Joints, Bones, And Soft Tissue Pathology Dr. Martin Flashcards
most common tumor is located where
and most common tumors
and survival
- long bones , (osteosarcoma is on knee and peaks at adolescents)
- osteosarcoma, chondrosarcoma, Ewing sarcoma
- 50% survival
Osteoid osteoma SX and due to what happening
- Worse at night pain , responding to NSAIDS And ASPIRIN
2. excess PGE2 made by osteoblasts
Osteoid Osteoma
- DX criteria
- histo
- TX
- Less then 2cm, if over 2cm = osteoblastoma
- Central nidus or translucent woven bone, surrounded by osteoblasts + thick rind or reactive cortical bone
- radiofrequency ablation
Osteoblastoma
- dx
- location
- sx
- tx
- B or M
- over 2cm , no bony reaction
- spine
- achy pain that does not respond to aspirin
- curetted or excised en bloc
- B
Osteosarcoma
1. what happens and sx
2. location
3 .AGE
- Malignant mesenchymal cells make bone matrix, (PAINFUL enlarging mass)
- knee 50%
- under 20yo, second peak in older males (PAGETS) from prior radiation = secondary osteosarcoma
Osteosarcoma
- DX
- histo
- X-ray, mixed lytic and blastic mass
+ CODMAN TRIANGLE (periosteum elevation after fractures = AGGRESSIVE TUMOR - bizarre giant tumor cells , lace like , can make cartilage = CHONDROBLASTIC OSTEOSARCOMA
Osteosarcoma
1. risk with what genes
- RB ; sporatic
- TP53 : Li-Fraumani syndrome (breast cancer)
- CDKN2A (INK4a gene) : encoding p14 and p16
Osteosarcoma looks like what
bulky gritty gray-white with hemorrhage and cystic degeneration (spreading to medullary cavity)
Osteosarcoma what do you assume about all pts with this
that they have occult metastases when dx and start radio/chemo, surgery
Osteosarcoma spreads to
lungs, bones, brains
most common benign bone tumor
osteochondroma
Osteochondroma
- happen how and age
- associated with
- and prevalence
- sporadic around 10-20yo early adulthood
- Multiple hereditary EXOSTOSIS : AD 5%-20% progress to chondrosarcoma
- men
Osteochondroma gene
EXT1 and EXT2 = heparin sulfate glycosaminoglycans
Osteochondroma
- how is it formed
- SX
- bony stalk capped by cartilage + medullary cavity continuous with bone
- pain if nerve impingement
Chondroma / Enchondroma
- location of both
- imaging looks like
- associated with what 2 syndromes
- tx
- Medullary cavity (Enchondroma), Surface of subperiosteal or juxtacortical (Chondroma)
- Circumscribed lucency with central irregular calcifications, sclerotic rim (nodules well circumscribed in hyaline cartilage)
- Ollier Syndrome + Maffucci Syndrome
Ollier Syndrome
many enchondromas , not hereditary
Maffucci syndrome
many enchondromas + hemangiomas
= increases risk of chondrosarcoma + brain glioma
= not hereditary
Enchondroma gene
IDH1 and IDH2
Chondrosarcoma
- location
- usually come from what
- SX
- pelvis, shoulders
- enchondromas, osteochondromas
- Painful Enlarging mass (usually to lungs)
Chondrosarcoma imaging
calcified matrix (looks like flocculent dansities)
Chondrosarcoma : 4 stages
- Conventional : glistening gray/white, translucent cartilage (cystic and ooze out)
- Dedifferentiated : low grade with a HIGH grade component (no cartilage)
- Clear Cell : large chondrocytes + osteoclast-type giant cells (malignant)
- Mesenchymal : well differentiated hyaline cartilage surrounded by sheets of small round cells
Chondrosarcoma TX
surgical excision = conventional, excision + chemo = mesenchymal and dedifferentiated (more aggressive)
Chondrosarcoma gene
EXT gene (multiple osteochondroma syndromes) IDH1 and IDH 2
Ewing Sarcoma Family Tumors (ESFT)
- prevalence
- looks like what
- gene
- whites
- small blue round tumor cells, primitive round cells
- PNET, EWSR1 gene Ch22, FLI1 gene CHR11
Ewing Sarcoma Family Tumors (ESFT) :
- SX
- imaging
- location
- PAINFUL, enlarging mass, tender, warm, swollen like an infection (fever, high WBCs, high sed rate)
- Periosteal reaction : onion skin on x-ray ( Moth eaten margins) + Sunbust / hair looking + Codman
