Bone and Soft TIssue Flashcards

(69 cards)

1
Q

Common pseudolipoblasts

A
fat atrophy
fat necrosis
hibernoma cells
silicone reaction
signet ring cells
fixation artifact
neoplastic fat infiltration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Pleomorphic lipoma/spindle cell lipoma histo features

A

well circ, superficial
floret cells, ropey collagen, may have little or no fat!
clin: older men, upper shoulder, H/D
IHC: CD34+ many cells (ALT will have rare cells)
MDM2 - never have amplification

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

CD34+

A

DFSP, peripheral nerve sheath tumours, pleomorphic lipoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

most common type of ALT

A

sclerosing variant - dense fibrous bands

low power enlarged hyperchromatic nuclei in fibrous septae

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Variants of ALT

A

lipoma-like
sclerosing (1st most common)
myxoid/round cell (2nd most common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Cytogenetics of ALT

A

12q amplification, many genes including MDM2 amplification

ring and marker chromosomes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

myxoma histo

A

can be cellular
few blood vessels
little atypia on low power

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

myxofibrosarcoma histo

A

nuclear atypia

vascular pattern - CD34 around vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

extraskeletal myxoid chondrosarcoma histo and ddx

A

cords of cells touching each other
cytoplasmic eosinophila
bland myxoid background between cords
ddx: myoepithelioma (CK+)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

myxoid liposarcoma histo

A

characteristic chicken-wire plexiform vascular pattern
lack of cytological atypia
even distribution of spindle cells
uniform myxoid stroma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

myxoid/round cell LS demographics and gross

A

peak age 5th decade
extremities, (rarely retroperitoneum)
never in children
hist: blue myxoid material in cystic like spaces
lymphangioma-like, cyst-like spaces but look for solid areas
be careful around periphery of nodules - can be very round cell and hypercellular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

how to tell round cell from myxoid

A

round cells predominate - whole 4X field

blood vessels inconspicuous and lost

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

myxoid/round cell LS cytogenetics

A
mostly t(12;16) DDIT3-FUS
some t(12;22) DDIT3-EWS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Evan’s tumour, low-grade fibromyxoid sarcoma histo (aka hyalinising spindle cell tumour with collagen/giant rosettes)

A

bland spindle cell tumour with diffuse distribution
alternating fibrous and myxoid zones
vascular plexiform areas
collagen rosettes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Evan’s tumour cytogenetics

A

t(7;16) - CREB3L2-FUS

t(11;16) - CREB3L1-FUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Extraskeletal myxoid chondrosarcoma cytogenetics

A

t(9;22) - NR4A3-EWS

t(9;17) - NR4A3/TAF2N

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Ollier disease

A

Multiple enchondroma - entire skel, half the body or one limb
non hereditary
increased cellularity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Maffucci’s syndrome

A

Multiple enchondromas
Soft tissue hemangiomas
increased risk of CS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

enchondroma histo features

A

abuttment of cortical bone with no evidence of invasion
long bones: hypocellular, bland cytology, degenerative changes
small bones: moderately cellular, increased nuclear size; no invasion of medullary or cortical bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

ways to differentiate cellular schwannoma and MPNST

A

schwannoma: encapsulated, rare necrosis, no divergent differentiation, S100 diffuse +
MPNST: not encapsulated, commonly necrotic
divergent differentiation seen in 10%, S100 focal or patchy or negative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

chondroblastoma demographics and gross

A

sclerotic rim
metaphyseal or epiphysis
DI - very benign looking

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

enchondroma vs osteochondroma

A

enchondroma - intramedullary

osteochondroma - cortex and medullary continuty at sites of tendon insertion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

chondroblastoma histo

A

oval nuclei, eosinophilic cytoplasm with well-defined cytoplasmic boundaries; chicken wire calcification (between cells)
small round cells with atypia minimal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

chondromyxoid fibroma

A

heterogeneous; benign DI appearance; metaphyseal in large bones but occ in hands and feet
macrolobular or microlobular growth patterns with stellate cytoplasmic extentions that are pale pink

