MSK TUMORS Flashcards

1
Q

BENIGN BONE LESIONS

A
Well defined
Narrow zone of transition
No cortical destruction
Confined within the bone
No soft tissue reaction
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2
Q

AGGRESSIVE/MALIGNANT FEATURES

A
Ill-defined border
Wide zone of transition
Large lesion
 Not confined by natural barriers
Growth plate, cortex
Cortical destruction / fracture
Soft tissue involvement
Ill- defined
Wide zone of transition
Cortical destruction
Not confined within the bone
Soft tissue reaction
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3
Q

BIOPSY TYPE
Provides cytologic (cellular) specimen only
Frequently used for carcinoma
Not used for sarcomas

A

FNA

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4
Q

BIOPSY TYPE
Allows for tumor structural examination
Cytologic and stromal elements
Frequently used for sarcomas

A

core biopsy

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5
Q

OSTEOID OSTEOMA

A

benign bone tumor
usually in lower extremities

Nidus = Central nodule with osteoblastic rim

Reactive zone = Area of thickened bone

Pain= Increased local concentration of prostaglandin E2 and COX 1&2 expression = which is why NSAIDS HELP!!!!

PAIN = constant & progressive
- pain is worse at night and after drinking alcohol

TREATMENT = NSAIDS!!!! - and observation
- can ablate or surgical resection with curettage

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6
Q

osteoid osteoma imaging

A

if < 1.5 cm = osteoid osteoma
if > 1.5 cm = osteoblastoma

Radiographs
2-3 views of the affected bone or joint
Intensively reactive bone around radiolucent nidus

CT
Helps to identify size and location of the nidus
osteoid osteoma Nidus <1.5cm
(osteoblastoma – nidus >1.5cm)

Bone scan
Hot area at the nidus

MRI
Usually not indicated

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7
Q

PROGNOSIS OF OSTEOID OSTEOMA

A

Pain from lesion usually resolves after ~ 3 years

Lesion spontaneously resolves in 5-7 years

In the spine early resection (within 18 months) leads to resolution of scoliosis in children <11 years

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8
Q

OSTEOSARCOMA

A

Most common primary sarcoma of bone

Usually occurs in children and young adults

Majority occur during the second decade of life

Can occur in the elderly and is associated with Paget’s disease

Most common site – DISTAL FEMUR

Other common sites – proximal humerus, proximal femur, pelvis

10-20% of patients will present with pulmonary metastases

Associated with the RETINOBLASTOMA TUMOR SUPPRESSOR gene (Rb)

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9
Q

MOST COMMON SIGHT FOR OSTEOSARCOMA

A

distal femur

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10
Q

what gene is osteosarcoma associated with

A

RETINOBLASTOMA TUMOR SUPPRESSOR gene (Rb)

also associated with Paget’s disease

and pulmonary mets

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11
Q

osteosarcoma symptoms & prognosis

A

Rapidly progressive painful mass

Prognosis
76% long-term survival with modern treatment

POOR PROGNOSTIC FACTORS
Advanced stage of disease
Poor response to chemotherapy
Large tumor size 
Pelvic location
Skip lesions
High serum ALP, LDH
Vascular involvement
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12
Q

osteosarcoma requires CT of chest?

A

to assess for pulmonary mets

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13
Q

OSTEOSARCOMA IMAGING

A

Radiographs
Characteristic blastic and destructive lesion
Sun burst or hair on end periosteal reaction
Codman’s triangle periosteal reaction
Large soft tissue mass

MRI
Soft tissue involvement
May see neurovascular involvement
Assesses for skip lesions

Bone scan
Hot

CT Chest
Required to look for pulmonary metastases

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14
Q

OSTEOSARCOMA TREATMENT

A

chemo, then surgical resection

may need amputation (if pathologic fracture, vascular involvement, or progression of tumor despite chemo)

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15
Q

ENCHONDROMA

A

A benign chondrogenic tumor composed of hyaline cartilage

Located in the medullary cavity

Caused by an abnormality of chondroblast function in the physis

Chondroblasts and fragments of epiphyseal cartilage escape from the physis into the metaphysis and proliferate there

Second most common benign cartilage lesion

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16
Q

1% chance of enchondroma transforming into

A

chondrosarcoma (malignant)

