Joints, Bones, And Soft Tissue Pathology Dr. Martin Flashcards
1
Q
most common tumor is located where
and most common tumors
and survival
A
- long bones , (osteosarcoma is on knee and peaks at adolescents)
- osteosarcoma, chondrosarcoma, Ewing sarcoma
- 50% survival
2
Q
Osteoid osteoma SX and due to what happening
A
- Worse at night pain , responding to NSAIDS And ASPIRIN
2. excess PGE2 made by osteoblasts
3
Q
Osteoid Osteoma
- DX criteria
- histo
- TX
A
- Less then 2cm, if over 2cm = osteoblastoma
- Central nidus or translucent woven bone, surrounded by osteoblasts + thick rind or reactive cortical bone
- radiofrequency ablation
4
Q
Osteoblastoma
- dx
- location
- sx
- tx
- B or M
A
- over 2cm , no bony reaction
- spine
- achy pain that does not respond to aspirin
- curetted or excised en bloc
- B
5
Q
Osteosarcoma
1. what happens and sx
2. location
3 .AGE
A
- Malignant mesenchymal cells make bone matrix, (PAINFUL enlarging mass)
- knee 50%
- under 20yo, second peak in older males (PAGETS) from prior radiation = secondary osteosarcoma
6
Q
Osteosarcoma
- DX
- histo
A
- 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
7
Q
Osteosarcoma
1. risk with what genes
A
- RB ; sporatic
- TP53 : Li-Fraumani syndrome (breast cancer)
- CDKN2A (INK4a gene) : encoding p14 and p16
8
Q
Osteosarcoma looks like what
A
bulky gritty gray-white with hemorrhage and cystic degeneration (spreading to medullary cavity)
9
Q
Osteosarcoma what do you assume about all pts with this
A
that they have occult metastases when dx and start radio/chemo, surgery
10
Q
Osteosarcoma spreads to
A
lungs, bones, brains
11
Q
most common benign bone tumor
A
osteochondroma
12
Q
Osteochondroma
- happen how and age
- associated with
- and prevalence
A
- sporadic around 10-20yo early adulthood
- Multiple hereditary EXOSTOSIS : AD 5%-20% progress to chondrosarcoma
- men
13
Q
Osteochondroma gene
A
EXT1 and EXT2 = heparin sulfate glycosaminoglycans
14
Q
Osteochondroma
- how is it formed
- SX
A
- bony stalk capped by cartilage + medullary cavity continuous with bone
- pain if nerve impingement
15
Q
Chondroma / Enchondroma
- location of both
- imaging looks like
- associated with what 2 syndromes
- tx
A
- 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
16
Q
Ollier Syndrome
A
many enchondromas , not hereditary
17
Q
Maffucci syndrome
A
many enchondromas + hemangiomas
= increases risk of chondrosarcoma + brain glioma
= not hereditary
18
Q
Enchondroma gene
A
IDH1 and IDH2
19
Q
Chondrosarcoma
- location
- usually come from what
- SX
A
- pelvis, shoulders
- enchondromas, osteochondromas
- Painful Enlarging mass (usually to lungs)
20
Q
Chondrosarcoma imaging
A
calcified matrix (looks like flocculent dansities)
21
Q
Chondrosarcoma : 4 stages
A
- 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
22
Q
Chondrosarcoma TX
A
surgical excision = conventional, excision + chemo = mesenchymal and dedifferentiated (more aggressive)
23
Q
Chondrosarcoma gene
A
EXT gene (multiple osteochondroma syndromes) IDH1 and IDH 2
24
Q
Ewing Sarcoma Family Tumors (ESFT)
- prevalence
- looks like what
- gene
A
- whites
- small blue round tumor cells, primitive round cells
- PNET, EWSR1 gene Ch22, FLI1 gene CHR11
25
Ewing Sarcoma Family Tumors (ESFT) :
1. SX
2. imaging
3. location
1. PAINFUL, enlarging mass, tender, warm, swollen like an infection (fever, high WBCs, high sed rate)
2. Periosteal reaction : onion skin on x-ray ( Moth eaten margins) + Sunbust / hair looking + Codman
3. long bones FEMUR, pelvic
26
Ewing Sarcoma Family Tumors (ESFT)
1. location in bone it starts
2. histo
3. TX and how to see if its working and prognosis
1. medullary cavity, invading cortex, periosteum, soft tissue
2. homer wright rosettes
