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Miller's Review > pathology > Flashcards

pathology Flashcards

1
Q

<5 Malignant bone lesion

A

Metastatic rhabdomyosarcoma Metastatic neuroblastoma

LCH

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2
Q

<5 Benign bone lesion

A

Osteomyelitis

Osteofibrious dysplasia

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3
Q

< 30 Malignant bone lesion

A

Ewing Sarcoma

Osteosarcoma

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4
Q

<30 benign bone lesion

A 
osteoid osteoma
osteoblastoma
chondroblastoma
ABC
LCH
osteofibrous dysplasia
NOF
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5
Q

> 30 malignant bone lesion

A 
chondrosarcoma
metastases
lyphoma
myeloma
chordoma
adamantinoma
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6
Q

> 30 benign bone lesion

A

GCT

paget disease

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7
Q

If there are multiple destructive lesions in middle-aged and older patients (age >40), the most likely diagnosis is

A

metastatic bone disease, multiple myeloma, or lymphoma

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8
Q

EPIPHYSEAL bone lesion

A

Chondroblastoma
Giant cell tumor
Clear cell chondrosarcoma (femoral head)

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9
Q

METAPHYSEAL bone lesion

A

Osteosarcoma
Chondrosarcoma
Metastatic disease

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10
Q

DIAPHYSEAL bone lesion

A 
A = adamantinoma
E = eosinophilic granuloma
I = infection
O = osteoid osteoma/osteoblastoma
U = Ewing sarcoma
Y = myeloma, lymphoma, fibrous dysplasia
Metastatic disease
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11
Q

SPINE bone lesion

A 
Anterior column
 Giant cell tumor
 Metastatic disease
Posterior column
 Osteoid osteoma/osteoblastoma
 Aneurysmal bone cyst
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12
Q

SACRUM bone lesion

A

Midline
 Chordoma
Eccentric
 Aneurysmal bone cyst/giant cell tumor/metastatic disease

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13
Q

Principles of Musculoskeletal Biopsy

A

Make longitudinal incision in line with future resection.
Biopsy through a single compartment.

Avoid critical structures (i.e., neurovascular bundles).

Biopsy the soft tissue component when present.
(Avoids weakening bone, decalcification)

Maintain strict hemostasis. Use a drain in line with the incision when needed.

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14
Q

Immunohistochemistry markers

SMA (smooth muscle actin)

A

smooth muscle

leiomyosarcoma

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15
Q

Immunohistochemistry markers

Desmin

A

skeletal muscle

rhabdomyosarcoma

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16
Q

Immunohistochemistry markers

MyoD1/myogenin (myf-4)

A

skeletal muscle

rhadomyosarcoma

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17
Q

Immunohistochemistry markers

S100

A

neural
schwannoma
MPNST

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18
Q

Immunohistochemistry markers

CD34/CD31`

A

Endothelial cells/vascularity

hemangioma, hemangioendothelioma, angiosarcoma

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19
Q

Immunohistochemistry markers

B-catenin

A

membrane marker, Wnt signaling pathway

Fibromatosis

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20
Q

Immunohistochemistry markers

CD99

A

Ewing Sarcoma

PNET

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21
Q

Immunohistochemistry markers

Keratin

A

Epitheloid sarcoma
synovial sarcoma
carcinoma
adamantinoma

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22
Q

Immunohistochemistry markers

EMA (epithelial membrance antigen)

A

epithelioid sarcomas

synovial sarcoma

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23
Q

Immunohistochemistry markers

vimentin

A

soft tissue sarcoma

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24
Q

Immunohistochemistry markers

CD20, CD45

A

lymphoma

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25
Q

Immunohistochemistry markers

CD138

A

Myeloma

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26
Q

Ewing sarcoma translocation and gene fusion product

A

11:22 (balanced)

EWS-FLI1 oncogene

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27
Q

Tumor suppressor gene mutations in oseosarcomas

A

Rb (35%)

p53 (50%)

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28
Q

Common Chromosomal Translocations and Gene

Ewings/PNET

A

11;22

EWS-FLI1

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29
Q

Common Chromosomal Translocations and Gene

myxoid liposarcoma

A

12;16

TLS-CHOP

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30
Q

Common Chromosomal Translocations and Gene

alveolar rhadomyosarcoma

A

2;13

PAX3-FKHR

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31
Q

Common Chromosomal Translocations and Gene

clear cell sarcoma

A

12;22

EWS-ATF1

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32
Q

Common Chromosomal Translocations and Gene

synovial sarcoma

A

x;19

SSX1-SYT

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33
Q 
Common Chromosomal Translocations and Gene
myxoid chondrosarcoma (extrasekelteal)
A