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17
Q

most common location for enchondroma

A

HANDS

location: Diaphyseal or metaphyseal medullary cavity

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18
Q

SYMPTOMS OF ENCHONDROMA

A

Often asymptomatic and discovered incidentally
Especially in long bone and foot enchondromas

Pathologic fracture

Hand enchondromas

Pain = uncommon

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19
Q

endochondroma imaging

A

in hand = appear more lytic

but in other locations = more popcorn stippling appearance

XRAY
Well defined, lucent, central medullary lesions that calcify over time

1-10cm in size

Appear in the metaphysis but become diaphyseal as the long bone grows

Popcorn stippling, arcs, whorls, rings
May be purely lytic in the hands

Minimal endosteal erosion
May see cortical expansion in the hands and feet

CT, bone scan, MRI
Usually not indicated

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20
Q

TREATMENT FOR ENDOCHONDROMA

A
  • observation
  • intralesional curettage & bone grafting
  • if pathologic fractures in hand = immobilization till healed, then intralesional curettage & bone grafting
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21
Q

MOST COMMON BENIGN TUMOR OVERALL

A

OSTEOCHONDROMA

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22
Q

OSTEOCHONDROMA

A

MOST COMMON BENIGN BONE TUMOR OVERALL

Chondrogenic lesion derived from aberrant cartilage from the perichondral ring

Cartilaginous cap with varying amounts of ossification and calcification

True incidence is unknown as many are asymptomatic

Common in adolescents and young adults

occur at the surface of the bone

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23
Q

MOST COMMON LOCATION FOR OSTEOCHONDROMA

A

around the knee - proximal tibia, distal femur

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24
Q

osteochondroma prognosis

A

Risk of malignant transformation

<1% with solitary osteochondroma

5-10% with multiple lesions

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25
Q

secondary chondrosarcoma

A

Malignant transformation of osteochondroma
Usually low grade
Occurs in older patients
Most common location is the pelvis

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26
Q

Malignant transformation of osteochondroma

A

secondary chondrosarcoma

must common location = pelvis

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27
Q

Disorder characterized by multiple osteochondromas

A

Multiple Hereditary Exostosis (MHE)

Autosomal dominant inheritance
Mutation in EXT1, EXT2, and EXT3 genes
Higher risk of malignant transformation

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28
Q

SYMPTOMS / PHYSICAL EXAM OF OSTEOCHONDROMA

A

Most lesions are asymptomatic
Usually present with painless mass
May have mechanical symptoms or symptoms of neurovascular compromise
Continue to grow until skeletal maturity
Acute onset of pain should raise suspicion for malignancy

Physical exam
Palpable mass
Firm, non-tender, and non-mobile

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29
Q

osteochondroma imaging

A

XRAY

  • can be sessile (within) or pedunculated
  • high risk of malignant transformation in sessile

Always point away from the joint
Cortex and medullary cavity of lesion is continuous with the cortex and medullary cavity of native bone
Cartilage cap is radiolucent

may use CT/MRI for better distinction

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30
Q

osteochondroma treatment

A

Observation = Asymptomatic lesions

Marginal resection at base of stalk including cartilage cap = Symptomatic lesions

Recurrence – 2-5%

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31
Q

CHONDROSARCOMA

A

Malignant primary bone tumor composed of chondrocytes

PRIMARY CHONDROSARCOMA
Low grade, high grade, dedifferentiated
Clear cell chondrosarcoma
Mesenchymal chondrosarcoma

SECONDARY CHONDROSARCOMA
Arises from benign cartilage lesions
Osteochondroma, multiple hereditary exostosis, enchondromas

Poor prognosis with axial and proximal extremity lesion and higher grade tumors

32
Q

most common location of chondrosarcoma

A

PELVIS

33
Q

symptoms of chondrosarcoma

A

PAIN is most common symptom

Variable rate of presentation dependent on tumor grade and location

Pelvic masses - may present with slowly growing mass of symptoms of bladder/bowel obstruction due to mass effect

Pathologic fracture

34
Q

chondrosarcoma imaging

A

XRAYS
Lytic or blastic lesion with reactive thickening of the cortex
Vast majority have significant cortical changes
Intralesional “popcorn” mineralization
More prevalent as the lesion ages

MRI
Helpful to determine marrow and soft-tissue involvement

CT
Helpful to assess cortical involvement

Bone Scan
Can determine location of metastatic disease
Chondrosarcoma itself is hot

