerhan cells : Epidermis LN Mucisa Bone marrow APC Treatment : Curettage Radiotherapy Intralesional injection with steroid