Tumor Flashcards
1
Q
What is the treatment for well-differentiated liposarcoma?
A
Marginal excision without radiation
2
Q
What is the genetic basis of well-differentiated liposarcoma?
A
MDM2 amplification
3
Q
t(12;16) is associated with what tumor?
A
Myxoid liposarcoma
4
Q
Expression of PAX3-FKHR fusion gene occurs in which tumor?
A
rhabdomyosarcoma (alveolar)
5
Q
Enchondromas are benign latent cartilaginous tumors composed of […] cartilage
A
hyaline
6
Q
Enchondromas undergo malignant transformation in <1% to […]
A
chondrosarcoma
7
Q
Maffucci’s syndrome is distinguished by soft-tissue […]
A
angiomas
8
Q
Maffucci’s syndrome has increased risk of […] malignancies
A
visceral (astrocytoma, GI malignancy)
9
Q
Overall risk of developing any malignancy with Maffucci’s syndrome is up to […] percent
A
100
10
Q
What is the operative treatment for enchondromas?
A
intralesional curettage and bone grafting
11
Q
What is the risk of malignant transformation to chondrosarcoma in Ollier’s disease?
A
25-30%
12
Q
The most common location of secondary chondrosarcoma is the […]
A
pelvis
13
Q
Secondary chondrosarcomas are most commonly a […]-grade tumor
A
low
14
Q
In MHE, mutations affect the […] chondrocytes of the physis
A
prehypertrophic
15
Q
What is the inheritance pattern of MHE?
A
autosomal dominant
16
Q
In MHE, a mutation in the EXT gene decreases production of […] by chondrocytes found at the physis.
A
heparin sulfate
17
Q
Which genetic mutation in MHE has a more severe form?
A
EXT-1
18
Q
In osteochondromas, do sessile or pedunculated lesions have a higher risk of malignant transformation?
A
sessile
19
Q
In adults with osteochondromas, a cartilage cap greater than […] is increased risk of chondrosarcoma
A
2 cm
20
Q
What is the treatment for secondary chondrosarcoma?
A
wide surgical resection
21
Q
Risk of malignant transformation is about […] percent with MHE develop secondary chondrosarcoma
A
5-10
22
Q
Mesenchymal chondrosarcomas are characterized by […] fusion protein
A
HEY1-NCOA2
23
Q
Mutations of […] are identified in primary and secondary conventional chondrosarcomas.
A
isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2)
24
Q
Clear cell chondrosarcoma has a strong predilection for the […] of long bones
A
epiphysis
25
The most common locations of conventional chondrosarcoma include the [...] (25% of all cases)
pelvis
26
What is the treatment for grade 2 or 3 chondrosarcoma?
Wide surgical resection
27
What is the treatment for chondrosarcoma of the pelvis, regardless of the grade?
Wide surgical resection
28
In chondrosarcomas, increased [...] activity has been shown to directly correlate with the rate of recurrence
telomerase
29
Which serum labs, if elevated, can suggest a a more aggressive osteosarcoma?
LDH and ALP
30
What are the standard chemotherapy agents for osteosarcoma?
methotrexate, doxorubicin (adriamycin), cisplatin +/- ifosfamide
31
In osteosarcoma, greater than [...] percent necrosis after neo-adjuvant chemotherapy is good prognostic sign
90
32
What is the MOA of methotrexate?
Inhibits DNA synthesis by inhibiting dihydrofolate reductase
33
What are the side effects of methotrexate?
mucositis, myelosuppression
34
What is the MOA of doxorubicin?
Blocks DNA/RNA synthesis by inhibiting topoisomerase II (pearl: fluoroquinolones inhibit prokaryotic form of topoisomerase II)
35
What are the side effects of doxorubicin?
cardiotoxicity
36
What is the MOA of cisplatin?
DNA disruption by covalent binding
37
What are the side effects of cisplatin?
renal failure, hearing loss, neurotoxicity
38
What is the MOA of ifosfamide?
DNA-alkylating agent
39
What are the side effects of ifosfamide?
hemorrhagic cystitis; renal failure
40
Osteosarcoma routinely expresses [...], which is an INHIBITOR of the JNK radiation-induced apoptosis pathway and is thus a radioresistant tumor
NFkB
41
What are two known risk factors for infection following endoprosthesis reconstruction for osteosarcoma?