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Chondrosarcoma demographics and gross
more central joints DI ugly cortical abN - scalloping, cortical destuction, expansion of bone and many with soft tissue mass
26
Grading systems vary from site to site
One to three, three high-grade while 2 is intermediate and is treated as high grade
27
Chondroblastic OS vs CS
OS will have higher grade nuclei and cartilage formation; metaphyseal lesion in young child with DI aggressive look --> will be OS CS - later in life with pelvic, shoulder girdle and prox humerus/femur
28
OS histology?
lace like osteoid high grade nuclei many variants
29
telangiectatic OS
sometimes very little osteoid but very ugly cells with large amounts of blood
30
Treatment effect grading
Huvos's grading | 1 90% good prognosis
31
Parosteal OS
low-grade but may look like oestochondroma with cartilage cap but beware; minimal atypia in spindled cells trabeculae of bone may contain high grade sarcoma of dediff
32
fibrous dysplasia
DI ground glass mineralisation with slcerotic rim | irregular spicules of woven bone with bland spindle cells wit h occ foam cells or myxoid change and fibrosis
33
Fibrous dysplasia molecular pathogenesiss and assoc syndrome?
GNAS1 gene activation mutation; McCune Albright syndrome - polysostoic fiborus dysplasi, endocrine abN, cutaenous pigmentation Mazabraud's syndrome - fibrous dysplasia and soft tissue myxoma
34
Non-ossifying fibroma (metaphyseal fibrous defect) site and characteristic DI and histology?
cortex or corticomedullary cavity with sclerotic margins distal femur, distal tibia and proximal tibia peds plump spindle cells in loose steriform pattern with multinuceated giant cells, foam cells, chronic inflammation
35
Ewing sarcoma site and characteristic DI and histology?
many in pelvic girdle and lower extremities;in bones femur > ilium > fibula metadiaphyseal lesion DI 'onion skinning" and moth-eaten places will move into marrow spaces small round blue cells with sheet-like growth and lobular or filagree in soft tissue
36
ES histological types
classical - small round blue cells with scant cytoplasm PNET - rosettes and neural atypical - cytological atypia
37
ES IHC?
CD99 - not useful alone, membranous FLI1+ focal + keratin molecular more helpful
38
What factors imply poor prognosis in ES?
older age >17 yo pelvic tumour large tumour >8 cm metastatic disease
39
What is giant cell tumour?
``` one of few tumours F>M epiphysis most solitatre but may be multiple 3-5th decades distal femur>proximal tibia>distal radius > sacrum DI look can ugly gross: red mahogany ```
40
What are the ddx of epiphyseal tumours
clear cell CS, chondroblastoma, giant cell tumour
41
What are the characteristic giant cell histological features?
multinucelated giant cells evenly distributed; monouclear cells with round to oval nuclei and eosinphilic cytoplasm mitotic activity foam cells, reactive new bone ddx: OS, ABC
42
ABC demographics and gross
may be 1' or 2' to other tumours (chondroblastoma) mkost common in >20 yo distal femur or proximal tibia metaphysis in spine, posterior elements DI fluid levels, cortical destruction, lucency in metaphysis
43
ABC histology?
multiple cyts fibrous septa with spindle cells without atypia, multnculeated giant cells, not enothelial lining osteoid and bony matirx with woven bone calcification seen
44
Unicameral bone cyst demographics and gross
``` similar to ABC usually first two decades of life site: prox humerus and prox femur present with path # DI no wider than epiphyseal plate, not expansile ```
45
ABC vs UBC
ABC higher recurrence, tx surgical | UBC may not be surgical
46
UBC histo
cyst wall fragments with bland spindlecells wit hfragments of bone irregular masses of degernating fibrin occ calcified
47
chordoma demographics and gross
M>F; 5th-7th decades pelvic or skull (skull ones a decade younger); some in spine DI - can be ugly lytic destructive and requent soft tissue mass gray-tan, red brown lobulated growth pattern
48
chordoma histo
``` lobules wit hfibrous sept cords and nests of tumours in pale blue myxoid background round nuclei wht central nucleoli physaliphorous cell chondroid chordoma --> skull ones ```
49
chordoma IHC
+ panCK + EMA + brachyury
50
osteochondroma demographics and gross
mostly prox humerus>prox tibia DI: cortex and medullary cavity continuous with underlying bone at site of tendon insertions gross: pedunculated with grey cap