9;22

EWS-CHN

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34
Q

Common Chromosomal Translocations and Gene

osteosarcoma

A

none

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35
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Li-Fraumeni

A

SBLA syndrome Osteosarcoma P53

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36
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Retinoblastoma

A

Bilateral malignant tumor of the eye in children Osteosarcoma RB1

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37
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Rothmund-Thomson

A

Sun-sensitive rash with prominent poikiloderma and telangiectasias
Osteosarcoma RECQL4

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38
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Multiple hereditary exostoses

A

Multiple osteochondromas Chondrosarcoma EXT1, EXT2

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39
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Ollier disease

A

Enchondromas Chondrosarcoma PTHR1

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40
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Maffucci syndrome

A

Enchondromas + angiomas and CNS, pancreatic, and ovarian malignancies
Chondrosarcoma and/or angiosarcomas
PTHR1

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41
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

McCune-Albright

A

Polyostotic fibrous dysplasia, precocious puberty, and café au lait spots
GNAS1

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42
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Mazabraud

A

Fibrous dysplasia + soft tissue myxomas GNAS1

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43
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Jaffe-Campanacci

A

Multiple nonossifying fibromas with café au lait skin patches

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44
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

POEMS

A

Polyneuropathy (peripheral nerve damage)
Organomegaly (abnormal enlargement of organs)
Endocrinopathy (damage to hormone-producing glands)
M protein (an abnormal immunoglobulin)
Skin abnormalities (hyperpigmentation)

Myloma

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45
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Hand-Schüller-Christian disease (<5 years old)

A

Multifocal LCH and exophthalmos, diabetes insipidus, and lytic skull lesions
LCH

46
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Letterer-Siwe disease (infants)

A

Multifocal LCH, visceral and bone disease, and is fatal LCH

47
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Stuart-Treves

A

Chronic lymphedema Angiosarcoma

48
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Neurofibromatosis type 1

A

Multiple neurofibromas MPNST NF1

49
Q

Musculoskeletal Syndromes, Genes, and Neoplasms

Familial adenomatous polyposis

A

Multiple intestinal polyps, colon cancer, hepatoblastomas Desmoid tumors APC

50
Q

Grading of bone/soft tissue tumors

A

Based on histology (I–> III, well to poorly differentiated, <10% to >50% of metastatic potential)

Most malignant bone lesions are G2 (G1 are rare) while soft tissue tumors have a greater range)

51
Q

Treatment - Chemotherapy + surgery

A 
rhadomyosarcoma
osteosarcoma 
nonosteogenic osteosarcoma (malignant fibrous histiocytoma [MFH] of bone, fibrosarcoma, etc.)
periosteal osteosarcoma 
Ewing sarcoma 
dedifferentiated chondrosarcoma
mesenchymal chondrosarcoma.
52
Q

Treatment - Radiation + surgery

A

Soft tissue sarcoma (except rhadomyosarcoma - chemo)

53
Q

Treatment - Limb salvage surgery/wide excision

A

Chondrosarcoma, adamantinoma, chordoma, parosteal osteosarcoma

54
Q

Treatment - ORIF (+ radiation/chemotherapy)

A

Metastases, lymphoma, myeloma

55
Q

Treatment - Intralesional resection

A

GCT, ABC, NOF, LCH, osteoblastoma, chondroblastoma

56
Q

Treatment - Radiofrequency ablation

A

Osteoid osteoma

57
Q

Most Common Musculoskeletal Tumors - Soft tissue tumor (children)

A

Hemangioma

58
Q

Most Common Musculoskeletal Tumors - Soft tissue tumor (adults)

A

Lipoma

59
Q

Most Common Musculoskeletal Tumors - Malignant soft tissue tumor (children)

A

Rhabdomyosarcoma

60
Q

Most Common Musculoskeletal Tumors - Malignant soft tissue tumor (adults)

A

Undifferentiated pleomorphic sarcoma (UPS)

61
Q

Most Common Musculoskeletal Tumors - Primary benign bone tumor

A

Osteochondroma

62
Q

Most Common Musculoskeletal Tumors - Primary malignant bone tumor

A

Osteosarcoma

63
Q

Most Common Musculoskeletal Tumors - Secondary benign lesion

A

Aneurysmal bone cyst

64
Q

Most Common Musculoskeletal Tumors - Malignant fibrous histiocytoma
Osteosarcoma
Fibrosarcoma