>8 hours of surgery; >14 days of drain use
42
What is the most common reason for enodoprosthetic reconstruction failure for osteosarcoma?
aseptic loosening
43
What is the anatomic location of chondroblastomas?
epiphysis of long bones
44
Chondroblastomas almost exclusively arise in the [...] of skeletally immature long bones
epiphysis
45
In chondroblastoma, chondroblasts produce areas of chondroid matrix, but [...] cartilage is rarely produced
hyaline
46
Are chondroblastomas benign or malignant?
benign (but locally destructive)
47
What additional chest imaging should be obtained in chondroblastomas?
Chest XR; Chest CT only if CXR shows mets
48
"coffee bean" nuclei are classic finding in what bony tumor?
Chondroblastoma
49
Which bony tumors are positive for vimentin, neuron-specific enolase, S100+, and Sox9
Chondroblastomas
50
Chondroblastomas are positive for what four cellular markers?
Vimentin, neuron specific enolase, S100, and Sox9
51
Bony tumor that is: diaphyseal, rarely epiphyseal; nocturnal pain relieved by NSAIDs; nidus on CT
Osteoid osteoma
52
Bony tumor that is: multicystic bone lesion with fluid-fluid levels on MRI
Aneurysmal bone cyst
53
Is a giant cell tumor typically in skeletally mature or immature patients?
mature
54
Is chondroblastoma typically in skeletally mature or immature patients?
Immature
55
What is the standard of care for most chondroblastomas?
Intralesional curettage, bone grafting, local adjuvant (phenol, cryotherapy, HPO, etc)
56
In chondroblastoma, surveillance radiographs should be performed for [...] years to monitor for local recurrence
5
57
What lab is a marker of prognosis/disease severity in Multiple Myeloma?
beta-2 microglobulin
58
Tumor with histology showing: round plasma cells with an eccentric nucleus, prominent nucleolus, and clock face organization of chromatin
Multiple myeloma
59
Greater than [...] percent plasma cells on bone marrow biopsy is needed for diagnosis of multiple myeloma
10
60
What is the treatment for solitary plasmacytoma?
external beam irradiation (45-50 Gy)
61
What is the treatment for chondrosarcoma?
wide surgical resection
62
NOF most commonly occur in the [...] of long bones
metaphysis
63
What congenital syndrome has multiple NOFs, cafe au lait pigmentation, and mental retardation?
Jaffe-Campanacci syndrome
64
Bony tumor with histology of: fibroblastic spindle cells in whirled or storiform pattern and positive for hemosiderin
NOF
65
What is the treatment for NOF?
Observation
66
What is the classification of unicameral bone cysts?
active or latent
67
In a latent unicameral bone cysts, [...] separates cyst from physis
normal bone
68
An active unicameral bone cyst is [...] to the physis
adjacent (~1cm)
69
A "fallen leaf" sign is pathognomic for what bony tumor?
Unicameral bone cyst
70
Aneurysm bone cysts typically have a transverse diameter [...] than the epiphysis
wider
71
Unicameral bone cysts have a transverse diameter is [...] than the epiphysis
smaller
72
What is the most commonly described translocation of an aneurysmal bone cyst?
TRE17-USP6
73
What is the treatment for an aneurysmal bone cyst without fracture?
Curettage, bone grafting, +/- local adjuvant (phenol, argon beam, liquid nitrogen)
74
What is the treatment for aneurysmal bone cyst with fracture?
non-operative, allow to heal, then treat with curettage + bone grafting
75
Fibrous dysplasia is a developmental abnormality caused by a [...] protein mutation
Gs alpha
76
Patients with fibrous dysplasia and subsequent malignant transformation have [...] overall survival rate compared with primary sarcomas
lower
77
Medical management with [...] is indicated in symptomatic patients with Paget's disease.
bisphosphonates
78
Increased [...] is the primary cellular abnormality in Paget's disease.
osteoclastic bone resorption
79
What is the primary cellular abnormality in Paget's Disease
Increased osteoclastic bone resorption
80
What viruses are associated with Paget's disease?
Paramyxovirus; RSV
81
In Paget's disease, serum calcium levels are [...]
normal
82
Paget's osteoclasts are [...] with [...] nuclei than typical osteoclasts
larger; more
83
Which medication is contraindicated in Paget's disease due to risk of secondary osteosarcoma?
Teriperatide
84
What is the most common complication of a TKA in a patient with Paget's disease?
Malignment (pagetoid bone increases risk)
85
What is the most common complication of a THA in a patient with Paget's disease?
