Onc Flashcards
1
Q
osteoid osteoma
age? what do nsaids inhibit?
what will you see in the spine? Metaphysis?
histology. whats the best imaging? treatment
A
2
Q
Biopsy Principles
What type of incision?
Proximal Humerus biopsy, what compartment?
In general go for ____, and avoid ___
core needle biopsy higher risk of ? than incisional biopsy
A
3
Q
radiation
Pre-op xrt ?
post op xrt ?
convential radition is?
What’s proton beam therapy?
risk of cryotherapy?
A
4
Q
genetics
MDM2 amplification
t(12;16)
t(X;18), SYT-SSX
t(11;22), EWS-FLI
t(2;13), PAX-FKHR
t(12;22), EWS-ATF
t(9;22), EWS-CHN
Infantile fibrosarcoma?
How to determine translocation?
A
5
Q
osteoblastoma
age?
how is it different from OO?
size of lesion?
histology?
tx?
A
6
Q
Osteiod Osteoma vs Osteoblastoma
histology?
treatment?
size?
A
7
Q
osteosarcoma
age?
location?
xray and histology?
testable items?
A
Treatment: chemo + surgery
no genetic translocation but associated with alterations in Rb and p53 genes
IHC: SATB2 new differentiator
8
Q
osteosarcoma surface lesions
Parosteal “PAR” =
Periosteal
A
ParostealPAR= stuck on bone
low grade, wide excision, good prognosis.
PERIosteal = high grade, sunburst surface lesion, eccentric
9
Q
osteosarcomas
Conventional vs Par vs Peri on imaging and histology
A
10
Q
parosteal vs HO vs MHE?
How to spot the difference on CT?
A
11
Q
telengiectatic OSA
what will you see on histology? How to differentiate with ABC?
A
12
Q
Paget’s disease
what cancer are they at risk for?
Prognosis?
Age?
A
13
Q
treatments for
Parosteal osteosarcoma vs Periosteal osteosarcoma vs all other osteosarcomas?
key test item
A
Parosteal = wide resection
PERI and All other Osteosarcoma get chemo and surgery
14
Q
chrondogenic lesions
name the 5?
A
15
Q
Soft tissue lesions
what size cm is concerning? (size in hands ant feet)
Don’t be fooled by Soft Tissue Sarcomas because they present as?
A
* ALL Require appropriate evaluation before
resection
* * > 5 cm (1.5 cm hands/ feet) needs 3-D imaging (CT/MRI)
* FNA/Biopsy
- Soft Tissue Sarcomas – DONT BE FOOLED!
(STEM)
– Painless
– Insidious onset
16
Q
whoops surgery: unplanned excision
What do you do if you excise a mass and it comes back as a sarcoma?
A
17
Q
unplanned excision
why should you be aware of “atraumatic hematoma”!!, what should you do before wash out?
A
- Imaging of sarcoma with hemorrhage and
hematoma appear the same - BIOPSY before washout
18
Q
Soft tissue sarcomas
size to be worried about?
how to stage?
better/worse prognosis indicators?
synovial sarcoma: genetics?
A
19
Q
STS
growth characteristics?
histology?
A
20
Q
STS
Most common STS?
A
21
Q
STS
Predisposing factors?
A
22
Q
STS imaging
MRI will show ?
What’s the exception
A
23
Q
Most Common STS Pathology?
A
24
Q
Synovial sarcoma
tx?
presentation?
genetic factos to know?
how does it look on histology?
A
tx: radiation therapy and surgery, some benefit to chemo (exception to the rule for STS)
Grow rapidly
t(x:18)
SYT/ssx1 & SYT/SSX2 fusion transcripts
25
# Fatty tumors
history?
MRI shows?
Pathology?
Treatment?
26
# liposarcoma
what are the subtypes to know?
27
# Fatty tumors
28
# Fatty tumor treatments
29
# Lipoma on MRI
30
# soft tissue sarcoma vs lipoma MRI T1 and T2
31
# Fibrosarcoma
imaging will look the same as other STS,
pathology has a characteristic pattern?
32
# Malignant peripheral nerve sheath tumor
what's the association?
how does it look on path?
Treatment?
33
# angiosarcoma
how does it spread?
what do you need to biopsy?
path?
Treatment?
34
# rhabdomyosarcoma
common in what population?
pathology to know? (stains, genes)
how is this treatment different?
## Footnote
likely will show up as a distractor
35
# epitheliod
most common in what location?
pathology?
Histology?
36
STS with lyphmode metases
SCARE:
Synovial Sarcoma
Clear cell sarcoma
Angiosarcoma
Rhabdomyosarcoma
Epithelioid sarcoma
37
how to treat soft tisse sarcoma?
how much radiation to give? pre vs post rads complications
## Footnote
high yield
38
# Chondroma
enchodroma vs enchondroma?
how does it look on xray?
histology? How does histology in hands and feet differ?
How to differentiate between low grade chrondrosarcoma and endchondroma?
