bone lecture 3 Flashcards
Osteoma
Benign tumors composed of mature compact or cancellous bone
Osteoma MC what region of body?
MC craniofacial region
osteomas may arise from what parts of the bone?
May arise from bone surface or within medullary bone
osteomas are distinct what from other growth?
Distinct from exostosis
Osteoma
Clinical features:
age
MC where
many/one? symptoms?
growth rate?
Large lesions may produce:
Observed in adults
MC mandibular body and condyle
Solitary and asymptomatic
Slow growth
Large lesions may produce marked facial deformity
Osteoma
Radiographic features:
Circumscribed radiopaque mass
Smaller endosteal osteomas – more discrete
dif dx
osteoma
osteoblastoma
from biopsy post man well defined/ circumscribed opacity in adult male
osteoma
Gardner syndrome
Characterized by:
Variant of?
numerous what?
inheritence/mutation?
Characterized by intestinal polyps and abnormalities of bone, teeth, skin, soft tissue
Variant of familial adenomatous polyposis
numerous intestinal polyps →colorectal cancer
AD disorder, mutations in APC (tumor suppressor gene)
Gardner syndrome
Clinical features:
Colorectal polyps develop by what decade?
symptoms?
May cause what GI disturbances?
If untreated, will transform into?
Colorectal polyps develop by 2nd decade
Often asymptomatic
May cause diarrhea, constipation, abdominal pain
If untreated, will transform into adenocarcinoma
Gardner syndrome
Clinical features:
% developing osteomas?
cysts where?
Pigmented lesions of?
90% develop skeletal abnormalities: osteomas (multiple)
Epidermoid cysts
Pigmented lesions of ocular fundus
Gardner syndrome
Dental abnormalities:
Odontomas, supernumerary teeth, impacted teeth
Gardner syndrome Treatment:
Without treatment?
Prophylactic?
Osteomas?
Dental management for?
Without treatment: 50% develop colorectal cancer by 30 years, nearly 100% by 5th decade
Prophylactic colectomy recommended
Osteomas removed for functional issues
Dental management for impacted teeth, odontoma
what are these findingd indicative of?
gardner syndrome
Osteoblastoma
occurance?
Arise from what cells?
MC where?
Rare, appx 1% of bone tumors
Arise from osteoblasts
MC in vertebral column, long bones, pelvis, facial bones, small bones of hands and feet
Osteoblastoma
Clinical features:
MC which jaw?
85% occur before?
symptoms?
Teeth?
MC in mandible
85% occur before 30 years
Dull pain, swelling
Tooth mobility, root resorption or displacement
Osteoblastoma
Radiographic features:
Mixed RL/RO, variable mineralization
May be well-defined or poorly defined
Osteoblastoma tx/recurrence
Local excision or curettage
Recurrence uncommon
dif dx
osteoma
osteoblastoma
biopsy from a ill defined opacity of the mandible, pt has dull pain
osteoblastoma
Cementoblastoma
Benign neoplasm of cementum
cementoblastoma Clinical features:
location?
50% cases arise by what age?
symptoms?
Bony expansion?
80% mandible (50% 1st molar)
50% cases arise by 20 years
Pain and swelling
Bony expansion uncommon
Cementoblastoma
Radiographic features:
Radiopaque mass that is fused to one or more tooth roots
May be surrounded by thin RL rim
Cementoblastoma
Treatment:
Surgical extraction of tooth and calcified mass
cementoblastoma
from extracted tooth
cementoblastoma
Langerhans cell histiocytosis
Proliferation of Langerhans cells (dendritic cells)
Antigen presenting cell
Normally found in epidermis, mucosa, lymph nodes, bone marrow
Langerhans cell histiocytosis mutation
BRAF mutations – 40-60% cases
Langerhans cell histiocytosis
Clinical features:
involvment patterns?
50% cases below what age?
Bone lesions patterns?
common bones?
symptoms?
Single organ involvement: bone or skin
Multi-organ involvement
50% cases <15 years
Bone lesions – solitary or multiple
Skull, ribs, vertebrae, mandible
Dull pain, tenderness
Langerhans cell histiocytosis
Mucosal involvement:
ulcerative or proliferative gingival mass
Langerhans cell histiocytosis
Radiographic features:
May resemble?
Sharply punched-out radiolucency (no corticated rim)
Occasionally ill-defined
May resemble aggressive periodontitis
Extensive alveolar involvement: teeth “floating in air”
Langerhans cell histiocytosis
Histopathologic features:
Diffuse infiltration of large, pale-staining mononuclear cells
Eosinophils
other mixed inflammatory cells
Immunohistochemistry (IHC): CD-1a, CD-207 (+)
Langerhans cell histiocytosis
Electron microscope:
Birbeck granules: rod-shaped cytoplasmic structures
dif dx
Langerhans cell histiocytosis
OKCs
ameloblastomas
Langerhans cell histiocytosis
Treatment:
Accessible bone lesions?
Chronic disseminated disease?
Accessible bone lesions often treated with curettage – prognosis is good
Chronic disseminated disease - management complex