- long bones FEMUR, pelvic
Ewing Sarcoma Family Tumors (ESFT)
- location in bone it starts
- histo
- TX and how to see if its working and prognosis
- medullary cavity, invading cortex, periosteum, soft tissue
- homer wright rosettes
- Chemo induced necrosis, more necrosis means its working more, higher prognosis
Giant Cell Tumor of bone : other name 1. looks like what other disease 2. B or M 3. cell type and what they have 4. location
OSTEOCLASTOMA
- looks like brown tumor of hyperparathyroidism
- 20yo-40yo, B, however usually locally destructive
- Osteoblasts with RANKL= osteoclasts
- Epiphyses (knee)
Giant Cell Tumor of bone :
- histology
- TX
- thin shell of reactive bone (red brown many large giant cells, cystic degeneration)
- Curetting and usually comes back + RANKL inhibitor = DENOSUMAB
Aneurysmal Bone Cyst (ABC) :
- B or M
- gene
- location
- age
- B , locally aggressive
- CRH17p13 = increase osteoblasts
- femur, tibia, vertebral body
- adolescence
Aneurysmal Bone Cyst (ABC) :
- SX
- imaging
- histo finding
- pain + swelling , limp, nerve compression
- LYTIC lesion, central lysis = EGGSHELL, trabeculae = SOAP,
BUBBLE - multinucleated OSTEOCLAST-GIANT CELLS lined by osteoblasts, BLUE BONE
Fibrous Cortical Defect :
- know as what
- who and age
- location
- DX criteria
- Metaphyseal Fibrous Defect (fibrous CT replace bone)
- over 2yo usually found in adolescents
- metaphysis around knee (usually bilateral)
- udner 0.5cm
Non-ossifying Fibroma
Fibrous Cortical Defect that has progressed and is 5cm-6cm
Fibrous Cortical Defect + Non-ossifying fibroma :
imaging
sharply demarcated radiolucent mass, rim of sclerosis
yellow/brown, fibroblasts in storiform pattern**
Fibrous Cortical Defect + Nonossifying Fibroma : SX and TX
- Asymptomatic
- spontaneously resolution over time
= Curettage needs bone grafting for proper healing
Fibrous Dysplasia :
what is it
B with fibrous tissue and bone growing and not maturing
Fibrous Dysplasia 4 patterns you see
- Monostatic ** : asymptmatic
- Polyostotic : many bones, crippling
- Craniofacial involvement
- shoulder and pelvic girdles (Crippling deformaties and fractures)
Fibrous Dysplasia can become what rarely
Malignant to sarcoma
Fibrous Dysplasia age and locations and SX and can grow more during what
- adolescence
- 1/3 craniofacial bones, 1/3 femur or tibia, 1/3 ribs
- pain , fractures, different length in limbs
- during pregnancy
Fibrous Dysplasia histology and gene
ground glass and GNAS1
Fibrous Dysplasia : McCune-Albright Disease
- what
- sx
- what is associated
- mutation
- unilateral bone lesions,
- Cafe a lait spots same side
- Precocious puberty in females
- GNAS mutation (early in embryo)
Fibrous Dysplasia : Mazabraud Syndrome
- what
- adult vs child
- polyostotic fibrous dysplasia
2. many skeletal deformaties (childhood), soft tissue myxomas (Adult)
Fibrous Dysplasia
- mutation happening after bone formation in embryo causes
- Histo
- monostotic
- Intramedullary lytic lesions , cause bowing and cortical thinning
+ hyaline nodules, disorganized growth plates , + curvilinear trabeculae with NO osteoblastic rimming
Fibrous Dysplasia Curvinilnear trabeculae looks like
chinese characters
most common metastatic cancers spreading to bone in ADULT and CHILDREN
- Prostate, breast, Kidney, Lung ** (PBL King)
2. Neuroblastoma, Wilms, Osteosarcoma, Ewing, Rhabdomyosarcoma
Metastatic Bone cancer is usually presented like what and locations
- multifocal except (thyroid and kidney)
2. Axial skeleton, hands and feet (lung, kidney, colon)
Metastiatic Bone Cancer LYTIC vs BLASTIC
- LYTIC : bone destroying = lung, kidney, GI, Melanoma
2. BLASTIC : bone forming = Prostatic adenocarcinoma
Hyaline Cartilage serves for what purpose
CT for elastic shock absorber, wear resistance
synovial fluid is composed of
hyaluronic acid rich plasma filtrate acting like a viscous lubricant + nutrition for hyaline cartilage
