Pathology Flashcards
(111 cards)
1
Q
What are the Prognostic factors in bone tumor staging, in order?
A
metastases, discontinuous tumor, grade, and size.
2
Q
What are the Prognostic factors in soft tissue tumor staging, in order?
A
metastases, grade, size, and depth.
3
Q
The most common site of metastases from bone and soft tissue sarcomas?
A
Lungs
4
Q
Broadly treatment options for Ewings and Osteosarcoma?
A
Chemotherapy is commonly used with limb salvage surgery
5
Q
Usual Soft tissue tumor treatment ?
A
surgery and XRT
6
Q
name 2 soft tissue tumors in which chemotherapy is used?
A
synovial sarcoma and rhabdomysarcoma
7
Q
generally how does chemotherapy work?
A
induces programmed cell death
8
Q
Generally how does XRT
work?
A
induces DNA damage of targeted cells.
9
Q
name 2 complications of XRT treatment?
A
late stress fractures
fibrosis
post irridation osteosarcoma
10
Q
Radiation may be pre- or postoperative. Postoperative external beam irradiation yields …………… local control rates, with a …………………of postoperative wound complications but a ……………….incidence of
postoperative fibrosis.
A
Radiation may be pre- or postoperative. Postoperative external beam irradiation yields equal local control rates, with a lower rate of postoperative wound complications but a higher incidence of
postoperative fibrosis.
11
Q
name 3 tumours that only wide local excision used ?
A
chondrosarcoma
adamantinoma, parosteal osteosarcoma, and chordoma.
12
Q
name 3 lesiosn that are treated with intralesional resection/ curettage?
A
GCT, ABC, NOF, LCH, osteoblastoma, and chondroblastoma.
13
Q
name 3 lesions in <5 yrs old?
A
rhabdomyosarcoma, osteofibrous dysplasia, leukemia
14
Q
name 3 lesions <30 yrs?
A
metaphyseal fibrous defect (nonossifying fibroma), enchondroma, unicameral bone cyst, osteosarcoma, Ewing sarcoma, osteoid osteoma, chondroblastoma, fibrous
dysplasia, giant cell tumor
15
Q
name 3 lesions >50?
A
metastatic bone disease, fibrosarcoma, malignant fibrous histiocytoma, myeloma, lymphoma, chondrosarcoma, Paget disease
16
Q
classic lesions in Ant Tibia?
A
adamantinoma, osteofibrous dysplasia
17
Q
classic lesions in Posterior cortex of distal femur:
A
parosteal osteosarcoma, periosteal desmoid
18
Q
classic lesions in epiphysis:
A
giant cell tumor, chondroblastoma, osteomyelitis (Brodie abscess), clear cell chondrosarcoma (femoral head)
19
Q
classic lesions in metaphysis
A
metaphyseal fibrous defect (nonossifying fibroma), aneurysmal bone cyst, giant cell tumor, osteosarcoma
20
Q
classic lesions in diaphysis:
A
Ewing sarcoma, fibrous dysplasia, eosinophilic granuloma (histiocytosis), multiple myeloma, osteoid osteoma/
osteoblastoma, infection.
21
Q
the principles of biopsy?
A
- Use longitudinal incisions and excise biopsy tracts if the lesion is malignant.
- Approach lesions through muscles wherever possible. However, avoid functionally important structures and neurovascular structures.
- Maintain meticulous hemostasis and—only in rare cases—use a small drain at the corner of the wound to prevent hematoma formation.
- Frozen-section analysis should be performed intraoperatively to ensure that adequate diagnostic tissue is obtained.
- Samples should be sent for bacteriologic analysis.
22
Q
What are four surgical margins of tumor excision:
A
intralesional, marginal (through reactive zone), wide (including a cuff of normal tissue), and radical (entire tumor and its compartment, including
surrounding muscles, ligaments, and connective tissues).
23
Q
RB +p53
A
Osteosarcoma: tumor suppressor genes Rb (retinoblastoma) and
24
Q
Ewing Sarcoma
A
t(11;22); gene product is EWS-FLI1
25
Synovial Sarcoma
t(X;18); gene products are SYT-SSX1 and SYT-SSX2
26
Myxoid liposarcoma
t(12;16); gene product is FUS-CHOP
27
Alveolar rhabdomyosarcoma:
t(2;13); gene product is PAX3-FKHR
28
Fibrous dysplasia:
GNAS1-activating mutation of the GSα surface protein
29
On MRI, most soft tissue malignancies are well defined (..…………………….) and heterogeneous. Any large ( cm) soft tissue
mass deep to fascia should be
On MRI, most soft tissue malignancies are well defined (pseudocapsule) and heterogeneous. Any large (>5 cm) soft tissue
mass deep to fascia should be considered a sarcoma.
