Diseases of Bone- Giant Cell Lesions and LCH Flashcards
describe cherubism
- autosomal dominant
- bilateral jaw lucencies with giant cell histology
- variable clinical course
what is the clinical presentation for cherubism
maxillary expansion
what is the radiographic appearance of cherubism
multiple multilocular RL
what is the resolution with cherubism
- can undergo spontaneous resolution
- no need for surgery unless cosmetic
- RL will resolve and clinical expansion will resolve
what is the histo for cherubism
- similar to central giant cell tumor
- variable numbers of multinucleated giant cells
- extravasated RBCs
- eosinophilic, cuff like deposits surrounding small blood vessels
describe central giant cell granuloma, predilection
- youn individuals 10-30 years
- females
- unilocular or multilocular lucency
- anterior jaw lesion, often crosses midline
- cortical expansion, perforation uncommon, resorption of tooth roots
what is the radiographic presentation for central giant cell granuloma
- multilocular or unilocular RL
- can cause root resorption
what is the tx for central giant cell granuloma
- curettage - 15-20% recurrencee
- intralesional corticosteroids
- calcitonin
- interferon alpha2a
what is the histo presentation of central giant cell granuloma
- cellular tumor stroma composed of benign, mononuclear cells
- multinucleated giant cells: osteoclasts
- extravasated RBCs
- hemosiderin: brownish
what is the histologic differential dX for central giant cell granuloma
- central giant cell lesion
- cherubism
- hyperparathyroidism (brown tumor)
- giant cell tumor
describe the giant cell tumor of long bone
- true neoplasm
- epiphyses of long bones of older adults
- metastasis of histologically benign tumor of lung
describe langerhans cell histiocytosis
neoplastic proliferation of langerhand histiocytes- clonal
- wide spectrum of involvement and biologic behavior- from solitary bone lesions to generalized bone lesions with organ and soft tissue lesions
what are the three categories for langerhans cell histiocytosis for tx and prognosis and describe each
- chronic focal: solitary lesion in one bone, no soft tissue or organ involvement (eosinophilic granuloma)
- chronic disseminated- mulitple bones, organs, lymph nodes and skin (hand- schuller- christian disease)
- acute disseminated- involves most organs, lymph nodes, bone marrow and skin of infants ( letterer-Siwe disease)
what is the most common form of langerhans cell histiocytosis
chronic focal
describe the radiographic appearance and predilection for chronic focal langerhans cell histiocytosis
- teens and young adults
- area of discomfort
- punched out lucency
- periapical pathoses
- advanced perio disease- teeth floating in air
describe chronic disseminated langerhans cells histiocytosis and who it affects
- hand- schuller christian disease
- most common in children under 10 years
- bone lesions similar to chronic focal form and soft tissue lesions
describe acute disseminated langerhans cell histiocytosis and who it affects
- letterer Siwe disease
- infants
- rapidly fatal course
- disseminated involvement of organs, lymph nodes, bone marrow and skin by anaplastic cells
what is the ddx for langerhans cell histiocytosis and how to tell difference
- mulitple myeloma
- multiple myeloma occurs in older pt
what is the histopath for langerhans cell histiocytosis
- sheets of large histiocytic cells with eosinophilic cytoplasma and centrally placed nuclei
- focal abundant infiltrates of eosinophils
- birbeck granules
what is the staining used for langerhans cell histiocytosis
S100 - if positive it stains brown
what is the tx for langerhans cell histiocytosis
- focal chronic form- curettage
- diffuse forms- chemotheraoy
- long term folllow up for recurrence and development of new lesions