51
osteochondroma histo
cap: chondrocytes in lacunae in clusters pheripherally and in coluns at base of cap - look like epitphyseal plate stalk - cancellous bone with fat and BM
52
osteochondroma ddx
``` parosteal osteosarcoma chondrosarcoma arising in ostochondroma bizarre parosteal osteochondromatous proliferatoin heterotopic ossification subungal exostosis ```
53
Subcutaneous granulomatous inflammatory reaction ddx?
Granuloma annulare Rheumatoid nodule Infection Necrobiosis lipoidica
54
What is granuloma annulare?
Benign self-limited dermatosis characterized by raised annular lesions. It is an otherwise asymptomatic disorder of dorsum of hands and legs, occasionally generalized
55
With what is granuloma annulare usually associated?
morphea, chronic hepatitis C infection, autoimmune thyroiditis, secondary hyperparathyroidism, Plummer’s disease, myelodysplastic syndrome, metastatic carcinoma and rarely associated with diabetes
56
What are the characteristic staining pattern of spindle cell lipoma?
+CD34, androgen receptors in spindle cells (in men and women)
57
What are the typical features of spindle cell lipoma?
``` Fat cells Spindle cells with short stubby proviles Ropey collagen Myxoid stroma occasionally Curvilinear or plexiform vasculature May have one or two mitoses but if atypical, consider other entities ```
58
What are some CD34+ tumours?
Vascular tumours Spindle cell lipoma DFSP (vs DF-) GIST solitary fibrous tumour (hemangiopericytoma) epithelioid sarcoma (vs. epithelioid mesotheliojma -) some neural tumours lymphocyte-rich thymoma (vs T cell lymphoma -)
59
What are the typical features of fibrous hamartoma of infancy?
3 distinct components in varying amounts 1) Intersecting bands and trabeculae of mature fibrous tissue, comprising spindle myofibroblasts and fibroblasts 2) Nests and whorls of immature round, ovoid, or spindle cells in loose myxoid stroma and often arranged around small veins 3) Interspersed mature fat
60
What is the French system grading of sarcomas?
Grade 1: total score of 2-3 points Grade 2: total score of 4-5 points Grade 3: total score of 6-8 points Tumor differentiation: 1 point: resembles normal adult mesenchymal tissue, may be confused with a benign lesion, such as well differentiated liposarcoma 2 points: histologic typing is certain, such as myxoid liposarcoma 3 points: synovial sarcoma, osteosarcoma, Ewing’s sarcoma/PNET, sarcomas of doubtful tumor type, embryonal and undifferentiated sarcomas ``` Mitotic count (count 10 successive high power fields [area of 0.17 mm squared] in most mitotically active areas): 1 point: 0-9 mitoses 2 points: 10-19 mitoses 3 points: 20 or more mitoses ``` Tumor necrosis: 0 points: no necrosis on any slides 1 point: less than 50% necrosis for all examined tumor surface 2 points: tumor necrosis of 50% or more of examined tumor surface
61
What is fibrous hamartoma of infancy?
Benign fibrous lesion of dermis or sc arising in first 2 y of life; trunk or other sites; recurrence if incomplete excision. Presents as painless enlarging mass.
62
Nodular fasciitis vs scar
Look at infiltration into muscle at edges - negative if scar, will dissect in fasciitis
63
Nodular fasciitis ddx
neural tumour cellular scar keloid fibromatosis (beta catenin)
64
RF for MPNST
NF1 | radiation
65
What proportion of MPNST arise from neurofibromas?
about 50%
66
In a NF patient what is the approximate risk of a MPNST?
5-15%
67
What is the mechanism of N! nerofibroma transformation?
INK4A and p53 downstream mutation
68
Features of calcifying aponeurotic fibroma?
Fibrous growth with multiple extensions into surrounding tissue Centrally located zones of cartilage formation and stippled calcification Associated osteoclast-like giant cells may be present Calcifications range from fine granules to large masses Chondrocyte-like cells can be arranged in linear columns radiating from pockets of calcification True ossification uncommon Mitoses scarce Calcification increases with age Lesions from infants and small children may lack calcification
69
Where and who gets calcifying aponerutoic fibroma?
Young children usually first two years of life but can be as old as 12 y. Hands and feet as painless growing mass. Prone to local recurrence (40%) as the tumour is highly infiltrative and difficult to excise fully (also cosmetic or functional effect)