## Footnote
Benign cartilage tumors on the surface of bone are called periosteal chondromas. When they are in the medullary cavity, they are called enchondromas. Most endochondromas necessitate no treatment
* Enchondromas appear radiographically as areas of stippled calcifications. The radiographic distinction between low-grade chondrosarcoma and enchondroma can be difficult; serial plain radiographs show cortical bone changes or lysis of the previously mineralized cartilage in chondrosarcoma. Patients with chondrosarcoma have pain
39
# olliers disease
multiple enchondromas
dysplastic bones (escpecially ?)
risk of transformation?
## Footnote
Ollier disease:
* Multiple enchondromas
* Dysplastic bones (particularly a shortened ulna)
* A 30% risk of transformation to chondrosarcoma
40
# multiple enchondromas
what are the 2 syndromes? how does the risk of malignancy differ?
## Footnote
* Ollier disease:
* Multiple enchondromas
* Dysplastic bones (particularly a shortened ulna)
* A 30% risk of transformation to chondrosarcoma
* Random spontaneous mutation
* Maffucci syndrome:
* Multiple enchondromas and soft tissue hemangiomas (extremity and visceral)
* A 100% risk of malignancy, chondrosarcoma, angiosarcoma, astrocytoma and GI malignancy
41
# osteochondroma
characteristic appearance?
cartilaginous cap?
Tx?
risk of malignant transformation?
## Footnote
* Characteristic appearance is a surface lesion in which the cortex of the lesion and the underlying cortex are confluent and the medullary cavity of the host bone also flows into (is continuous with) the osteochondroma.
* thin cartilaginous cap, usually 2-3 mm, in a growing child may exceed 1-2cm
* When asymptomatic, these lesions are treated with observation only
* Malignant transformation into a secondary chondrosarcoma is rare, occurring in far fewer than 1% of cases.
* Thickness of the cartilage cap (>2 cm) may increase the risk of malignancy.
42
# MHE
Genetics
Inheritance pattern?
Which mutation is worst?
Risk of transformation?
## Footnote
Multiple hereditary exostoses
## Footnote
* autosomal disorder manifesting in childhood with multiple osteochondromas.
* Mutations are found in the EXT1, EXT2, and EXT3 gene loci; the EXT1 mutation is associated with a greater
* burden of disease and higher risk of malignancy. Approximately 5%–10% of affected patients a secondary chondrosarcoma develops, which is low grade.
"EXT 1 = #1 burden, #1 risk of transformation"
43
# chondromyxoid fibroma
where do you commonly see it? age population?
aggressive?
treatment?
44
# Chondroblastoma
age and location?
Xrays show? MRI shows? classic histology pattern?
treatment
## Footnote
* Centered in the epiphysis in young patients, usually with open physes
* Radiographs show a central region of bone destruction that is usually sharply demarcated from the normal medullary cavity by a thin rim of sclerotic bone. MRI shows edema far out of proportion to the lesion.
* Treatment is with intralesional resection and curettage.
45
# epiphyseal lesions
what are they?
which ones are seen in the immature vs mature?
must know for the test!
46
# chrondogenic lesions treatment
osteochondroma
enchondroma
chondroblastoma
CMF
47
# chondrosarcoma
Age?
Most common location?
treatment?
chemotherapy use?
## Footnote
* Occurs in patients over 50 years old, and the pelvis is the most common location
* May be primary or may arise secondarily in a previous lesion (enchondroma, osteochondroma)
* It may be extremely difficult to differentiate malignant cartilage on the basis of histologic features alone. The clinical, radiographic, and histologic features of a particular lesion must be considered in combination to avoid incorrect diagnosis.
* Treatment consists of wide-margin surgical resection.
* Chemotherapy is used only as an adjunct for dedifferentiated and mesenchymal chondrosarcomas.
* Dedifferentiated chondrosarcoma, which is the most malignant cartilage tumor, has a biomorphic histologic and radiographic appearance. In typical cases, a high-grade spindle cell sarcoma is intimately associated with the low-grade cartilage component. Treatment is with wide-margin surgical resection and chemotherapy.
48
# chondrosarcoma
Age?
Most common location?
MRI findings?
treatment?
chemotherapy use?
## Footnote
* Occurs in patients over 50 years old, and the pelvis is the most common location
* May be primary or may arise secondarily in a previous lesion (enchondroma, osteochondroma)
* It may be extremely difficult to differentiate malignant cartilage on the basis of histologic features alone. The clinical, radiographic, and histologic features of a particular lesion must be considered in combination to avoid incorrect diagnosis.
* Treatment consists of wide-margin surgical resection.
* Chemotherapy is used only as an adjunct for dedifferentiated and mesenchymal chondrosarcomas.
* Dedifferentiated chondrosarcoma, which is the most malignant cartilage tumor, has a biomorphic histologic and radiographic appearance. In typical cases, a high-grade spindle cell sarcoma is intimately associated with the low-grade cartilage component. Treatment is with wide-margin surgical resection and chemotherapy.
49
# chondrosarcoma
Age?
Most common location?
histology
treatment?
chemotherapy use?
## Footnote
* Occurs in patients over 50 years old, and the pelvis is the most common location
* May be primary or may arise secondarily in a previous lesion (enchondroma, osteochondroma)
* It may be extremely difficult to differentiate malignant cartilage on the basis of histologic features alone. The clinical, radiographic, and histologic features of a particular lesion must be considered in combination to avoid incorrect diagnosis.