35
Q

chondrosarcoma treatment

A

Generally RESISTANT to chemotherapy and radiation

Intralesional curettage = Low grade lesions

WIDE SURGICAL EXCISION** = Most commonly performed

36
Q

chondrosarcomas are generally resistant to chemo and radiation, so most common treatment =

A

wide surgical excision

37
Q

NON-OSSIFYING FIBROMA

A

most common benign tumor in childhood

Related to dysfunctional ossification

Metaphysis of long bones

80% in the lower extremity
Distal femur > proximal tibia > proximal fibula > distal tibia

38
Q

most common benign tumor in childhood

A

non-ossifying fibroma

39
Q

symptoms of non-ossifying fibroma

A

ASYMPTOMATIC
Found incidentally
Painless
May present with pathologic fracture

40
Q

imaging of non-ossifying fibroma

A

Diagnostic

Metaphyseal eccentric “bubbly” lytic lesion with surrounding sclerotic rim
Cortex may be expanded and thin
As bone grows lesion enlarge and migrate into the diaphysis
Become sclerotic as patient reaches skeletal maturity

41
Q

non-ossifying fibroma treatment

A

most resolve on their own

1st line = non-operative

Operative = curettage & bone grafting

42
Q

UNICAMERAL BONE CYST

A

BENIGN

Serous filled lesion thought to result from temporary failure of medullary bone formation near the physis

Usually found in patients <20 years old

Proximal humerus and proximal femur most common

Arises in the metaphysis adjacent to the physis
Progresses towards the diaphysis with bone growth

43
Q

most common locations for unicameral bone cysts

A

near physis

proximal humerus, proximal femur

44
Q

prognosis of unicameral bone cyst

A

Often decrease in size and may heal as growth is complete

Fracture healing may lead to cyst resolution

Pathologic fracture may lead to growth arrest

45
Q

active vs latent unicameral bone cyst

A

Active = Cyst adjacent to the physis

Latent = If normal bone separates cyst from the physis

46
Q

unicameral bone cyst symptoms

A

Usually asymptomatic

Pain with pathologic fracture
From minor trauma
~50% of patients present with pathologic fracture

47
Q

fallen leaf sign

A

unicameral bone cyst

Pathologic fracture with fallen cortical fragment in base of the cyst

48
Q

unicameral bone cyst treatment

A

observe asymptomatic lesions - immobilization of pathologic fractures
- aspiration / methylprednisolone injection

operative = curettage & bone grafting

49
Q

ANEURYSMAL BONE CYST

A

Benign reactive bone lesion

Filled with multiple blood filled cavities

Can be locally destructive to normal bone

Can extend to soft tissues

Vast majority of patients are < 20 years

Associated with other tumors 30% of the time
Giant cell tumor
Chondroblastoma
Fibrous dysplasia
Chondromyxoid fibroma
Non-ossifying fibroma

location = spine or long bones (metaphysis)

50
Q

ASSOCIATED WITH OTHER TUMORS

A

ANEURYSMAL BONE CYST

51
Q

location of aneurysmal bone cysts

A

metaphysis - spine or long bones

52
Q

symptoms of aneurysmal bone cysts

A

Pain and swelling
Pathologic fracture
Neurologic deficits possible with spine lesions

53
Q

imaging of aneurysmal bone cysts

A
Radiographs
Expansile, eccentric, lytic lesion 
Bony septae – bubbly appearance
Usually metaphyseal
Thin rim of periosteal new bone

MRI and CT Scan
Multiple fluid lines*** within cyst
May show soft tissue involvement

54
Q

TREATMENT OF ANEURYSMAL BONE CYST

A

Non-operative = Asymptomatic lesion
Pathologic fracture - May lead to spontaneous resolution

Operative
Aggressive curettage and bone grafting
Symptomatic lesions with no fracture
\+/- phenol, argon beam, liquid nitrogen
Recurrence up to 25%
55
Q

EWING’S SARCOMA

A

Second most common malignant bone tumor in children
Distinctive small round cell sarcoma
Typically found in patients from 5-25 years of age
Location
~50% are found in the diaphysis of long bones
Pelvis, femoral diaphysis, distal femur, proximal tibia, proximal humerus

