Tumor Flashcards

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?

A

CD99

24
Q

What is Jaffe Campanacci syndrome?

A

Associated with:

Multiple NOF

Cafe Au Lait spots

Mental retardation

25
Q

POEMS syndrome?

A

Polyneuropathy

Organomegaly

Endocrinopathy

M protein (multiple myeloma)

Skin changes (hyperpigmentation)

26
Q

Mazabraud syndrome?

A

Polyostotic fibrous dysplasia

Intramuscular myxomas

27
Q

McCune Albright?

A

Polyostotic FD (usually unilateral)

Precocious puberty

Cafe au lait (coast of Maine)

also renal phosphate wasting from high expression of FGF23

28
Q

Orthopaedic features of Gardner syndrome (aka Familial adenomatous polyposis)

A

Desmoid tumours

Osteomas

29
Q

Adjuvants for treatment of GCT after curettage

A

Cement

H202

Liquid nitrogen

Phenol

Ethanol

30
Q

Common translocations tested:

Myxoid liposarcoma

Rhabdomyosarcoma

Synovial sarcoma

Ewing’s Sarcoma

Clear cell sarcoma

Myxoid chondrosarc

A

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
Q

How to differentiate chordoma from chondrosarcoma?

A

Chordoma stains positive for keratin.

32
Q

Other metastatic tumour antigens?

A

CEA for colorectal

CA 19-9 for pancreatic

CA 125 for ovarian

CA 15-3 for breast

AFP for hepatocellular

33
Q

Genetic associations with osteosarcoma

A

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
Q

Preop rads vs postop rads for STS treatment? Dose?

A

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
Q

Bone tumours (benign) with pulmonary mets?

A

Chondroblastoma (<1%)

GCT (3-5%)

36
Q

Poor prognostic factors for osteosarcoma

A

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
Q

Poor prognostic factors for Ewing’s

A

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
Q

Oncologic use of bisphosphonates

A

Giant cell tumour

Fibrous dysplasia

Multiple myeloma

Mets

Paget’s

(don’t forget hypercalcemia of malignancy)

(non tumour? AVN and OI)

39
Q

Complications of bisphosphonates?

A

Nausea/Vomiting

Myalgias

AVN jaw (not kids)

GERD/Gastritis

Acute renal failure

Uveitis

Hypocalcemia/hypophosphatemia

40
Q

Symptoms of malignant hypercalcemia

A

Confusion/Agitation

Weakness

Nausea/anorexia

Polyuria

Dehydration

Short QT, bradycardia, 1st degree AV block

41
Q

Oncogenic osteomalacia - mechanism and give 4 tumour types that cause it

A

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
Q

Osteopetrosis:
Which cell is affected?
Underlying problem?

Describe 2 types and treatment of each.

A

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
Q

List 5 sequelae of osteopetrosis

A
  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
Q

5 causes of erlenmeyer flask deformity

A

CHONG

Craniometaphyseal dysplasia

Hemoglobinopathies (sickle, thal)

Osteopetrosis

Niemann Pick (fat storage dz)

Gaucher’s Disease (glucocerebrosidase deficiency, another fat storage dz)

45
Q

Metastatic workup!

A

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
Q

Common tumours in the spine:

Vertebral body?

Posterior elements?

Non-bony?

A

Body

Mets, MM, Leukemia, Lymphoma, GCT, EG, Chordoma, Hemangioma,

Posterior elements:

Mets, OO, OB, ABC

47
Q

Difference between PVNS and Synovial chondromatosis on MRI?

A

PVNS low on T1 AND T2

Synovial chondromatosis low on T1 and HIGH on T2

48
Q

Criteria for diagnosis of post-radiation sarcoma

A

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
Q

Lesions from which a secondary ABC may arise

A

GCT

Osteoblastoma

Chondroblastcoma

Osteosarcoma

Fibrous Dysplasia

Non ossifying fibroma

Chondromyxoid fibroma

50
Q

4 tumours to consider preop embolization

A

Renal cell mets

Thyroid mets

Melanoma mets

Large pelvic masses

51
Q

Tumours where XRT may be only treatment

A

Hemangioendothelioma

Ewings (if inoperable)

Lymphoma (primary lymphoma of bone)

Plasmacytoma (solitary)

“HELP”

52
Q

2 things about chordoma

A

Stains positive for keratin

S100 positive

53
Q

Four syndromes associated with vascular malformations

A

Klippel Trenaunay

Proteus Syndrome

Mafucci Syndome

Rendu-Osler-Weber

54
Q

Hand Schuller Christian dz - classic triad?

A

Exophthalmos

Diabetes Insipidus

Multiple bony lesions (especially skull)

55
Q

8 body systems that may be affected by Langerhans Cell Histiocytosis

A

Skeletal

Skin

Pulmonary

Endocrine

Hematologic

Lymphatic

Auditory

Gastrointestinal

56
Q

5 indications for advanced imaging of a soft tissue mass

A

Size >5cm

Deep to fascia

Firm

Non-mobile

Rapid growth

57
Q

Diagnosis of atypical femur fracture

5 major

6 minor

What is needed to make dx

A

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
Q
A