30
Soft tissue sarcomas are..................…. intensity on T1-weighted sequences and ............intensity on T2-weighted images.
Soft tissue sarcomas are low intensity on T1-weighted sequences and high intensity on T2-weighted images.
31
Metastatic workup includes
CT scan of the chest
32
For liposarcoma, a CT scan of the ……………………………………….. is required because of
synchronous ..............
...............liposarcoma.
For liposarcoma, a CT scan of the abdomen and pelvis is required because of
synchronous retroperitoneal liposarcoma.
33
..................of a soft tissue sarcoma is the most common error. Residual tumor may exist, and ………………... should be
performed.
Unplanned removal of a soft tissue sarcoma is the most common error. Residual tumor may exist, and repeat excision should be
performed.
34
The most common soft tissue sarcoma of the hand
epithelioid sarcoma
35
The most common soft tissue sarcoma of the foot
synovial sarcoma
36
The primary site of metastases from soft tissue sarcomas is
Lung
37
Lymphatic metastasis occurs in what % of cases?
5%
38
tumors with a predilection for lymph node metastases are
ESARC (epithelioid sarcoma, synovial sarcoma, angiosarcoma, rhabdomyosarcoma,
clear cell sarcoma) and are the most common.
39
Extraabdominal desmoid tumors are ………….. Patients with Gardner syndrome (familial adenomatous polyposis) have a …………….-fold increased risk for such tumors. ………………….. can be used for treatment. .........….surgical resection
is recommended, but …………………. common.
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. Wide-margin surgical resection
is recommended, but local recurrence is common.
40
Nodular fasciitis is a painful rapidly enlarging mass in a person ….to …... years of age. Perform a resection with a ………. margin.
Nodular fasciitis is a painful rapidly enlarging mass in a person 15 to 35 years of age. Perform a resection with a marginal margin.
41
Undifferentiated pleomorphic sarcoma, previously known as ………………………………., is the most common malignant sarcoma of soft tissue in adults. It appears on MRI as a ...........................….mass that has a ...….signal on T1-weighted images and a ......…..signal on T2-weighted images. Treatment is with
……..-margin local excision and adjuvant ……………………………..
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.
42
Lipomas appear on MRI as well-demarcated lesions with the .........….signal characteristics as those of .................on all sequences. On ...............sequences, the lipoma has a uniformly ..............signal. Treatment of asymptomatic lesions is observation, whereas that for
expanding or symptomatic lesions is ...............excision.
Lipomas appear on MRI as well-demarcated lesions with the same signal characteristics as those of mature fat on all sequences. On fat-suppression sequences, the lipoma has a uniformly low signal. Treatment of asymptomatic lesions is observation, whereas that for
expanding or symptomatic lesions is marginal excision.
43
Myxoid liposarcoma has a classic.....................chromosomal translocation
Myxoid liposarcoma has a classic 12;16 chromosomal translocation
44
how do you identify Liposarcoma on MRI?
areas of the lesion supress with t2 fat suppression but other areas are bright on T2 fat suppression.
45
name 4 features of NF
neurofibrima
café au lait spots
Lisch nodules
anterior lateral tibial bowing
46
Patients with NF1 have a …...% chance of malignant degeneration of a neurofibroma to a …………………………………………
Patients with NF1 have a 5% chance of malignant degeneration of a neurofibroma to a malignant peripheral nerve sheath tumor (MPNST)
47
MPNST follows the same imaging patterns on MRI and has the same treatment as any high-grade soft tissue sarcoma, with ...............….surgery, perioperative …………., and long-term …………………….
MPNST follows the same imaging patterns on MRI and has the same treatment as any high-grade soft tissue sarcoma, with limb salvage
surgery, perioperative radiotherapy, and long-term surveillance
48
the most common soft tissue sarcoma in children
Rhabdomyosarcoma
49
Rhabdomyosarcoma translocation and gene product?
2:13
| PAX-FKHR
50
Angiosarcoma is associated with
Stuart-Treves syndrome, cutaneous and lymph node metastases. It is treated with radiation and wide
surgical excision.
51
Multiple hemangiomas are associated
Maffucci syndrome.