* Treatment consists of wide-margin surgical resection.
* Chemotherapy is used only as an adjunct for dedifferentiated and mesenchymal chondrosarcomas.
* Dedifferentiated chondrosarcoma, which is the most malignant cartilage tumor, has a biomorphic histologic and radiographic appearance. In typical cases, a high-grade spindle cell sarcoma is intimately associated with the low-grade cartilage component. Treatment is with wide-margin surgical resection and chemotherapy.
50
# chondrosarcoma
Age?
Most common location?
staging?
treatment?
chemotherapy use?
## Footnote
* Occurs in patients over 50 years old, and the pelvis is the most common location
* May be primary or may arise secondarily in a previous lesion (enchondroma, osteochondroma)
* It may be extremely difficult to differentiate malignant cartilage on the basis of histologic features alone. The clinical, radiographic, and histologic features of a particular lesion must be considered in combination to avoid incorrect diagnosis.
* Treatment consists of wide-margin surgical resection.
* Chemotherapy is used only as an adjunct for dedifferentiated and mesenchymal chondrosarcomas.
* Dedifferentiated chondrosarcoma, which is the most malignant cartilage tumor, has a biomorphic histologic and radiographic appearance. In typical cases, a high-grade spindle cell sarcoma is intimately associated with the low-grade cartilage component. Treatment is with wide-margin surgical resection and chemotherapy.
51
# fibrous lesions of bone
name the 4
52
# Non-ossifying fibroma
where does it occur? how does it look on xray? treatment?
## Footnote
* Metaphyseal fibrous defect (nonossifying fibroma) is an extraordinarily common lesion, occurring in approximately 30%–40% of children. The characteristic radiographic appearance is that of a lucent lesion that is metaphyseal, eccentric, and surrounded by a sclerotic rim. The overlying cortex may be slightly expanded and thinned.
* Treatment is with observation. Curettage and bone grafting are indicated in symptomatic lesions (painful or fractured) with more than 50% of cortical involvement.
53
# osteofibrous dysplasia
where does it occur?
how does pathology look?
## Footnote
* This condition manifests in a manner similar to that of fibrous dysplasia but in children younger than 10 years.
* The tibia is its characteristic location.
54
# fibrous dysplasia
how does it present? how does it look on xray? what does pathology look like?
## Footnote
* This condition is caused by a genetic activating mutation of the GSα surface protein, which results in increased production of cAMP.
* Its radiographic appearance is variable but classically referred to as “ground glass.”
* Its histologic appearance has been likened to “alphabet soup” and “Chinese letters.”
* Autogenous cancellous bone grafting is never used for this disorder because the transplanted bone is quickly transformed into the woven bone of fibrous dysplasia.
* Polyostotic fibrous dysplasia is less common but more symptomatic and is diagnosed earlier (before the age 10 years) than monostotic fibrous dysplasia.
* Polyostotic fibrous dysplasia with endocrinopathy is termed McCune-Albright syndrome (café au lait spots, precocious puberty, and polyostotic fibrous dysplasia).
55
# fibrous dysplasia
how does it present? how does it look on xray? fracture risk?
## Footnote
* This condition is caused by a genetic activating mutation of the GSα surface protein, which results in increased production of cAMP.
* Its radiographic appearance is variable but classically referred to as “ground glass.”
* Its histologic appearance has been likened to “alphabet soup” and “Chinese letters.”
* Autogenous cancellous bone grafting is never used for this disorder because the transplanted bone is quickly transformed into the woven bone of fibrous dysplasia.
* Polyostotic fibrous dysplasia is less common but more symptomatic and is diagnosed earlier (before the age 10 years) than monostotic fibrous dysplasia.
* Polyostotic fibrous dysplasia with endocrinopathy is termed McCune-Albright syndrome (café au lait spots, precocious puberty, and polyostotic fibrous dysplasia).
56
# fibrous dysplasia
how does it present? how does it look on xray? pathology? associated syndrome?
## Footnote
* This condition is caused by a genetic activating mutation of the GSα surface protein, which results in increased production of cAMP.
* Its radiographic appearance is variable but classically referred to as “ground glass.”
* Its histologic appearance has been likened to “alphabet soup” and “Chinese letters.”
* Autogenous cancellous bone grafting is never used for this disorder because the transplanted bone is quickly transformed into the woven bone of fibrous dysplasia.
* Polyostotic fibrous dysplasia is less common but more symptomatic and is diagnosed earlier (before the age 10 years) than monostotic fibrous dysplasia.
* Polyostotic fibrous dysplasia with endocrinopathy is termed McCune-Albright syndrome (café au lait spots, precocious puberty, and polyostotic fibrous dysplasia).
57
# Osteofibrous Dysplasia vs Fibrous Dysplasia
## Footnote
OFD: * This condition manifests in a manner similar to that of fibrous dysplasia but in children younger than 10 years. * The tibia is its characteristic location.
58
hemangioma in spine?
59
Multiple myeloma:
what to look for urine?
xrays will show?
classic histology findings of?
