Labs to order in multiple myeloma workup
- increased Cr
- ESR (increased)
- SPEP (M-spike)
- UPEP (Bence-JOnes protein)
What is a Bence-Jones protein?
Light chain immunoglobulin found in multiple myeloma
can be kappa or lambda light chain
UPEP will show monoclonal light chains
Lifetime risk of malignant transformation for solitary osteochondroma?
Most common benign tumour of childhood?
Two important differences between Maffucis and Olliers?
Maffucis has hemangiomas
Maffucis has higher risk of secondary malignant transformation (100% vs. 30%)
Which type of liposarcoma does not require adjuvant radiotherapy?
Well differentiated liposarcoma
Can you do limb salvage in osteosarcoma in a patient with a pathologic fracture?
However increased rates of local recurrence and decreased survival overall in these patients
Adult Patient with osteochondrama has acute onset of pain at the location of lesion. What do you suspect?
Treamtent for osteosclerotic myeloma
Chemo + radio+ plasmapheresis
Generally no surgery needed
(This is a form of MM associated with POEMS)
What is the multidrug resistance gene (MDR)
Pumps chemo out of cancer cells
Present in 25% of primaries
50% of mets
Very poor prognostic indicator
What primary is very vascular, requiring embolization of lesions primary to ORIF?
What does GCT look like on MRI?
Dark on T1 and T2
List 4 types of surgical resection in terms of margins.
- intralesional (you cut into the tumor)
- marginal (you resect directly at the interface between tumor and normal tissue)
- wide (you resect the tumor with a cuff of normal tissue surrounding the tumor)
- radical (you resect the entire compartment from which the tumor arose)
Ddx for lytic lesion in greater than 40 year old
4. Metabolic (browns Tumor)
General treatment of soft-tissue sarcoma
En-bloc resection + adjuvant radiation
Periosteal formation of new bone
Looks like dripping candle wax
Benign but painful
Symptomatic treatment ± excision
Name these translocations!!
1. Alveolar rhabdomyosarcoma; t(2;13)
2. Ewings Sarcoma; t(11;22)
4. Synovial sarcoma; t(X;18)
5. Myxoid liposarcoma; t(12:16)
Who gets Ewing's sarcoma?
White males aged 10-20
What blood markers can be used to measure bone turnover?
What is stone man's disease?
Fibrodysplasia ossificans progressiva
- Progressive and EXTENSIVE heterotopic ossification (muscles, fascia, tendons, ligaments, joint capsules)
- Congenital malformation of the great toe
Mutation in ACVR1 gene (activin A type I receptor)
Enchondromatosis is characterized by:
A) multiple enchondromas, hemangiomas, lymphangiomas
B) multiple enchondromas in a unilateral distribution
C) auto-dominant transmission, multiple enchondromas and osteochondromas
Poor prognostic indicators in Multiple myeloma (9)
Renal failure (worst)
Type (plasmacytoma has best prognosis)
chromosome 13 deletion or translocation (t4;14), 4(14;16)
circulating plasma cells
increased beta 2 microgloblulin (indicates elevated tumor burden)
decreased serum albumin
increased marrow microvessels
Surveillance of soft-tissue & osteosarcoma?
(They are the same)
Physical exam, CXR, CT Chest
1-2 years: 3 months
3-5 years: 6 months
5-10 years: yearly
Common locations of ABC?
Femur > tibia > spine
What part of an osteochondroma is usually the site for secondary malignant transformation?
Name the tumours associated with NF1
Optic glioma - part of dx criteria
pancreatic endocrine tumors
Options for fixation of a met? (Be general)
1. ORIF Plate with cement versus nail
Describe FU for solitary enchondroma with no concerning features:
q3-6 months for 1-2 years, then annually
What is the incidence of post radiation sarcoma in an area previously radiated to treat malignancy?
(more common if also had chemo)
When do osteochondromas stop growing?
At skeletal maturity