A

Malignant fibrous histiocytoma
Osteosarcoma
Fibrosarcoma

65
Q

Most Common Musculoskeletal Tumors - Phalangeal tumor

A

Enchondroma

66
Q

Most Common Musculoskeletal Tumors - Soft tissue sarcoma of the hand and wrist

A

Epithelioid sarcoma

67
Q

Most Common Musculoskeletal Tumors - Soft tissue sarcoma of the foot and ankle

A

Synovial sarcoma

68
Q

Soft tissue should be presumed to be a sarcoma if…

A

> 5 cm
growing
deep to fascia
firm

get 3D imaging

69
Q

what imaging is needed for soft tissue sarcomas

A

MRI of lesion

CT of chest for mets

CT a/p if liposarcoma b/c of synchronous retroperitoneal liposarcoma

70
Q

What should you do if unplanned excision of a sarcoma occurs?

A

repeat excision

71
Q

where do most soft issue tumors metastasize?

A

lungs

lymph nodes 5%

72
Q

soft tissue sarcomas that metastasize to lymph nodes?

A

ESARC
Epithelioid sarcoma (hand, young adults)
Synovial sarcoma (x;18, SYT-SSX)
Angiosarcoma (Stewart-Treves syndrome, cutaneous spread)
Rhadomyosarcoma (peds)
Clear cell sarcoma (lower extremity/foot, young adults)

73
Q

Extraabdominal desmoid tumor

A

Most locally invasive of all benign soft tissue tumors

Patients with Gardner syndrome (familial adenomatous polyposis) have colonic polyps and a 10,000-fold increased risk of developing desmoid tumors.

distinctive “rock-hard” character

estrogen receptor positive

wide resection, but recurrence is common often years later

74
Q

malignant fibrous lesions - types, presentation, MRI, treatment

A

UPS (previously MFH)

fibrosarcoma

**30-80 years, enlargeing, painless mass

**MRI w/ deep inhomogeneous mass w/ low T1 and high T2

**treatment: wide excision, RT if >5 cm

75
Q

Liposarcomas metastasize according to…

A

the grade of the lesion:

•
Well-differentiated liposarcomas have a very low rate of metastasis (<10%).

•
The metastasis rate of intermediate-grade liposarcomas is 10% to 30%.

•
The metastasis rate of high-grade liposarcomas is more than 50%.

76
Q

Rhabdomyosarcoma -

A

most common sarcoma in young patients; may grow rapidly

spindle cells in parallel bundles, multinucleated giant cells, and racquet-shaped cells

sensitive to multiagent chemotherapy and wide-margin surgical resection after induction of chemotherapy. External beam irradiation plays a prominent role in treatment.

77
Q

Ganglia

A

Outpouching of the synovial lining of an adjacent joint

Filled with gelatinous mucoid material

Paucicellular connective tissue without a true epithelial lining

MRI low T1, high T2, do not enhance

78
Q

PVNS

A

proliferation of synovium
Knee>hip>shoulder
local = partial synovectomy
diffuse=arthroscopic for intraarticular and open posterior for extraarticular extension
high recurrence, may use RT to reduce risks

79
Q

Synovial sarcoma

A

it rarely arises from an intraarticular location.

ages of 15 and 40 years

Lymph nodes may be involved.

most common sarcoma in the foot.

t(X;18); SYT-SSX1 and SYT-SSX2.; staining of tumor cells yields positive results for keratin and epithelial membrane antigen.

Radiographs or CT scans may show mineralization within the lesion in up to 25% of cases (spotty mineralization may even resemble the peripheral mineralization seen in heterotopic ossification).

80
Q

Epithelioid sarcoma

A

Most common sarcoma of the hand

May ulcerate and mimic a granuloma or rheumatoid nodule

Lymph node metastases are common.

81
Q

night pain relieved by NSAIDs

A

Osteoid osteoma has a characteristic night pain or diurnal pain pattern relieved with aspirin or NSAIDS.

82
Q

bone sarcoma vs soft tissue sarcoma presentation

A

Malignant bone tumors manifest most commonly with pain. This is in contrast to soft tissue tumors, which most commonly manifest as a painless mass.

83
Q

bone sarcoma metastasize route…

A

Bone sarcomas metastasize primarily via the hematogenous route; lungs are the most common site.

Osteosarcoma and Ewing sarcoma may also metastasize to other bone sites either at initial manifestation or later in the disease.

84
Q

three lesions in which tumor cells produce osteoid

A

osteoid osteoma, osteoblastoma, and osteosarcoma.