**treatment?**
* Multiple myeloma is the most common primary tumor of bone.
* Light-chain subunits of immunoglobulins G and A are found in the urine.
* Radiographic appearance of multiple myeloma is punched-out lytic lesions.
* The classical histologic appearance is sheets of plasma cells that appear monoclonal with immunostaining. Well-differentiated plasma cells have eccentric nuclei that have peripherally clumped, chromatic “clock faces.”
* Treatment is multimodal and includes **chemotherapy, radiation therapy, and surgery**
60
# lymphoma of bone
Age >
xrays show?
histology shows? How to differentiate from other blue cell tumors?
IHC?
## Footnote
NHL: nonhodgkin lymphoma
large soft tissue mass out of proportion to the amount of bone destruction is characteristic of lymphoma of the bone.
IHC CD45 and leukocyte antigen positive (LCA+)
Multiagen chemo (CHOP)
surgery to stabilize fx
61
# myeloma
what kind of malignancy?
age?
lesions on bone scan show up as?
Histology?
IHC stains?
how to tell it from lymphoma?
62
# chordoma
arise from what origin?
most common location?
MRI/CT scan will show?
Histology?
Tx?
## Footnote
* Chordoma is a malignant neoplasm that arises from primitive notochordal tissue.
* The most common location is the sacrococcygeal region, and the second most common is the sphenooccipital region.
* CT scans show a midline bone mass.
63
# Bone tumors of unknown origin
64
Adamantinoma: what is it and where does it arise? DDX and how to confirm dx? Treatment?
## Footnote
Adamantinoma is a rare tumor that classically manifests in the anterior cortex of the tibial diaphysis. Treatment is with wide-margin surgical resection.
65
# Langerhans Cell Histiocytosis (Histiocytosis X)
age?
neoplastic proliferation of?
xrays show?
histology?
vertebra plana? wtf
## Footnote
* LCH monostotic/eosinophilic granuloma
* Monostotic
* Highly destructive lesion with well-defined margin
* Self-limiting
* Hand-Schüller-Christian disease
Multiple bone lesions and visceral disease (skull defects, exophthalmos, and diabetes insipidus)
* Letterer-Siwe disease (a fulminating condition in young children), Typically fatal
66
# GCT
## Footnote
always on exam
## Footnote
* GCT is a benign but aggressive neoplasm that in rare cases metastasizes to the **lung.**
* It most commonly occurs about the knee and sacrum. Pain is referred to the involved joint.
* Radiographs demonstrate a **purely lytic destructive lesion in the metaphysis that extends into the epiphysis** and often borders the subchondral bone.
* Treatment is aimed at removal of the lesion with preservation of the involved joint. Curettage and subsequent reconstruction with subchondral bone grafts or methylmethacrylate is frequently performed.
67
# GCT patholgy
## Footnote
always on exam
## Footnote
* GCT is a benign but aggressive neoplasm that in rare cases metastasizes to the **lung.**
* It most commonly occurs about the knee and sacrum. Pain is referred to the involved joint.
* Radiographs demonstrate a **purely lytic destructive lesion in the metaphysis that extends into the epiphysis** and often borders the subchondral bone.
* Treatment is aimed at removal of the lesion with preservation of the involved joint. Curettage and subsequent reconstruction with subchondral bone grafts or methylmethacrylate is frequently performed.
68
# GCT bone
* stromal malignant cells produce?
* what's responsible for the osteolytic nature of disease?
* treatment?
* how does Denosumab work?
* RANKL (receptor activator for nuclear factor kB ligand)
* Multinucleated giant cell express RANK, and are responsible for the osteolysis
* tx: remove lesion preserve jt. Chemical cauterization can be used (phenol, peroxide). Area of defect reconstructed with cubchondral bonegrafts, methylmethacrylate, or both.
* Denosumab: monoclonal antibody that binds to RANKL, inhibiting maturation of osteoclasts
69
# GCT
most commonly seen in what location?
whats the lineage of the neoplastic cell?
Distal femur
preosteoblastic mononuclear cell
70
# Ewings
immunohistochemistry will show?
chromosomal translocation?
Why is bone marrow biopsy performed?
Metastatic disease?
Tx?
CD99 positivity, FLI-1 +
t(11;22)—with the formation of a fusion protein (EWS-FLI1)
BM biopsy for staging purposes
Mets to lung 50%, Bone 25%, Bone Marrow 20%
standard tx: chemotherapy and surgery;
irradiation may be used where surgical resection is not feasible (sacrum/spine).
## Footnote
* A distinctive small, round cell sarcoma that occurs most often in children and young adults; most affected children are older than 5 years.
* Radiographs show a large destructive lesion that involves the metaphysis and diaphysis. Periosteum may be lifted off in multiple layers, resulting in a characteristic but uncommon onion-skin appearance.
71
# ewings sarcoma
age?
presentation?
Histology features?
Xrays/MRI features?
Tx?
72
# mets
What cancers metastasize to bone?
## Footnote
* The patient over 50 years old with a destructive bone lesion should be presumed to have metastatic disease.
