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MSK TUMORS Flashcards

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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
secondary chondrosarcoma
Malignant transformation of osteochondroma Usually low grade Occurs in older patients Most common location is the pelvis
26
Malignant transformation of osteochondroma
secondary chondrosarcoma must common location = pelvis
27
Disorder characterized by multiple osteochondromas
Multiple Hereditary Exostosis (MHE) Autosomal dominant inheritance Mutation in EXT1, EXT2, and EXT3 genes Higher risk of malignant transformation
28
SYMPTOMS / PHYSICAL EXAM OF OSTEOCHONDROMA
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
29
osteochondroma imaging
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
30
osteochondroma treatment
Observation = Asymptomatic lesions Marginal resection at base of stalk including cartilage cap = Symptomatic lesions Recurrence – 2-5%
31
CHONDROSARCOMA
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
most common location of chondrosarcoma
PELVIS
33
symptoms of chondrosarcoma
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
chondrosarcoma imaging
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
chondrosarcoma treatment
Generally RESISTANT to chemotherapy and radiation Intralesional curettage = Low grade lesions WIDE SURGICAL EXCISION**** = Most commonly performed
36
chondrosarcomas are generally resistant to chemo and radiation, so most common treatment =
wide surgical excision
37
NON-OSSIFYING FIBROMA
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
most common benign tumor in childhood
non-ossifying fibroma
39
symptoms of non-ossifying fibroma
ASYMPTOMATIC Found incidentally Painless May present with pathologic fracture
40
imaging of non-ossifying fibroma
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
non-ossifying fibroma treatment
most resolve on their own 1st line = non-operative Operative = curettage & bone grafting
42
UNICAMERAL BONE CYST
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
most common locations for unicameral bone cysts
near physis | proximal humerus, proximal femur
44
prognosis of unicameral bone cyst
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
active vs latent unicameral bone cyst
Active = Cyst adjacent to the physis Latent = If normal bone separates cyst from the physis
46
unicameral bone cyst symptoms
Usually asymptomatic Pain with pathologic fracture From minor trauma ~50% of patients present with pathologic fracture
47
fallen leaf sign
unicameral bone cyst Pathologic fracture with fallen cortical fragment in base of the cyst
48
unicameral bone cyst treatment
observe asymptomatic lesions - immobilization of pathologic fractures - aspiration / methylprednisolone injection operative = curettage & bone grafting
49
ANEURYSMAL BONE CYST
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
ASSOCIATED WITH OTHER TUMORS
ANEURYSMAL BONE CYST
51
location of aneurysmal bone cysts
metaphysis - spine or long bones
52
symptoms of aneurysmal bone cysts
Pain and swelling Pathologic fracture Neurologic deficits possible with spine lesions
53
imaging of aneurysmal bone cysts
``` 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
TREATMENT OF ANEURYSMAL BONE CYST
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
EWING'S SARCOMA
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
T(11:22) translocation
ewing's sarcoma
57
biggest symptoms of ewing's sarcoma
FEVER
58
EWING'S SARCOMA SYMPTOMS
``` Pain FEVER Often mimics infection Swelling Local tenderness ```
59
imaging of ewing's sarcoma
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
which 2 need a chest ct to look for pulmonary mets
Osteosarcoma Ewing's sarcoma
61
LABS FOR EWING'S SARCOMA
ESR elevated WBC elevated Anemia LDH elevated **Bone marrow biopsy = required to rule out mets to bone marrow
62
treatment for ewing's sarcoma
chemo + radiation for nonresectable resectable = chemo + limb salvage resection + radiation
63
MULTIPLE MYELOMA
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
CLINICAL FEATURES OF MM (CRAB)
hypercalcemia renal insufficiency anemia bone lesions symptoms = Localized bone pain - Spine or ribs Pathologic fracture Fatigue - Secondary to anemia, renal insufficiency, hypercalcemia
65
imaging for MM
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
LABS FOR MM
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
mainstay of treatment for MM
multi-agent chemotherapy*** other treatment = stem cell, bisphosphonates operative
68
METS - BONE LESIONS
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
most common site for METS
SPINE - THORACIC SPINE also common = proximal femur
70
symptoms of METS
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
work up for mets
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
METS TREATMENT
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
lipoma
Mineralization / calcification is worrisome for synovial cell sarcoma
74
associated conditions for ganglion
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
1st line treatment for ganglion cyst
observation aspiration = 2nd line tx

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