Tumor Flashcards
Where does chondromyxoid fibroma arise?
Diaphysis
If diameter of lesion is smaller than diameter of growth plate with respect to cysts, which is it ubc or abc?
Ubc
Osteochondroma pedunculated
Surface lesion, ct shows corticomedullary continuity
Apophyseal lesion
Chondroblastoma, gct
Intraarticular lesion?
Synovial chondromatosis Pvns
Fibrous dysplasia
Smudged out trabeculae
Surface osteochondroma?
Exists, neocortex, thickened sclerotic border
Sclerotic lesion stuck on to distal femur?
Parosteal sarcoma - low grade
Mirel’s Score?
>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
Inheritance pattern of multiple hereditary exostoses?
AD
So 50% chance of passing it on
Parent 1: Xx Parent 2: xx
Kids: Xx, Xx, xx or xx
Ddx for small round blue cell tumours in kids
Ewings/PNET
Rhabdomyosarcoma
Lymphoma
Neuroblastoma
Wilm’s tumour
Serum and urinary studies for Paget’s disease
Increased ALP
Increased urinary hydroxyproline, n-telopeptide, alpha c-telopeptide and deoxypyridoline
Normal calcium!
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
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)
Most common tumours by location!
For hand and foot, list most common benign and sarcomatous malignant tumours of ST and bone
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:
List 3 soft tissue sarcomas where chemotherapy may be part of the treatment plan
Rhabdomyosarcoma (in kids)
Synovial sarcoma
Soft tissue Ewing’s
Soft tissue osteosarc
Dedifferentiated chondrosarc
Mesenchymal chondrosarc
Quoted survival rates of bone sarcomas
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%
Risk factors for worse prognosis in chondrosarcoma
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)
Risk factors for development of secondary chondrosarcoma
(hint, think clinical, radiographic and genetic
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
Regarding blastic-appearing metastatic lesions
Lung 30% blastic
Breast 60% blastic
Prostate 90% blastic
Epiphyseal lesions
Chondroblastoma (age <25)
GCT (age 30s-50s)
Clear cell chondrosarcoma
Infection (osteomyelitis)
Soft tissue “tumours” that may have calcifications.
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
Stains positive for S100
EG
Clear cell
Chordoma
Stains for CD1A
EG
Ewing’s stains for what?
CD99
What is Jaffe Campanacci syndrome?
Associated with:
Multiple NOF
Cafe Au Lait spots
Mental retardation
POEMS syndrome?
Polyneuropathy
Organomegaly
Endocrinopathy
M protein (multiple myeloma)
Skin changes (hyperpigmentation)
Mazabraud syndrome?
Polyostotic fibrous dysplasia
Intramuscular myxomas
McCune Albright?
Polyostotic FD (usually unilateral)
Precocious puberty
Cafe au lait (coast of Maine)
also renal phosphate wasting from high expression of FGF23
Orthopaedic features of Gardner syndrome (aka Familial adenomatous polyposis)
Desmoid tumours
Osteomas
Adjuvants for treatment of GCT after curettage
Cement
H202
Liquid nitrogen
Phenol
Ethanol
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)
How to differentiate chordoma from chondrosarcoma?
Chordoma stains positive for keratin.
Other metastatic tumour antigens?
CEA for colorectal
CA 19-9 for pancreatic
CA 125 for ovarian
CA 15-3 for breast
AFP for hepatocellular
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)
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
Bone tumours (benign) with pulmonary mets?
Chondroblastoma (<1%)
GCT (3-5%)
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
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
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)
Complications of bisphosphonates?
Nausea/Vomiting
Myalgias
AVN jaw (not kids)
GERD/Gastritis
Acute renal failure
Uveitis
Hypocalcemia/hypophosphatemia
Symptoms of malignant hypercalcemia
Confusion/Agitation
Weakness
Nausea/anorexia
Polyuria
Dehydration
Short QT, bradycardia, 1st degree AV block
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)
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
List 5 sequelae of osteopetrosis
- Cranial nerve palsies (overgrowth of skull base foramina. Includes HEARING LOSS)
- Osteomyelitis (poor marrow vascularity and WBC function)
- Long bone #s
- Coxa vara (from repetitive stress #s)
- Anemia (bone marrow encroachment)
- Renal tubular acidosis (loss of carbonic anhydrase)
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)
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)
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
Difference between PVNS and Synovial chondromatosis on MRI?
PVNS low on T1 AND T2
Synovial chondromatosis low on T1 and HIGH on T2
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)
Lesions from which a secondary ABC may arise
GCT
Osteoblastoma
Chondroblastcoma
Osteosarcoma
Fibrous Dysplasia
Non ossifying fibroma
Chondromyxoid fibroma
4 tumours to consider preop embolization
Renal cell mets
Thyroid mets
Melanoma mets
Large pelvic masses
Tumours where XRT may be only treatment
Hemangioendothelioma
Ewings (if inoperable)
Lymphoma (primary lymphoma of bone)
Plasmacytoma (solitary)
“HELP”
2 things about chordoma
Stains positive for keratin
S100 positive
Four syndromes associated with vascular malformations
Klippel Trenaunay
Proteus Syndrome
Mafucci Syndome
Rendu-Osler-Weber
Hand Schuller Christian dz - classic triad?
Exophthalmos
Diabetes Insipidus
Multiple bony lesions (especially skull)
8 body systems that may be affected by Langerhans Cell Histiocytosis
Skeletal
Skin
Pulmonary
Endocrine
Hematologic
Lymphatic
Auditory
Gastrointestinal
5 indications for advanced imaging of a soft tissue mass
Size >5cm
Deep to fascia
Firm
Non-mobile
Rapid growth
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