* History, physical examination, and radiographic staging (CT of chest, abdomen, and pelvis) identify the primary source of metastasis in 85% of cases; biopsy is necessary for definitive diagnosis.
* The five carcinomas that are most likely to metastasize to bone are those of the prostate, thyroid, breast, lung, and kidney.
* Pathologic fractures secondary to metastatic disease occur most commonly in the spine.
* Histologic hallmark is the appearance of epithelial cells in a fibrous stroma; the epithelial cells are often arranged in a glandular pattern.
* Bone destruction in metastatic disease results from activation of osteoclasts. Tumor cells secrete PTHrP, which stimulates RANKL release and results in activation of osteoclasts.
73
lytic lesion in patient over 50?
What's the next test? whats on the differential? What other tests need to be ordered?
## Footnote
* The patient over 50 years old with a destructive bone lesion should be presumed to have metastatic disease.
* History, physical examination, and radiographic staging (CT of chest, abdomen, and pelvis) identify the primary source of metastasis in 85% of cases; biopsy is necessary for definitive diagnosis.
* The five carcinomas that are most likely to metastasize to bone are those of the prostate, thyroid, breast, lung, and kidney.
* Pathologic fractures secondary to metastatic disease occur most commonly in the spine.
* Histologic hallmark is the appearance of epithelial cells in a fibrous stroma; the epithelial cells are often arranged in a glandular pattern.
* Bone destruction in metastatic disease results from activation of osteoclasts. Tumor cells secrete PTHrP, which stimulates RANKL release and results in activation of osteoclasts.
74
# mets
general evaluation for metastatic disease: what tests need to be ordered?
## Footnote
* The patient over 50 years old with a destructive bone lesion should be presumed to have metastatic disease.
* History, physical examination, and radiographic staging (CT of chest, abdomen, and pelvis) identify the primary source of metastasis in 85% of cases; biopsy is necessary for definitive diagnosis.
* The five carcinomas that are most likely to metastasize to bone are those of the prostate, thyroid, breast, lung, and kidney.
* Pathologic fractures secondary to metastatic disease occur most commonly in the spine.
* Histologic hallmark is the appearance of epithelial cells in a fibrous stroma; the epithelial cells are often arranged in a glandular pattern.
* Bone destruction in metastatic disease results from activation of osteoclasts. Tumor cells secrete PTHrP, which stimulates RANKL release and results in activation of osteoclasts.
75
metastasis distal to elbow and knee are usually what kind of primaries??
distal to elbow and knee, usually lung and renal primaries
76
# mets
how do metastatic lesions look on histology?
77
# mets
treatment of impending path fx and healing rates?
78
# anuerysmal bone cyst
age?
location?
characteristic xray findings
mri findings
tx
IHC
## Footnote
* ABC is nonneoplastic but aggressive in its ability to destroy normal bone and extend into the soft tissues.
* Characteristic radiographic finding is an eccentric, lytic, expansile area of bone destruction in the metaphysis. Fluid-fluid levels are characteristically visible on T2-weighted MRI.
* Treatment is with curettage and bone grafting. Local recurrence is common in children
IHC: USP6 positive (chromosome 17p13)
79
# simple/unicameral bone cyst
age? location? presentation?
imaging findings?
histology?
tx?
## Footnote
* Unicameral bone cyst most commonly involves the proximal humerus and manifests either with pain or with a pathologic fracture.
* Characteristic radiographic finding is symmetric cystic expansion with thinning of the involved cortices.
* Comparison of ABC and unicameral bone cyst (simplified):
* ABC manifests with pain and swelling. Unicameral bone cyst manifests with pathologic fracture and pain.
* ABC is eccentric and can expand wider than the growth plate. Unicameral bone cyst is central and does not expand wider than the growth plate.
80
# ABC vs UBS
occurance with other lesions?
fallen leaf sign?
width of lesion on xray?
treatment?
81
# Pagets disease
disorder characterized by?
Medical treatment?
risk of malignancy and prognosis?
* This disorder is characterized by abnormal bone remodeling, which results in coarsened trabeculae and remodeled cortices.
* Medical treatment of Paget disease is aimed at retarding the activity of the osteoclasts. Agents used include bisphosphonates and calcitonin.
* Fewer than 1% of patients with Paget disease experience malignant degeneration with the formation of a sarcoma within a focus of Paget disease.
* Paget sarcomas are deadly tumors with a poor prognosis (the rate of long-term survival is less 20%).
82
# pagets vs pelvic mets prostate ca
how to spot the difference
83
osteomyelitis can turn into??
Chronic draining wounds can turn into squamous cell carcinoma (marjolin's ulcer)
## Footnote
beware of chronic draining wounds
often stimulates primary tumor
culture what you biopsy, and biosy what you culture
84
myxoid liposarcoma classic translocation
12;16 chromosomal translocation
85
2nd most common soft tissue sarcoma in adults?
Liposarcoma is the second most common soft tissue sarcoma in adults. It virtually never occurs in the subcutaneous tissues. MRI demonstrates thicker and more irregular septa than in lipomas, and also appear bright on T2-weighted images.
86
what is the most common malignant soft tissue sarcoma in adults?