Bleeding (treat preoperatively with meds)
86
Heterotopic ossification occurs between what two structures?
muscle and capsule
87
What start point has a higher risk of heterotopic ossification in antegrade femoral nailing?
piriformis
88
Which approach to the acetabulum has the highest rate of HO?
Extended iliofemoral
89
Which approach to the femoral head has the highest rate of HO?
Anterior
90
Do cemented or cementless components have a higher risk of HO in THA?
Cementless
91
Which approach in a THA has the lowest risk for HO?
Posterior
92
What dose of indomethacin is used to prevent HO?
75 mg/day x 10days-6 weeks
93
What genetic defect is seen in fibrous dysplasia?
G protein; affects the cAMP
94
In Achondroplasia, a mutation in the FGFR3 gene leads to abnormal [...] production by chondroblasts
chondroid
95
Osteopetrosis is an inherited metabolic bone disease caused by defective [...] resorption of immature bone
osteoclastic
96
Prior to excision of HO, which lab should be normalized?
ALP (CRP could also be a marker)
97
Giant Cell Tumors are benign, aggressive tumors typically found in the [...] of long bones
epiphysis
98
What is the most common location of giant cell tumor?
Distal femur
99
In giant cell tumor, which locations have the higher risk of metastasis?
hand and wrist
100
In giant cell tumors, overexpression of [...] by mononuclear stromal cells considered the instrumental pathogenic pathway
RANK-Ligand
101
What two medications can be used in the treatment of giant cell tumor?
denosumab; bisphosphonate
102
What is the treatment for giant cell tumor?
curettage, bone grafting, local adjuvant (phenol, cryo, argon beam, etc)
103
What is the MOA of denosumab?
monoclonal antibody against RANK-Ligand
104
Giant cell tumor can undergo malignant transformation to what tumor?
high grade sarcoma
105
What is the translocation of Ewing Saroma?
11:22
106
What is the fusion protein of Ewing sarcoma?
EWS-FLI1
107
Positive CD99 is seen in what tumor?
Ewing Sarcoma
108
What is the treatment for Ewing sarcoma?
neoadjuvant chemo + wide surgical resection + adjuvant chemo +/- radiation
109
Patients with Ewing sarcoma and lung mets should undergo what additional adjuvant therapy?
Radiation
110
What chemotherapy agents are used in Ewing sarcoma?
vincristine, doxorubicin, cyclophosphamide
111
What is the most important prognostic indicator in Ewing sarcoma?
mets
112
Are lung mets or bone mets a better prognostic indicator for Ewing sarcoma?
lung mets
113
Ewing sarcoma tumors greater than [...] in size are a poor prognostic indicator
8 cm
114
Age greater than [...] is a poor prognostic indicator in Ewing sarcoma.
14
115
A [...] mutation in addition to t(11:22) translocation is a worse prognostic indicator
p53
116
What are the four most common bony sites for metastatic cancer?
spine >pelvis >proximal femur > humerus
117
acral (distal extremities) lesions are rare, but when present are most commonly from [...] carcinoma
lung
118
What cell adhesion molecule (CAM) on tumor cells modulates release from primary tumor focus into bloodstream?
E cadherin
119
It metastatic cancer, tumor cells secrete [...] which stimulates the release of RANKL from osteoblasts
PTHrP (parathyroid related protein)
120
Which two metastatic cancers cause osteoblastic lesions?
prostate and breast
121
Tumor secretion of [...] leads to metastatic osteoblastic lesions
endothelin-1
122
What is the valveless venous plexus of the spine that provides a route of metastasis?
Batson's vertebral plexus
123
A Mirel's score greater than [...] indicates prophylactic fixation
8
124
What is the best predictor for fracture healing due to a metastatic pathologic fracture?
life expectancy >6 months
125
Radiation therapy for metastatic pathologic fracture should begin [...] weeks post surgery
2-3
126
Which bony mets should undergo preoperative embolization?
renal and thyroid cancer
127
What translocation is associated with clear cell sarcoma?
t12:22
128
What translocation is associated with synovial sarcoma?
t(X;18)
129
What translocation is associated with chondrosarcoma?
t(9;22)
130
What translocation is associated with myxoid liposarcoma?
t(12;16)
131
What is the most common site for mets in giant cell tumor of the bone?
lungs
132
What medication has been found to be most effective at reducing skeletal morbidity in giant cell tumor of the bone?
Denosumab (more effective the bisphosphonates)
133
What fusion protein is seen in synovial sarcoma?
