Tumor Flashcards

(59 cards)

0
Q

Where does chondromyxoid fibroma arise?

A

Diaphysis

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1
Q

If diameter of lesion is smaller than diameter of growth plate with respect to cysts, which is it ubc or abc?

A

Ubc

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2
Q

Osteochondroma pedunculated

A

Surface lesion, ct shows corticomedullary continuity

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3
Q

Apophyseal lesion

A

Chondroblastoma, gct

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4
Q

Intraarticular lesion?

A

Synovial chondromatosis Pvns

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5
Q

Fibrous dysplasia

A

Smudged out trabeculae

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6
Q

Surface osteochondroma?

A

Exists, neocortex, thickened sclerotic border

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7
Q

Sclerotic lesion stuck on to distal femur?

A

Parosteal sarcoma - low grade

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8
Q

Mirel’s Score?

A

>8 operate

Location 1 UL, 2 LL, 3 peritroch

Pain 1 mild 2 mod 3 functional

Lesion 1 blastic 2 mixed 3 lytic

Size 1 less than 1/3, 2 between 1/3 and 2/3, 3 greater than 2/3

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9
Q

Inheritance pattern of multiple hereditary exostoses?

A

AD

So 50% chance of passing it on

Parent 1: Xx Parent 2: xx

Kids: Xx, Xx, xx or xx

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10
Q

Ddx for small round blue cell tumours in kids

A

Ewings/PNET

Rhabdomyosarcoma

Lymphoma

Neuroblastoma

Wilm’s tumour

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11
Q

Serum and urinary studies for Paget’s disease

A

Increased ALP

Increased urinary hydroxyproline, n-telopeptide, alpha c-telopeptide and deoxypyridoline

Normal calcium!

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12
Q

Extravaganza of unique histological features! Neurofibroma

Synovial Sarcoma

Epitheliod Sarcoma

OO vs osteoblastoma?

Enchondroma vs CS?

Chondroblastoma

Chondromyxoid fibroma

Malignant fibrous histiocytoma

Chordoma

ABC

Fibrous Dysplasia

Osteofibrous dysplasia

Adamantinoma

Paget’s

Liposarcoma

A

Neurofibroma: Elongated, wavy nuclei. May see pacinian/meissner corpuscles

Synovial Sarc: BIPHASIC 2 cell types (spindle and epithelial)

Epitheliod Sarc: Epithelial appearance, central necrosis. Pleiomorphism is rare and may be misdiagnosed as benign!

OO/Osteoblastoma: Both have woven bone (immature) WITH osteoblastic rim, but OO won’t have giant cells

Enchondroma vs CS? Enchondroma has “blue balls” of cartilage. CS hypercellular, binucleate cells, hyperchromatic cell.

Chondroblastoma: Chickenwire chondroblasts Chondromyxoid fibroma: Stellate cells

MFH: Spindle cells in “storiform” pattern. Herringbone pattern

Chordoma: Foamy, vaculoated “physaliferous” cells

ABC: NO endothelial lining

Fibrous dysplasia: Alphabet soup/Chinese letters. Woven bone (immature) WITHOUT osteoblastic rimming

Osteofibrous dysplasia: Woven bone WITH osteoblastic rimming. HAS islands of lamellar (mature) bone.

Adamantinoma: Gland like fibrous stroma. NO islands of lamellar bone (unlike OFD)

Paget’s dz: Mosaic pattern of woven bone. Disorganized cement lines. Osteoclastic rimming, osteoclasts look multinucleated.

Liposarcoma: Signet ring cell (lipoblast)

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13
Q

Most common tumours by location!

For hand and foot, list most common benign and sarcomatous malignant tumours of ST and bone

A

HAND:

Benign ST: Ganglion (GCT of tendon sheath 2nd most common)

Benign Bone: Enchondroma

Malignant ST: Epithelioid sarcoma. (Note: SCC is most common soft tissue overall in the hand, but not a sarcoma)

Malignant Bone: Chondrosarcoma

FOOT:

Benign ST: Plantar fibromatosis, GCT of tendon sheath, ganglion,

Benign Bone:

Malignant ST: melanoma, synovial sarcoma (most common ST sarcoma of lower extremity)

Malignant Bone:

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14
Q

List 3 soft tissue sarcomas where chemotherapy may be part of the treatment plan

A

Rhabdomyosarcoma (in kids)

Synovial sarcoma

Soft tissue Ewing’s

Soft tissue osteosarc

Dedifferentiated chondrosarc

Mesenchymal chondrosarc

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15
Q

Quoted survival rates of bone sarcomas

A

Classic osteosarc: 76% at 5 years

Parosteal: 95% at 5 years

Periosteal: In between osteosarc & parosteal.

Telangiectatic OS: 70% at 5 years.