Undifferentiated pleomorphic sarcoma, previously known as malignant fibrous histiocytoma, is the most common malignant sarcoma of soft tissue in adults. It appears on MRI as a deep-seated, inhomogeneous mass that has a low signal on T1-weighted images and a high signal on T2-weighted images. Treatment is with wide-margin local excision and adjuvant radiotherapy.
87
# fibromatosis: extra-abdominal desmoid
associated with?
grows during?
pathology?
treatment?
## Footnote
Extraabdominal desmoid tumors are “rock-hard.” Patients with Gardner syndrome (familial adenomatous polyposis) have a 10,000-fold increased risk for such tumors. Estrogen receptor β inhibitors can be used for treatment.
88
plantar/palmar fibrosis vs sarcoma? how to tell the difference?
## Footnote
Fibrosarcoma follows the same imaging patterns on MRI scan and has the same treatment as any high-grade soft tissue sarcoma—limb salvage surgery, perioperative radiotherapy, and long-term surveillance.
89
schwannoma
90
neurofibroma:
gene association?
* Neurofibromatosis can manifest with more than one neurofibroma or one plexiform neurofibroma, with café au lait spots, with Lisch nodules (melanocytic hamartomas in the iris), and with anterolateral tibial bowing.
* Patients with NF1 have a 5% chance of malignant degeneration of a neurofibroma to an MPNST.
91
neurofibroma vs schwannoma
92
malignant peripheral nerve sheath tumor: MRI imaging patterns, and treatment?
MPNST follows the same imaging patterns on MRI and has the same treatment as any high-grade soft tissue sarcoma—limb salvage surgery, perioperative radiotherapy, and long-term surveillance.
93
# AVM/hemangioma
how does it present, what will imaging show? pathology whill show? treatment?
## Footnote
Vascular malformations are common in children and adults. Radiographs may reveal phleboliths. Multiple hemangiomas are associated with Maffucci syndrome.
94
# PVNS
Pigmented vilonodular synovitis
Tenosynovial Giant Cell tumor tendon sheath
## Footnote
* PVNS most commonly affects the knee, followed by the hip and shoulder. Recurrent, atraumatic hemarthrosis with associated pain is the most common manifestation. Radiographs may show cystic erosions on both sides of the joint. Histologic study reveals highly vascular villi lined with plump, hyperplastic synovial cells; hemosiderin-stained, multinucleated giant cells; and chronic inflammatory cells.
* On both T1- and T2-weighted MRI sequences, PVNS appears as a low-signal lesion.
* Treatment of PVNS is complete synovectomy for diffuse disease and local resection for focal nodular PVNS.
95
# synovial chondromatosis
96
synovial sarcoma vs synovial chondromatosis
* Synovial sarcoma does not originate from an intraarticular location. It most commonly occurs about the knee and is the most common sarcoma of the foot. Spotty mineralization on radiographs is highly characteristic.
* Histologic study of synovial sarcoma reveals a biphasic pattern: epithelial cells (resembling carcinoma) and spindle cells (resembling fibrosarcoma).
* All cells of synovial sarcomas have a translocation between chromosome 18 and the X chromosome that produces two gene fusion products (SYT-SSX1 and SYT-SSX2). Staining of tumor cells yields positivity for keratin and EMA.
97
# mineralization in soft tissue
Benign (5)
malignant (3)
Benign: lipoma, hemangioma, myositis ossificans, cppd, calcific tendonitis
Malignant: synovial sarcoma, liposarcoma, angiosarcoma
98
lesions on both sides of the joint?
HIP (P) Cats
Hemophilia (Blood)
Infection (Pus)
Pannus (inflammatory arthritidity)
PVNS
CPPD
Arthritis
TB
Synovial chondromatosis
99
most common sarcoma of the hand?
Epithelioid sarcoma is the most common sarcoma of the hand. Lymph node metastases are common.
100
# rare
clear cell sarcoma, produces? translocation? mets to?
* Clear cell sarcoma is a melanin-producing lesion, but its cells have a t(12;22) translocation not present in melanoma cells. Lymph node metastases are common.
101
excisional biopsy vs incisional biopsy/core/fna?
Excisional biopsy ok if
lesion 3cm or less (1.5 cm in hand/feet)
not near critical structures
lesion consistent with fat on all images and sequences
102
# tumor markers
CEA
CA-19-1
CA-125
CA-15-3
AFP
CEA: colorectal
CA-19-1 Pancreatic
CA-125 Ovarian
CA-15-3 Breast
AFP- HCC
103
# Fibrous Dysplasia: HY
mutation?
xrays show?
if in proximal femur?
Associated condition?
Histology?
104
# Osteoid Osteoma HY
Mechanism of Pain?
Scoliosis occurs on what side?
Histology shows?
Osteoblastoma differs in presentation how?
New non-invasive option?
105
# Osteochondroma HY
Imaging: exotosis grows which way? Continous with?
What size of cap do you need to worry about?
Symptomatic osteochomdroma that fails non op, tx?