3. Chemo induced necrosis, more necrosis means its working more, higher prognosis
27
```
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
1. looks like brown tumor of hyperparathyroidism
2. 20yo-40yo, B, however usually locally destructive
3. Osteoblasts with RANKL= osteoclasts
4. Epiphyses (knee)
28
Giant Cell Tumor of bone :
1. histology
2. TX
1. thin shell of reactive bone (red brown many large giant cells, cystic degeneration)
2. Curetting and usually comes back + RANKL inhibitor = DENOSUMAB
29
Aneurysmal Bone Cyst (ABC) :
1. B or M
2. gene
3. location
4. age
1. B , locally aggressive
2. CRH17p13 = increase osteoblasts
3. femur, tibia, vertebral body
4. adolescence
30
Aneurysmal Bone Cyst (ABC) :
1. SX
2. imaging
3. histo finding
1. pain + swelling , limp, nerve compression
2. LYTIC lesion, central lysis = EGGSHELL, trabeculae = SOAP,
BUBBLE
3. multinucleated OSTEOCLAST-GIANT CELLS lined by osteoblasts, BLUE BONE
31
Fibrous Cortical Defect :
1. know as what
2. who and age
3. location
4. DX criteria
1. Metaphyseal Fibrous Defect (fibrous CT replace bone)
2. over 2yo usually found in adolescents
3. metaphysis around knee (usually bilateral)
4. udner 0.5cm
32
Non-ossifying Fibroma
Fibrous Cortical Defect that has progressed and is 5cm-6cm
33
Fibrous Cortical Defect + Non-ossifying fibroma :
| imaging
sharply demarcated radiolucent mass, rim of sclerosis
| yellow/brown, fibroblasts in storiform pattern****
34
Fibrous Cortical Defect + Nonossifying Fibroma : SX and TX
1. Asymptomatic
2. spontaneously resolution over time
= Curettage needs bone grafting for proper healing
35
Fibrous Dysplasia :
| what is it
B with fibrous tissue and bone growing and not maturing
36
Fibrous Dysplasia 4 patterns you see
1. Monostatic ** : asymptmatic
2. Polyostotic : many bones, crippling
3. Craniofacial involvement
4. shoulder and pelvic girdles (Crippling deformaties and fractures)
37
Fibrous Dysplasia can become what rarely
Malignant to sarcoma
38
Fibrous Dysplasia age and locations and SX and can grow more during what
1. adolescence
2. 1/3 craniofacial bones, 1/3 femur or tibia, 1/3 ribs
3. pain , fractures, different length in limbs
4. during pregnancy
39
Fibrous Dysplasia histology and gene
ground glass and GNAS1
40
Fibrous Dysplasia : McCune-Albright Disease
1. what
2. sx
3. what is associated
4. mutation
1. unilateral bone lesions,
2. Cafe a lait spots same side
3. Precocious puberty in females
4. GNAS mutation (early in embryo)
41
Fibrous Dysplasia : Mazabraud Syndrome
1. what
2. adult vs child
1. polyostotic fibrous dysplasia
| 2. many skeletal deformaties (childhood), soft tissue myxomas (Adult)
42
Fibrous Dysplasia
1. mutation happening after bone formation in embryo causes
2. Histo
1. monostotic
2. Intramedullary lytic lesions , cause bowing and cortical thinning
+ hyaline nodules, disorganized growth plates , + curvilinear trabeculae with NO osteoblastic rimming
43
Fibrous Dysplasia Curvinilnear trabeculae looks like
chinese characters
44
most common metastatic cancers spreading to bone in ADULT and CHILDREN
1. Prostate, breast, Kidney, Lung **** (PBL King)
| 2. Neuroblastoma, Wilms, Osteosarcoma, Ewing, Rhabdomyosarcoma
45
Metastatic Bone cancer is usually presented like what and locations
1. multifocal except (thyroid and kidney)
| 2. Axial skeleton, hands and feet (lung, kidney, colon)
46
Metastiatic Bone Cancer LYTIC vs BLASTIC
1. LYTIC : bone destroying = lung, kidney, GI, Melanoma
| 2. BLASTIC : bone forming = Prostatic adenocarcinoma
47
Hyaline Cartilage serves for what purpose
CT for elastic shock absorber, wear resistance
48
synovial fluid is composed of
hyaluronic acid rich plasma filtrate acting like a viscous lubricant + nutrition for hyaline cartilage
