Bone Diseases 3 Flashcards
Autosomal dominant
Bilateral jaw lucencies with giant cell histology
Variable clinical course
-Orbits pushed upwards due to bilateral maxilla expansion
-Chubby face
-Affects eruption and position of teeth
-Multiple multilocular radiolucencies
Cherubism
What else should be considered radiographically when there are multiple multilocular radiolucencies besides cherubism?
Nevoid basal cell carcinoma syndrome
How does cherubism progress over time?
Resolves
Histology of _____:
Similar to central giant cell tumor
Variable numbers of multinucleated giant cells
Extravasated RBCs
Eosinophilic, cuff-like deposits surrounding small blood vessels
Cherubism
What has the same histology as cherubism?
Giant cell tumor
Young individuals
- 10 to 30 years
Females
Unilocular or multilocular lucency
Anterior jaw lesion, often crosses midline
Cortical expansion, perforation uncommon, resorption of tooth roots
Central giant cell granuloma
Tx of \_\_\_\_\_\_\_: Curettage – 15 to 20% recurrence Intralesional corticosteroids Calcitonin Interferon alpha 2a
CENTRAL GIANT
CELL GRANULOMA
Histo of \_\_\_\_\_\_\_\_\_\_\_ Cellular tumor stroma composed of benign, mononuclear cells Multinucleated giant cells - osteoclasts Extravasated RBCs Hemosiderin - brownish
Central giant cell granuloma
What 4 things should be included in the HISTOLOGIC DIFFERENTIAL DIAGNOSIS of the CENTRAL GIANT CELL GRANULOMA?
Central giant cell lesion
Cherubism
Hyperparathyroidism (brown tumor)
Giant Cell Tumor
True neoplasm Epiphyses of long bones of older adults Metastasis of histologically benign tumor to lung
Giant cell tumor
Neoplastic proliferation of Langerhans histiocytes - clonal
Wide spectrum of involvement and biologic behavior – from solitary bone lesions to
generalized bone lesions with organ and soft tissue lesions
Three categories for treatment and prognostic purposes
Chronic focal – solitary lesion in one bone, no soft tissue or organ involvement
(eosinophillic granuloma)
Chronic disseminated – multiple 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)
LANGERHANS CELL
HISTIOCYTOSIS (LCH)
_____ LCH – solitary lesion in one bone, no soft tissue or organ involvement
(eosinophillic granuloma)
Chronic focal LCH
______ LCH – multiple bones, organs, lymph nodes and skin (Hand-Schuller-
Christian disease)
Chronic disseminated LCH
______ LCH – involves most organs, lymph nodes, bone marrow and skin of
infants (Letterer-Siwe disease)
Acute disseminated
What two things present as “floating teeth”?
Langerhan cell histiocytosis
Cherubism
Most common form Teenagers and young adults Area of discomfort Variation in radiographic appearance Punched out lucency Periapical pathosis Advanced periodontal disease – “teeth floating in air”
CHRONIC FOCAL LANGERHANS
CELL HISTIOCYTOSIS
“Eosinophilic granuloma”
Hand-Schuller-Christian disease
Most common in children under 10 years
Bone lesions similar to chronic focal form and soft tissue lesions
CHRONIC DISSEMINATED
LANGERHANS CELL HISTIOCYTOSIS
Letterer-Siwe disease
Infants
Rapidly fatal course (if not treated properly)
Disseminated involvement of organs, lymph nodes, bone marrow
and skin by anaplastic cells
ACUTE DISSEMINATED LANGERHANS
CELL HISTIOCYTOSIS
If you see punched out radiolucencies in a older pt, what is the diagnosis?
Multiple myeloma
If you see punched out radiolucencies in a younger pt, what is the diagnosis?
Langerhans cell histiocytosis
Histology of _______;
Sheets of large histiocytic cells with eosinophilic cytoplasm and
centrally-placed nuclei
Focal abundant infiltrates of eosinophils
Birbeck granules
Langerhans cell histiocytosis
Tx of \_\_\_\_\_\_: Focal chronic forms – curettage Diffuse forms – chemotherapy Long-term follow-up for recurrence and development of new lesions
Langerhans cell histiocytosis