SYT-SSX1
134
Which drug is a human monoclonal antibody which targets receptor activator of nuclear factor kappa-Β ligand (RANKL)?
Denosumab
135
Is renal cell carcinoma radiosensitive?
no
136
Is renal cell carcinoma chemosensitive?
no
137
Who’s soft tissue sarcomas metastasize to the lymph nodes?
Rhabdomyosarcoma, angiosarcoma, clear cell sarcoma, epithelia sarcoma, synovial sarcoma
138
A poor prognostic indicator for soft tissue sarcoma is a mass greater than […] cm
5
139
What additional CT imaging is needed for myxoid liposarcoma?
CT chest, abdomen, pelvis
140
What is the standard treatment for soft tissue sarcoma?
Wide surgical resection + radiation
141
What is the treatment for synovial sarcoma?
Wide surgical resection + radiotherapy + chemotherapy
142
What is the first line treatment for lung Mets in soft tissue sarcoma?
Surgical resection
143
In soft tissue sarcoma, resection of greater than […] cm of periosteum increases the risk of fracture and prophylactic fixation should be considered
10
144
What is the standard preop dose of radiation therapy for soft tissue sarcomas?
50 Gy
145
What is the standard post dose of radiation therapy for soft tissue sarcomas?
66 Gy
146
Pre-operative radiotherapy for soft tissue sarcoma is associated with a […] percent risk of wound complications.
20-30
147
Following resection of soft tissue sarcoma, the most common location for recurrence of low grade STS is […]
Locally
148
What is the most common mistake in treatment of soft tissue sarcomas?
Unplanned excision
149
What is the most common location for PVNS?
Anterior knee (patellofemoral compartment)
150
PVNS is caused by over expression of […] gene
CSF-1
151
What is the gold standard for diagnosis of PVNS?
Diagnostic arthroscopy
152
What is the MOA pexidartinib?
CSF-1 receptor antagonist
153
What medication can be used for PVNS?
Pexidartinib
154
155
Translocation t(X;18) is seen in what tumor?
Synovial sarcoma
156
What is the translocation mutation fusion protein seen in synovial sarcoma?
SS18;SSX
157
What is the most common sarcoma of the foot?
Synovial sarcoma
158
What chromosomal translocation is observed in synovial sarcoma?
t(X;18)
159
Which fusion protein observed in synovial sarcoma has a better survival prognosis?
SYT-SSX2
160
What type of biopsy should be performed for synovial sarcoma?
Core need or incision (not FNA)
161
What is the treatment for synovial sarcoma?
Wide resection + radiotherapy
162
What is the most important prognostic factor for synovial sarcoma?
SYT-SSX fusion protein type
163
Extra-abdominal desmoid tumors are 100% positive for what genetic receptor?
Estrogen receptor beta
164
What is the most common type of liposarcoma?
Myxoid liposarcoma
165
Is myxoid liposarcoma low, intermediate, or high grade?
Intermediate
166
What additional CT imaging is used for staging in myxoid liposarcoma?
CT CAP with contrast
167
Immature lipoblasts (signet ring type cells) are seen in what type of tumor?
Liposarcoma
168
What is the treatment for atypical lipomatous tumors?
Marginal resection without radiotherapy
169
Myxoid chondrosarcoma is associated with what translocation?
t(9;22)
170
Clear cell sarcoma is associated with which translocation?
12;22
172
Bone cells are derived from undifferentiated […] cells
Mesenchymal
173
Osteoclasts originate from […] cells
Myeloid hematopoietic cells
174
[…] are site of bone resorption where ruffled borer meets bone surface.
Howship’s lacunae
175
Which osseous cell secretes tartrate-resistant acid phosphatase?
Osteoclast
176
What is the major proteolytic enzyme that digests organic matrix at the ruffled border?
Cathepsin K
177
Which sequence of extracellular bone proteins directly allows binding to integrins?
Arg-Gly-Asp (RGD)
178
Does calcitonin activate or inhibit osteoclasts?
Inhibit
179
Which somite layer becomes the vertebral bodies and annulus fibrosus?
Sclerotome
180
Which embryonic structure forms the spinal cord?
Neural tube
181
Which embryonic structure forms the anterior vertebral bodies and nucleus pulposus?
Notochord
182
The nucleus pulposus is formed by what embryonic structure?
Notochord
183
The annulus fibrosus is formed by what embryonic structure?
Sclerotome