MFH bone/Fibrosarc: 50-60% at 5 years (drops to 30% if high grade)

Chondrosarc Depends on Grade!

Gr 1: 90%, Gr 2: 60-70%, Gr 3: 30-50%

Dediff: 10%

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16
Q

Risk factors for worse prognosis in chondrosarcoma

A

Axial skeleton or proximal appendicular location

Increased telomerase activity on RT PCR

Hypercellularity on histo (indiates high grade)

Bimorphic cellularity (low grade chondroid and high grade spindle - indicates de-differentiation)

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17
Q

Risk factors for development of secondary chondrosarcoma

(hint, think clinical, radiographic and genetic

A

Clinical: pain, male gender, enlarging lesion, pelvic location, age in 30s

Radiographic: Surface irregularity/blurriness, Cartilage cap >2cm, osteochondroma >5cm

Genetic: Hereditary multiple exostosis (EXT mutations)

Mafuccis, Olliers

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18
Q

Regarding blastic-appearing metastatic lesions

A

Lung 30% blastic

Breast 60% blastic

Prostate 90% blastic

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19
Q

Epiphyseal lesions

A

Chondroblastoma (age <25)

GCT (age 30s-50s)

Clear cell chondrosarcoma

Infection (osteomyelitis)

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20
Q

Soft tissue “tumours” that may have calcifications.

A

Hemangioma (phleboliths)

Synovial chondromatosis

Synovial Sarcoma

Epithelioid Sarcoma (calc seen 10-20% as per orthobullets)

Soft tissue osteosarcoma

Soft tissue chondroma

Hamartoma

Tumoural Calcinosis

Myositis ossificans

Heterotopic ossification

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21
Q

Stains positive for S100

A

EG

Clear cell

Chordoma

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22
Q

Stains for CD1A

A

EG

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23
Q

Ewing’s stains for what?

