Bone Diseases 3

Question 1

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

Answer 1

Cherubism

Question 2

What else should be considered radiographically when there are multiple multilocular radiolucencies besides cherubism?

Answer 2

Nevoid basal cell carcinoma syndrome

Question 3

How does cherubism progress over time?

Answer 3

Resolves

Question 4

Histology of _____:
 Similar to central giant cell tumor
 Variable numbers of multinucleated giant cells
 Extravasated RBCs
 Eosinophilic, cuff-like deposits surrounding small blood vessels

Answer 4

Cherubism

Question 5

What has the same histology as cherubism?

Answer 5

Giant cell tumor

Question 6

 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

Answer 6

Central giant cell granuloma

Question 7

Tx of \_\_\_\_\_\_\_:
Curettage – 15 to 20% recurrence
Intralesional corticosteroids
Calcitonin
Interferon alpha 2a

Answer 7

CENTRAL GIANT

CELL GRANULOMA

Question 8

Histo of \_\_\_\_\_\_\_\_\_\_\_
 Cellular tumor stroma composed of benign, mononuclear cells
 Multinucleated giant cells 
- osteoclasts
 Extravasated RBCs
 Hemosiderin - brownish

Answer 8

Central giant cell granuloma

Question 9

What 4 things should be included in the HISTOLOGIC DIFFERENTIAL DIAGNOSIS of the CENTRAL GIANT CELL GRANULOMA?

Answer 9

Central giant cell lesion
Cherubism
Hyperparathyroidism (brown tumor)
Giant Cell Tumor

Question 10

True neoplasm
Epiphyses of long bones of 
older adults
Metastasis of histologically
benign tumor to lung

Answer 10

Giant cell tumor

Question 11

 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)

Answer 11

LANGERHANS CELL

HISTIOCYTOSIS (LCH)

Question 12

_____ LCH – solitary lesion in one bone, no soft tissue or organ involvement
(eosinophillic granuloma)

Answer 12

 Chronic focal LCH

Question 13

______ LCH – multiple bones, organs, lymph nodes and skin (Hand-Schuller-
Christian disease)

Answer 13

 Chronic disseminated LCH

Question 14

______ LCH – involves most organs, lymph nodes, bone marrow and skin of
infants (Letterer-Siwe disease)

Answer 14

 Acute disseminated

Question 15

What two things present as “floating teeth”?

Answer 15

Langerhan cell histiocytosis

Cherubism

Question 16

 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”

Answer 16

CHRONIC FOCAL LANGERHANS
CELL HISTIOCYTOSIS

“Eosinophilic granuloma”

Question 17

 Hand-Schuller-Christian disease
 Most common in children under 10 years
 Bone lesions similar to chronic focal form and soft tissue lesions

Answer 17

CHRONIC DISSEMINATED

LANGERHANS CELL HISTIOCYTOSIS

Question 18

 Letterer-Siwe disease
 Infants
 Rapidly fatal course (if not treated properly)
 Disseminated involvement of organs, lymph nodes, bone marrow
and skin by anaplastic cells

Answer 18

ACUTE DISSEMINATED LANGERHANS

CELL HISTIOCYTOSIS

Question 19

If you see punched out radiolucencies in a older pt, what is the diagnosis?

Answer 19

Multiple myeloma

Question 20

If you see punched out radiolucencies in a younger pt, what is the diagnosis?

Answer 20

Langerhans cell histiocytosis

Question 21

Histology of _______;
 Sheets of large histiocytic cells with eosinophilic cytoplasm and
centrally-placed nuclei
 Focal abundant infiltrates of eosinophils
 Birbeck granules

Answer 21

Langerhans cell histiocytosis

Question 22

Tx of \_\_\_\_\_\_:
Focal chronic forms – curettage
Diffuse forms – chemotherapy
Long-term follow-up for recurrence and development 
of new lesions

Answer 22

Langerhans cell histiocytosis