Imaging: exostosis grows away from physis; medullary cavity of exostosis is continuous with medullary canal of involved bone
Cartilage cap (hyaline cartilage) >2cm poses risk for malignant transformation
Symptomatic osteochondroma that fails nonoperative treatment: marginal resection at base of stalk; make sure to remove entire cartilage cap
106
# NOF HY
how does it look?
Histology shows?
Prognosis?
Eccentric “soap bubbles” lesion in metaphysis, thin sclerotic rim
Histology: spindle cells in storiform pattern
Usually resolve spontaneously
107
# MHE HY
What are the genetics involved?
risk of malignant transformation?
How are the patients clinically?
MHE patient with loss of forearm rotation gets?
108
# Eosinophilic Granuloma HY
Spine: buzz word
Histology
Hand-Shuller-Christian:
Spine: vertebra plana
Histology: Langerhan’s cells with coffee bean nuclei, Birbeck granules
Hand-Schuller-Christian: lytic skull lesions, diabetes insipidus, exophthalmos
109
# GCT HY
GCT needs what addiotional study?
most common location?
Neoplastic cells are? not?
Treatment?
Adjuvants
* CXR (CT) Chest to evaluate for lung metastasis
* Most common in distal femur
* Neoplastic cells are**mononuclear stromal cells (preosteoblastic mononuclear cells)**, not multinucleated giant cells
* Treatment: denosumab (RANKL inhibitor); curettage & bone graft or cement
* Adjuvants: Adjuvant: argon beam, liquid nitrogen, phenol, hydrogen peroxide, denosumab
110
# Unicameral Bone Cyst HY
lesion characteristics and type?
over time it will?
Tx?
Pathologic fracture known as? What is the treatment in this scenario?
111
# ABC HY
molecular pearl to know?
Recurrence risk associated with?
Compared to UBC, ABC is?
MRI will show which correlates to what on histology?
Tx:
112
# Bone tunmors in anterior tibia?
Adamantinoma
Histology: nests of cells in palisading pattern Metastasis to lungs
Wide excision
Osteofibrous dysplasia (ossifying fibroma): osteoblastic rimming
113
# Osteosarcoma HY
low grade and parosteal osteosarcomas associated with?
tumor proto oncogene?
Work up includes?
Metastisis to ?>>>?
Risk factors?
Generally presents a what stage?
MSTS (enneking staging system): whats worst prognostic factor?
Tx:
improved survival if?
Whats a good prognostic factor of treatment?
Worst prognosis if?
Standard 3 drug protocol?
Local recurrence related to?
Surgery alone for osteosarcoma: ?% risk of mets dz in 5 years?
Knee endoprosthesis: what reduces infection?
## Footnote
- Worse prognosis if axial tumor or >10cm
- Standard 3-drug treatment is doxorubicin, cisplatin, and high-dose MTX
- Local recurrence of primary malignant bone tumors is related to surgical margin
- Surgery alone for osteosarcoma: 80% risk of metastatic disease within 5 yrs
- Knee endoprosthesis: medial gastroc muscle flap reduces infection
114
# Ewing Sarcoma HY
Treatment?
Histology?
What increases risk of VTE in ewings?
Elevated ?? on labs is associatd with worse tumor burden?
Proximal femur replace in ewings/osteosarc, most common deficit is?
Distal femur ewing sarc or osteosarc, most durable reconstruction is?
115
# malignant fibrous histocytoma (undifferentiated pleomorphic sarcoma) HY
what is it?
How does it look on histology>
secondary MFH/UPS may arise in what patients?
Tx?
## Footnote
think UP(post radiation, pagets; pleomorphic)S(spindle cells, storiform patter)
116
# MM HY
cell type?
tests on UA?
CRAB?
whats a solitary lesion?
what causes the punched out lesions?
how does it look on bone scan?
Treatment?
117
# lymphoma HY
symptoms?
lesions are?
diagnosis?
histology?
treatment?
* B symptoms
* Diffuse lytic lesions, mottled, permeative
* Flow cytometry & cytogenetic analysis for diagnosis: need unfixed fresh tissue during biopsy
* Histology: small round blue cells, CD20+, CD45+
* Chemotherapy +/- XRT
118
# enchondroma HY
what is it?
Most common tumor in the ?
MRI: T1 and T2
Histology?
How to manage?
Isolated enchondroma has ?% risk of malignant transformation?
Olliers disease: risk?
Maffuccis: risk?
Chondrosarcoma will show?
119
# Chondroblastoma HY
1 of 3 types of tumors with this location?
Xrays show?
Histology buzz words
Mets to ?
If symptomatic?
120
# Periosteal chondroma HY
what is it? Most common location?
Xrays will show?
management?
Periosteal chondroma
Benign cartilage tumor on surface (juxtacortical) of long bones,
most often in proximal humerus
Imaging: stippled calcification, scalloping of cortex Observation if asymptomatic; marginal excision if symptomatic
121
# chondrosarcoma HY
recurrence is correlated with ??
epiphyseal location?
mesenchymal chondrosarc is different from the rest of the group in that?
Dediff chondrosarcoma:
Management?
122
# schwannoma
associated with?
MRI buzz word
Histology? (3)
management?