85
Q

Osteoid osteoma

A

self limited, young patient, night pain better w/ NSAIDs

nonstructural scoliosis from muscle spams

imaging: radiolucent nidus (<1cm) w/ intense bone sclerosis around
treatment: 50% burn out w/ NSAIDs, RFA unless close to NV bundle or spine then surgery

86
Q

osteoblastoma

A

~osteoid osteoma but random pain not better w/ NSAIDs, nidus >2 cm w/ less reactive bone, not self limited so requires intralesional excision

87
Q

High-grade intramedullary osteosarcoma

A

Rb and P53 (Li-Fraumeni syndrome)

occurs about the knee in children and young adults

90% high grade and penetrate cortex to form soft tissue mass (stage IIB)

imaging: mixed bone destruction and formation

two histologic criteria: (1) tumor cells produce osteoid and (2) stromal cells are frankly malignant.

Treatment: neoadjuvant chemotherapy (i.e., before surgery), followed by wide-margin surgical resection and adjuvant chemotherapy (i.e., after surgery)

88
Q

Parosteal osteosarcoma (low-grade surface)

A

surface of the metaphysis of long bones - usually around knee posteriorly

painless mass

Treatment: resection with a wide margin, which is usually curative; no chemo

89
Q

Periosteal osteosarcoma (intermediate grade surface)

A

Rare surface form of osteosarcoma occurs most often in the diaphysis of long bones (typically femur or tibia).

Radiographic appearance is fairly constant: a sunburst-type lesion rests on a saucerized cortical depression

Treatment: Preoperative chemotherapy, resection, and maintenance chemotherapy constitute the preferred treatment. The risk of pulmonary metastasis is 10% to 15%.

90
Q

chondrosarcoma treatment

A

wide resection

Chemotherapy is added with dedifferentiated and mesenchymal chondrosarcoma.

91
Q

enchondroma vs low grade chondrosarcoma

A

In low-grade chondrosarcomas, cortical bone changes (large erosions [>50%] of the cortex, cortical thickening, and destruction) or lysis of the previously mineralized cartilage is visible.

Radiographs are obtained every 3 to 6 months for 1 to 2 years and then annually as necessary.

92
Q

Ollier disease/Maffucci syndrome

A

involved bones are dysplastic, and the lesions tend toward unilaterality, the diagnosis is multiple enchondromatosis, or Ollier disease.

Inheritance pattern is sporadic.

If soft tissue angiomas are also present, the diagnosis is Maffucci syndrome.

Patients with multiple enchondromatosis are at increased risk of malignancy (in Ollier disease, 30%; in Maffucci syndrome, 100% usually visceral).

surgical treatment is necessary, enchondromas are treated by curettage and bone grafting. Periosteal chondromas are usually excised with a marginal margin.

93
Q

MHE

A

osteochondromas are often sessile and large.

autosomal dominant condition with mutations in the EXT1 and EXT2 gene loci.

Approximately 10% of patients with multiple exostoses develop a secondary chondrosarcoma.

The EXT1 mutation is associated with a greater burden of disease and higher risk of malignancy.

94
Q

Chondroblastoma

A

Centered in the epiphysis in young patients, usually with open physes; may also occur in an apophysis (vs. GCT)

histo: Chondroblasts
“Chicken-wire” calcifications in a lacelike pattern

Treatment: curettage (intralesional margin) and bone grafting

95
Q

Intramedullary chondrosarcoma

A

older adults

Radiographs usually show diagnostic findings, with bone destruction, thickening of the cortex, and mineralization consistent with cartilage within the lesion

Differentiating malignant cartilage may be extremely difficult on the basis of histologic features alone.

Treatment: wide-margin surgical resection

Chemotherapy has not been shown to improve survival.

96
Q

Dedifferentiated chondrosarcoma

A

More than 80% of the lesions are typical chondrosarcomas with a superimposed highly destructive area

Prognosis is poor; rate of long-term survival is less than 10%.

Treatment: wide-margin surgical resection and multiagent chemotherapy

97
Q

Metaphyseal fibrous defect (also known as nonossifying fibroma, nonosteogenic fibroma, and xanthoma)

  • natural hx
  • appearance
  • histo
  • treatment
A

Most such lesions resolve spontaneously and are probably not true neoplasms.

Characteristic radiographic appearance: a lucent lesion that is metaphyseal, eccentric, and surrounded by a sclerotic rim. The overlying cortex may be slightly expanded and thinned.

histo: Cellular fibroblastic connective tissue background, with cells arranged in whorled bundles
treatment: observe unless If more than 50% to 75% of the cortex is involved and the patient has symptoms, curettage and fixation are performed.