Genetics
T(11:22) translocation - Found in 95% of cases

prognosis: spine/pelvic tumors < proximal extremities < distal extremities

56
Q

T(11:22) translocation

A

ewing’s sarcoma

57
Q

biggest symptoms of ewing’s sarcoma

A

FEVER

58
Q

EWING’S SARCOMA SYMPTOMS

A
Pain
FEVER
Often mimics infection
Swelling 
Local tenderness
59
Q

imaging of ewing’s sarcoma

A

Image entire bone

Large destructive lesion in the diaphysis or metaphysis with a permeative moth-eaten appearance

Lesion may be purely lytic or have variable amounts of reactive new bone formation

Periosteal reaction = Onion skin or sunburst appearance

60
Q

which 2 need a chest ct to look for pulmonary mets

A

Osteosarcoma

Ewing’s sarcoma

61
Q

LABS FOR EWING’S SARCOMA

A

ESR elevated
WBC elevated
Anemia
LDH elevated

**Bone marrow biopsy = required to rule out mets to bone marrow

62
Q

treatment for ewing’s sarcoma

A

chemo + radiation for nonresectable

resectable = chemo + limb salvage resection + radiation

63
Q

MULTIPLE MYELOMA

A

MOST COMMON PRIMARY BONE MALIGNANCY

Neoplastic proliferation of plasma cells

Presents with skeletal lesion

Plasma cells produce immunoglobulins
IgG, IgA, IgM

Patients > 40 years of age

Males > females

2x as common in African-Americans

64
Q

CLINICAL FEATURES OF MM (CRAB)

A

hypercalcemia

renal insufficiency

anemia

bone lesions

symptoms =

Localized bone pain - Spine or ribs

Pathologic fracture

Fatigue - Secondary to anemia, renal insufficiency, hypercalcemia

65
Q

imaging for MM

A

won’t show up in bone scan**

Radiographs
Multiple “punched-out” lytic lesions
Caused by osteoclastic bone resorption

MRI
Better for pelvis and spine lesions

PET scan
93% sensitive
An emerging gold standard

66
Q

LABS FOR MM

A

Anemia
Elevated creatinine
Hypercalcemia - Excessive bone resorption
Elevated ESR
Serum protein electrophoresis (SPEP) - M spike - Abnormal immunoglobulin production

Proteinuria
Urine protein electrophoresis
May show Bence Jones proteins (secreted light chain immunoglobulins)

67
Q

mainstay of treatment for MM

A

multi-agent chemotherapy***

other treatment = stem cell, bisphosphonates

operative

68
Q

METS - BONE LESIONS

A

BONE = 3RD MOST COMMON SITE OF METS
- after lung & liver

Carcinomas that commonly spread to bone:
Breast
Lung 
Thyroid
Renal 
Prostate

Prognosis
Lung > kidney > breast > prostate > thyroid

69
Q

most common site for METS

A

SPINE - THORACIC SPINE

also common = proximal femur

70
Q

symptoms of METS

A

Pain

  • Mechanical due to bone destruction
  • Night time pain

Pathologic fracture

Neurologic deficits - Spinal cord compression with metastatic disease in the spine

Metastatic hypercalcemia

  • Confusion
  • Muscle weakness
  • Polyuria/polydipsia
  • Nausea/vomiting
  • Dehydration
71
Q

work up for mets

A

Plain radiographs of the affected limb

CT chest/abdomen/pelvis

Bone scan

Skeletal survey
Obtain in suspected myeloma and thyroid carcinoma only

Labs
CBC w/ diff, ESR, BMP, LFT’s, Ca, Phos, ALP
SPEP and UPEP

Biopsy
Indicated when a primary carcinoma is not identified on above work-up

72
Q

METS TREATMENT

A

Based on primary carcinoma

NON-OPERATIVE
Bisphosphonate therapy
Prevents osteoclastic bone destruction
Chemotherapy
Radiation
Hormone therapy

OR OPERATIVE

PREOP EMBOLIZATION = if thyroid or renal mets (very vascular)

73
Q

lipoma

A

Mineralization / calcification is worrisome for synovial cell sarcoma

74
Q

associated conditions for ganglion

A

Median or ulnar nerve compression
Volar ganglion
Hand ischemia due to vascular occlusion
Volar ganglion

most common location for ganglion cyst = hand - dorsal hand

75
Q

1st line treatment for ganglion cyst

A

observation

aspiration = 2nd line tx