52
PVNS most commonly affects the
knee
53
On both T1- and T2-weighted MRI sequences, PVNS appears as
low-signal lesions.
54
Synovial sarcoma most commonly occurs in the
Knee
55
the most common sarcoma of the foot?
synovial sarcoma
56
synovial sarcoma translocation and gene product?
10:18
SYT SSX1
SYT SSX2
57
positive stain for Synovial Sarcoma?
Keratin
58
clear cell sarcoma translocation?
12:22
59
Sarcomas may spontaneously hemorrhage. On advanced imaging, the appearance of “hematomas” that do not have ............or ……………………. suggests that the bleeding is contained by a …………………., and this finding is suspect for a...…………….
Sarcomas may spontaneously hemorrhage. On advanced imaging, the appearance of “hematomas” that do not have fascial plane tracking or subcutaneous ecchymosis suggests that the bleeding is contained by a pseudocapsule, and this finding is suspect for a
sarcoma
60
Malignant bone tumors manifest most commonly with .
Malignant bone tumors manifest most commonly with pain.
61
soft tissue tumors, which most commonly manifest as p.
soft tissue tumors, which most commonly manifest as painless masses.
62
Bone sarcomas metastasize primarily via the …………………. route. The ...............is the most common site of metastasis.
Bone sarcomas metastasize primarily via the hematogenous route. The lung is the most common site of metastasis.
63
Osteoid Osteoma • Classically manifests with
increasing pain that is relieved by salicylates and other NSAIDs
64
In Osteoid osteoma Radiographs show
intensely reactive bone and a radiolucent nidus.
65
What are the effects of a spinal osteoid osteoma?
This lesion may produce a painful nonstructural scoliosis. This results in a curve with the osteoid osteoma on the concave side and is thought to result from marked paravertebral muscle
spasm.
66
treatment of Osteoid osteoma
CT scan–guided radiofrequency ablation is the dominant method of treatment; however, in 50% of patients treated with NSAIDs alone,
the symptoms resolve.
67
differences between OO and osteoblastoma?
Osteoblastoma may be confused with osteoid osteoma. The self-limited nidus in osteoid osteoma is <1 cm and in osteoblastoma >2 cm, with unlimited growth and pain not relieved by salicylates/
NSAIDS; both have the same histology.
68
the most common malignant bone tumor in children
Osteosarcoma
69
name 6 types of Osteosarcoma?
```
intramedullary osteosarcoma (ordinary or classic osteosarcoma), parosteal osteosarcoma, periosteal osteosarcoma, telangiectatic osteosarcoma, osteosarcoma occurring
with Paget disease, and postradiation osteosarcoma.
```
70
treatment of osteosarcoma?
Treated with neoadjuvant chemotherapy followed by resection of the tumor and adjuvant chemotherapy
the rate of long-term survival is approximately 60% to 70%.
71
treatment of parosteal OS and survival?
Treatment is by wide-margin surgical resection. Because this lesion is low grade, chemotherapy is not required.
• Long-term survival is 95%.
72
Periosteal osteosarcoma is a surface form that appears radiographically as a ............….lesion resting on a ..............cortical ……………....
• Treatment is ..................and ……………………….
• Long-term survival is %.
Periosteal osteosarcoma is a surface form that appears radiographically as a sunburst-type lesion resting on a saucerized cortical depression.
• Treatment is chemotherapy and wide surgical resection.
• Long-term survival is 85%.
73
Telangiectatic osteosarcoma is described as a “...………………..” Radiographic features are similar to those of
……………………………..
Treatment is chemotherapy and wide surgical resection.
Telangiectatic osteosarcoma is described as a “bag of blood.” Radiographic features are similar to those of aneurysmal bone cysts.
Treatment is chemotherapy and wide surgical resection.
74
Benign cartilage tumors on the surface of bone are called? and when in the medulla are called ?
Chondroma and enchondroma
75
Enchondromas appear radiographically as areas of
stippled calcifications
76
olliers disease?
Multiple enchondromas • Dysplastic bones (particularly a shortened ulna) • 30% risk of transformation to chondrosarcoma
• Random spontaneous mutation
77
Maffucci syndrome:
Multiple enchondromas and soft tissue hemangiomas (extremity and visceral)
• 100% risk of malignancy
78
characteristic feature of osteochondroma?
Characteristic appearance is a surface lesion in which the cortex of the lesion and the underlying cortex are continuous and the medullary cavity of the host bone also flows into (is continuous with)
the osteochondroma.