98
Q

Malignant Fibrous Histiocytoma

  • origin
  • treatment
A

Also known as nonosteogenic osteosarcoma. This is a primary bone osteosarcoma with a mesenchymal origin and cellular pattern, but no osteoid is produced or seen in histology.

Treatment: same as osteogenic sarcoma—chemotherapy and surgery

99
Q

chordoma

A

primitive notochordal tissue

Occurs predominantly at the ends of the vertebral column clivus of the skull or sacrum (sacrococcygeal)

CT scans show midline bone destruction and a soft tissue mass

Treatment: wide-margin surgical resection
Radiation therapy may be added if a wide margin is not achieved.

100
Q

blue cell tumors

A

LERNM (learn ‘em)

lymphoma

ewing sarcoma

rhadomyosarcoma

neuroblastoma

myeloma

101
Q

Giant cell tumor

A

Most common in the epiphysis and metaphysis of long bones; about 50% of lesions occur about the knee. Vertebra, sacrum, and distal radius are involved in about 10% of cases.

The sacrum is the most common axial location of giant cell tumors of bone.

A purely lytic destructive lesion in the metaphysis that extends into the epiphysis and often borders the subchondral bone

Treatment is aimed at removing the lesion, with preservation of the involved joint. may use phenol, peroxide too

102
Q

Ewing tumor

A

small round cell sarcoma that occurs most often in children and young adults

When a small blue cell tumor is found in a child younger than 5 years, metastatic neuroblastoma and leukemia should be confirmed or ruled out. In patients older than 30 years, metastatic carcinoma must be confirmed or ruled out

Periosteum may be lifted off in multiple layers, which produces a Codman triangle and an onionskin appearance.

CD99 positivity. 11 : 22. EWS/FLI1

Standard treatment includes chemotherapy
Local tumor control may be irradiation or surgery.

103
Q

Adamantinoma

A

Rare low-grade malignant tumor of long bones that contains epithelium-like islands of cells

Radiographic appearance: multiple sharply circumscribed, lucent defects of different sizes, with sclerotic bone interspersed between the zones and extending above and below the lucent zones

Treatment: wide-margin surgical resection

104
Q

ABC vs UBC radiograpahs

A

ABC - eccentric, no fallen leaf sign, curettage instead of aspirate, eccentric
Width of the tumor is greater than the width of the physis

105
Q

ABC

A

May arise primarily in bone or be found in association with other tumors (e.g., giant cell tumor, chondroblastoma, chondromyxoid fibroma, fibrous dysplasia)

May arise primarily in bone or be found in association with other tumors (e.g., giant cell tumor, chondroblastoma, chondromyxoid fibroma, fibrous dysplasia)

Essential histologic feature: cavernous blood-filled spaces without an endothelial lining

Treatment: careful curettage and bone grafting
Local recurrence is common in children with open physes.

106
Q

Unicameral bone cyst (simple bone cyst)

A

proximal humerus

Symmetric cystic expansion with thinning of the involved cortices

Treatment: aspiration to confirm the diagnosis, followed by methylprednisolone acetate injection

107
Q

Five carcinomas most likely to metastasize to bone

A

“BLT and a Kosher Pickle”

breast, lung, prostate, kidney, and thyroid

Bone destruction is caused not by the tumor cells themselves but by activation of osteoclasts

108
Q

bone destruction in metastatic bone disease

A

Tumor cells secrete parathyroid hormone–related peptide (PTHrP), which stimulates release of the receptor activator for nuclear factor κB ligand (RANKL) from osteoblasts and marrow stromal cells.

RANKL attaches to the receptor activator for nuclear factor κ (RANK) receptor on osteoclast precursor cells.

109
Q

Paget disease

A

abnormal bone remodeling. Affected patients may present with degenerative joint disease, fracture, or neurologic encroachment; joint degeneration is common in the hip and knee.

Radiographs demonstrate coarsened trabeculae and remodeled cortices

Medical treatment: aimed at retarding activity of osteoclasts
Agents used include diphosphonates and calcitonin (pamidronate and Zometa).

Paget sarcoma (<1% of patients) is a deadly tumor with a poor prognosis (rate of long-term survival <20%).

110
Q

Osteofibrous dysplasia (also called ossifying fibroma or Jaffe-Campanacci lesion)

A

anterior tibial cortex: Multilocular, eccentric, lytic defects of cortex with a well-defined sclerotic border

Fibroosseous lesion WITH rimming osteoblasts (vs fibrous dysplasia which doesn’t have)

Bowing is very common

children < 10 years

These lesions usually regress and do not cause problems in adults

Miller's Review (3 decks)

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