24
What is Jaffe Campanacci syndrome?
Associated with: Multiple NOF Cafe Au Lait spots Mental retardation
25
POEMS syndrome?
Polyneuropathy Organomegaly Endocrinopathy M protein (multiple myeloma) Skin changes (hyperpigmentation)
26
Mazabraud syndrome?
Polyostotic fibrous dysplasia Intramuscular myxomas
27
McCune Albright?
Polyostotic FD (usually unilateral) Precocious puberty Cafe au lait (coast of Maine) also renal phosphate wasting from high expression of FGF23
28
Orthopaedic features of Gardner syndrome (aka Familial adenomatous polyposis)
Desmoid tumours Osteomas
29
Adjuvants for treatment of GCT after curettage
Cement H202 Liquid nitrogen Phenol Ethanol
30
Common translocations tested: Myxoid liposarcoma Rhabdomyosarcoma Synovial sarcoma Ewing's Sarcoma Clear cell sarcoma Myxoid chondrosarc
Myxoid liposarcoma 12;16 (TLS-CHOP) Rhabdomyosarcoma 1;13 & 2;13 (Pax3 FKHR) Synovial sarcoma X;18 (SYT-SSX1 fusion protein) Ewings 11:22 (EWS-FLI1 fusion protein) Clear cell 12:22 (EWS-ATF1) Myxoid chondrosarc 9:22 (EWS-CHN)
31
How to differentiate chordoma from chondrosarcoma?
Chordoma stains positive for keratin.
32
Other metastatic tumour antigens?
CEA for colorectal CA 19-9 for pancreatic CA 125 for ovarian CA 15-3 for breast AFP for hepatocellular
33
Genetic associations with osteosarcoma
Rb mutation (35% of osteosarcs) Li-Fraumeni syndrome (p53 mutation) Rothmund-Thomson syndrome (sun facial rash, no eyebrows/lashes, juvenile cataracts, other cancers BCC/SCC)
34
Preop rads vs postop rads for STS treatment? Dose?
Preop Lower dose 50 Gy More wound problems Lower field of exposure Postop Higher dose 60-65 Gy Higher field of exposure More fibrosis, joint contractures Post radiation fracture
35
Bone tumours (benign) with pulmonary mets?
Chondroblastoma (\<1%) GCT (3-5%)
36
Poor prognostic factors for osteosarcoma
Size \>8cm Proximal tumours worse than distal Axial location 2dary osteosarc (Pagets, post XRT) Present with mets (skip mets or lung) Dz progression during chemo Necrosis \<95% after chemo Positive margins Tumour recurrance within 2 years High LDH & ALP
37
Poor prognostic factors for Ewing's
Older age \>15 Axial location Large tumour volume (\>100cm3) Presents with mets/skip lesions High LDH p53 mutation in addition to 11;22 transloc. Poor response to chemo Progression while on chemo Positive margins Recurrence within 2 years
38
Oncologic use of bisphosphonates
Giant cell tumour Fibrous dysplasia Multiple myeloma Mets Paget's (don't forget hypercalcemia of malignancy) (non tumour? AVN and OI)
39
Complications of bisphosphonates?
Nausea/Vomiting Myalgias AVN jaw (not kids) GERD/Gastritis Acute renal failure Uveitis Hypocalcemia/hypophosphatemia
40
Symptoms of malignant hypercalcemia
Confusion/Agitation Weakness Nausea/anorexia Polyuria Dehydration Short QT, bradycardia, 1st degree AV block
41
Oncogenic osteomalacia - mechanism and give 4 tumour types that cause it
Overexpression of FGF23 (upregulation of phosphate transporters in kidney - leads to excess phophate excretion. Also PTHrp) Phosphaturic mesenchymal tumour Osteoblastoma Osteofibrous dysplasia Fibrous dysplasia (think McCune Albright and hypophosphatemia)
42
Osteopetrosis: Which cell is affected? Underlying problem? Describe 2 types and treatment of each.
Osteoclast - defective resorption Carbonic anhydrase II mutation Type 1 (AR) infantile - "malignant" -High dose calcitriol (1-25 dihydroxyvit D3) and bone marrow transplant Type 2 (AD) older patients -interferon gamma 1beta
43
List 5 sequelae of osteopetrosis
1. Cranial nerve palsies (overgrowth of skull base foramina. Includes HEARING LOSS) 2. Osteomyelitis (poor marrow vascularity and WBC function) 3. Long bone #s 4. Coxa vara (from repetitive stress #s) 5. Anemia (bone marrow encroachment) 6. Renal tubular acidosis (loss of carbonic anhydrase)
44
5 causes of erlenmeyer flask deformity
CHONG Craniometaphyseal dysplasia Hemoglobinopathies (sickle, thal) Osteopetrosis Niemann Pick (fat storage dz) Gaucher's Disease (glucocerebrosidase deficiency, another fat storage dz)
45
Metastatic workup!
_Hx_ Personal hx cancer Family hx cancer (only care if 1st degree relative under 50) _Physical_ Palpate thyroid, breast, abdo for HSM, nodal exam, possibly rectal. Listen to chest/percuss. _Imaging_ Local - whole bone xray/CT/MRI Systemic - bone scan, CT C/A/P, skeletal survey _Labs_ CBC/Lytes/BUN/Creat/LFTs Ca/Mg/Phos/VitD SPEP/UPEP/TSH/PSA/CEA/CA125 ALP (osteosarc) LDH (Ewing's)
46
Common tumours in the spine: Vertebral body? Posterior elements? Non-bony?
Body Mets, MM, Leukemia, Lymphoma, GCT, EG, Chordoma, Hemangioma, Posterior elements: Mets, OO, OB, ABC
47
Difference between PVNS and Synovial chondromatosis on MRI?
PVNS low on T1 AND T2 Synovial chondromatosis low on T1 and HIGH on T2
48
Criteria for diagnosis of post-radiation sarcoma
Histology of PRS must differ from original lesion PRS has to be within irradiated field PRS must arise \>5years after the index cancer Cancer syndromes excluded (Li Fraumeni/Rothmund Thomson)
49
Lesions from which a secondary ABC may arise
GCT Osteoblastoma Chondroblastcoma Osteosarcoma Fibrous Dysplasia Non ossifying fibroma Chondromyxoid fibroma
50
4 tumours to consider preop embolization
Renal cell mets Thyroid mets Melanoma mets Large pelvic masses
51
Tumours where XRT may be only treatment
Hemangioendothelioma Ewings (if inoperable) Lymphoma (primary lymphoma of bone) Plasmacytoma (solitary) "HELP"
52
2 things about chordoma
Stains positive for keratin S100 positive
53
Four syndromes associated with vascular malformations
Klippel Trenaunay Proteus Syndrome Mafucci Syndome Rendu-Osler-Weber
54
Hand Schuller Christian dz - classic triad?
Exophthalmos Diabetes Insipidus Multiple bony lesions (especially skull)
55
8 body systems that may be affected by Langerhans Cell Histiocytosis
Skeletal Skin Pulmonary Endocrine Hematologic Lymphatic Auditory Gastrointestinal
56
5 indications for advanced imaging of a soft tissue mass
Size \>5cm Deep to fascia Firm Non-mobile Rapid growth
57
Diagnosis of atypical femur fracture 5 major 6 minor What is needed to make dx
_5 major_ Atraumatic or low energy Transverse/Short oblique Non-comminuted Diaphyseal location (below LT) Medial spike in complete #, incomplete only lateral _6 minor_ Lateral periosteal thickening Generalized cortical thickening Prodromal sx May have bilateral symptoms Delayed fracture healing Comorbidities or implicated meds (ie bisphos) Need all major and none/some of the minor
58