79
rate of malignant transformation of osteochondroma?
1 %
80
By what Thickness of the cartilage cap may increase the risk of malignancy.
>2 cm)
81
Multiple hereditary exostosis?
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% to 10% of affected patients develop a secondary
chondrosarcoma, which is low grade
82
location of a chondroblastoma?
epiphysis?
83
most common location of chondrosarcoma?
treatment?
treatment of dedifferentiated form?
pelvis
wide marginal resection
dediff - chemo and wide marginal resection.
84
Metaphyseal fibrous defect (nonossifying fibroma) is an extraordinarily common lesion, occurring in approximately what % of children?
30-40%
85
describe a characteristic NOF?
radiographic appearance is of a lucent lesion that is metaphyseal, eccentric, and surrounded by a sclerotic rim. The overlying cortex may be slightly expanded and
thinned
86
characteristic histological appearance?
fibroblastic connective tissue background, with the cells arranged in whorled bundles. Numerous
giant cells and hemosiderin deposits are visible.
87
treatment of NOF
Treatment is with observation. Curettage and bone grafting are indicated in symptomatic lesions with more than 50% of cortical
involvement.
88
Chordoma is a malignant neoplasm that arises from
primitive notochordal tissue
89
most common location of chordoma?
The most common location is the sacrococcygeal region, and second most common is the spheno-occipital region.
90
the most common primary tumor of bone?
Multiple myeloma is the most common primary tumor of bone.
91
what are bence jones proteins?
Light-chain subunits of immunoglobulins G and A are found in the urine.
92
Radiographic appearance of multiple myeloma is
punched-out lytic lesions
93
In Multiple myeloma The classic histologic appearance is sheets of …………. that appear monoclonal with immunostaining. Well-differentiated plasma cells have an ..................nucleus and a peripherally clumped,
chromatic “………………..”
The classic histologic appearance is sheets of plasma cells that appear monoclonal with immunostaining. Well-differentiated plasma cells have an eccentric nucleus and a peripherally clumped,
chromatic “clock face.”
94
treatment of MM?
chemotherapy, radiation therapy, and surgery
95
radiographic features of Ewings sarcoma?
Radiographs show a large destructive lesion that involves the metaphysis and diaphysis. Periosteum may be lifted off in multiple layers, which results in a characteristic but uncommon onionskin
appearance.
96
ewings immunohistochemistry staining?
CD99
97
treatments of Ewings
chemo radio and surgery
98
Adamanitinoma?
tibia , wide marginal resection
99
ABC
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.
100
UBC
commonly involve the proximal humerus and manifest either with pain or with a pathologic fracture.
FALLEN LEAF SIGN
101
ABC vs UBC
Aneurysmal bone cyst manifests with pain and swelling. Unicameral bone cyst manifests with pathologic fracture and pain.
• Aneurysmal bone cyst commonly occurs in the distal femur and proximal tibia. Unicameral bone cyst occurs in proximal humerus and proximal femur.
• Aneurysmal bone cyst is eccentric and can expand wider than the growth plate. Unicameral bone cyst is central and does not
expand wider than the growth plate.
102
LCH 3 forms:
This lesion manifests as entities: • 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
103
Fibrous Dysplasia • This condition is caused by a genetic activating mutation of the
GSα surface protein (GNAS1), which results in increased production of
cAMP
104
radiographic appearance of FD
ground glass
105
histology of FD
ts histologic appearance has been likened to “alphabet soup” and “Chinese letters.”
106
how do u never treat FD>
Autogenous cancellous bone grafting is never used for this disorder because the transplanted bone is quickly transformed into the woven
bone of fibrous dysplasia.
107
Polyostotic fibrous dysplasia with endocrinopathy is termed
McCune-Albright syndrome (café au lait spots, precocious puberty, and polyostotic fibrous dysplasia)
108
osteoblastic rimming?
Osteofibrous Dysplasia
109
The five carcinomas most likely to metastasize to bone
BREAST, LUNG, PROSTATE, KIDNEY, THYROID
110
Histologic hallmark OF METS?
appearance of epithelial cells in a fibrous stroma; epithelial cells are often arranged in a glandular
pattern
111
Bone destruction in metastatic disease results from :
m activation of osteoclasts. Tumor cells secrete PTHrP, which stimulates RANKL
release and results in activation of osteoclasts, CELLS THEN RELEASE TGFb AND ILGF WHICH STIMULATE CANCEL CELLS TO PRODUCE MORE PTHrP!!!!