Schwannoma (neurilemmoma) Benign, associated with neurofibromatosis II
Target sign on MRI
Histology: S100+, Antoni A (hypercellular) & Antoni B (hypocellular) biphasic pattern
Asymptomatic → observation; symptomatic → marginal intraneural resection
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# malignant peripheral nerve sheath tumor (nerofibrosarcoma)
arise from what condition?
what suggests malignant transformation?
histology?
management?
Arise from peripheral nerve of neurofibroma (NF-1) → increased PET-CT uptake suggests malignant transformation
S100+
Wide excision & radiation
124
# neuroblastoma
most common solid tumor in?
usually in?
histology?
* Most common solid tumor in children less than2 years
* Usually in adrenal gland
* Small round blue cells arrange in rosette pattern
125
# Giant-cell tumor of tendon sheath
where does it occur?
stains?
overexpression of?
2 types?
peak age of occurance?
Presents as?
MRI will show?
Management?
High local recurrence after?
126
# desmoid tumor (extraabdominal fibromatosis)
common in what 2 locations?
associated with ? and ? so may consider ordering what test?
Treatment?
What has the lowest risk of morbidity?
127
# soft tissue sarcoma HY
work up of a soft tissue mass includes:
biopsy every soft tissue mass unless?
Treatment:
Unplanned excision w/ inadequate/positive margins, next steps?
What is most associated with local recurrence risk?
128
# Synovial Sarcoma HY
tx:
origin is not?
most common malignant sarcoma of?
Histology:
stains + for?
wide excision + radiation
Synovial sarcoma Origin is not synovium
Most common malignant sarcoma of foot
Biphasic: spindle cells & epithelial cells
Vimentin+, epithelial membrane antigen
129
# Liposarcoma HY
what cell type?
Myxoid liposarcoma: has mets to where? tx?
well differentiated liposarcoma (ALT): management?
other liposarcomas need what management:
130
# epithelioid sarcoma
most common soft tissue sarcoma of the?
histology:
Epithelioid sarcoma Most common soft tissue sarcoma of hand → can cause overlying skin ulceration (often mistaken for non-healing ulcer)
Histology: keratin+
131
# angiosarcoma HY
risk factors
CD?
Risk factor: polyvinyl chloride exposure
CD31+
132
# Leiomyosarcoma HY
sarcoma of?
Malignant sarcoma of smooth muscle, bone leiomyosarcoma (lytic bone lesions with soft tissue extension)
133
# Metastatic Disease HY
origins of bone mets?
worst vs best prognosis?
Lytic lesions from? because of?
Blast lesions from ? because of ?
If metastatic spine lesion: mgmt is?
Wide-spread lesions gets?
Embolize?
Proteinases are divided into cathepsins and MMPs: how do these work?
134
# lymphatic mets HY
what sarcomas have mets to lymph? which one is most common in kids?
**SCARE**
synovial cell
clear cell
angiosarcoma
rhabdomyosarcoma
epithelioid
135
# PPX fixation
hx of primary cancer w/ no isolated bone lesion or multiple bone lesions: next steps?
if path fx with no clear dx, next steps?
If IMN is performed and path + for sarcoma, next steps?
Mirels score?
PPX fixation with ?
136
# Myiositis ossificans HO
Fibrodysplasia ossificantion progressiva: mutation of the?
MO compared to malignancy?
Waint until when from resection?
137
# melorhesotosis HY
what is it and whats the management?
Dysplasia of cortical bone → hyperostosis, flowing candle wax along cortical surface.
Treatment is obs & medical treatment of symptoms
138
# PVNS
"Tenosynovial giant cell tumor"
overexpression of?
signal on MRI T1 and T2
management
Overexpression of Colony-stimulating factor-1 (CSF-1)
Hemosiderin deposits → low signal on T1 & T2 MRI
Symptomatic → marginal excision (synovectomy) +/- radiation
139
# synovial chondromatosis
what is it?
management?
histology shows?
Synovial chondromatosis Intraarticular loose bodies comprising synovium / cartilage
Symptomatic → synovectomy & removal of loose bodies
Histology: synovial metaplasia
140
# Dermatofibrosarcoma protuberans
what is it and is over expressed?
Cutaneous soft tissue sarcoma
Increased PDGF-beta
141
# glomus tumor
how does it present?
management?
Subungual bluish lesion: paroxysmal pain, cold intolerance
Small round blue cells
Marginal excision
## Footnote
the glomus body is a perivascular temperature regulating structure frequently located at the tip of a digit or beneath the nail
142
# Chordoma HY
cell origin?
most common in?
histology?
management?
What nerve root is most important to preserve? bowel/bladder function?
Malignant tumor of notochordal cells, most common in sacrum/coccyx
Histology: physaliferous cells; positive for keratin, S100, epithelial membrane antigen & brachyury
Wide resection (vertebrectomy with anterior & posterior reconstruction) +/- radiation
S3 nerve root is most important for preservation of bowel/bladder function
143
# tumoral calcinosis
caused by a dysfunction in?
presents as?
mutations in ?
Dysfunction of phosphate regulation → periarticular calcinosis in extra-capsular soft tissues
More prevalent in African-Americans Mutations in FGF23
144
