MSK Tumors Flashcards

1
Q

Which type of tumor is more malignant in nature?

A

Sarcomas.

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2
Q

Benign tumor, cartilage of origin and MC in 10-30 yrs of age?

A

Osteochondroma, Enchondroma.

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3
Q

Malignant tumor, cartilage in origin and MC in >60 yrs of age?

A

Chondrosarcoma.

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4
Q

Benign tumor, bone in origin and MC in 10-30 yrs of age?

A

Osteoid Osteoma, Osteoblastoma.

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5
Q

Malignant tumor, bone in origin and MC in 10-30 yrs of age?

A

Osteosarcoma.

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6
Q

Malignant tumor, marrow in origin and MC in 10-30 yrs of age?

A

Ewing Sarcoma.

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7
Q

Malignant tumor, marrow in origin and MC in >50 yrs of age?

A

Multiple Myeloma.

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8
Q

Benign tumor, fibrous tissue/uncertain origin and MC in 20-30 yrs of age?

A

Giant Cell Tumor.

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9
Q

Describe the invasiveness of benign tumors?

A
  • Well-defined, slerotic borders.
  • Lack of soft tissue mass.
  • Solid periosteal reaction.
  • Geographic bone destruction.
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10
Q

Describe the invasiveness of malignant tumors?

A
  • Interrupted periosteal reaction.
  • ‘Moth-eaten’ or permeative bone destruction.
  • Soft-tissue mass.
  • Wide-zone of transition.
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11
Q

What is the most common cause of destructive bone lesions in adults?

A

Bone Metastasis.

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12
Q

What is the most common site of bone metastasis?

A

The thoracic spine.

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13
Q

In general, what are the most common sites of metastasis?

A

Lung and liver; bone is 3rd.

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14
Q

What are the most common malignancies that metastasize to the bone?

A

“BLT with a KP (KP = kosher pickle).

-Breast, Lung, Thyroid, Kidney, Prostate.

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15
Q

What are other common sites of bone metastasis?

A

Bone #1 followed by Proximal Femur, Pelvis, Ribs, Shoulders.

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16
Q

What is a common cause for pathologic fractures?

A

Bone metastasis.

**Bone mets will dramatically shorten survival once present; 6-48 months (also depends on primary CA).

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17
Q

Where are most Osteoid Osteomas located (bone) and site (part of bone)?

A

Location = proximal femur > Tibia Diaphysis > spine.

Site = >50% in long bone Diaphysis.

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18
Q

Where are most Osteoblastoma’s located (bone) and site (part of bone)?

A

Location = Vertebral column > proximal humerus > hip.

Site = >35% in posterior elements of the spine.

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19
Q

Describe the lesion of an Osteoid Osteoma?

A
  1. Benign; small (1.5-2cm), discrete, PAINFUL lesion.
  2. Originates in the CORTEX of bone.
  3. Osteoid rich NIDUS in highly vascular connective tissue.
  4. MC in long bones (Proximal femur).
  5. M>F, ped population (5-25y/o).
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20
Q

Tumor that most often presents as PAIN, most commonly at NIGHT, that is constant and progressive and relieved by NSAIDs?

A

Osteoid Osteoma.

*Localized soft tissue swelling, erythema, tenderness and deformity can be present.

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21
Q

Diagnosis of Osteoid Osteoma and DDx?

A
  1. Always start w/an XR.
  2. F/U CT to determine location/size of nidus (1.5-2cm).
  3. BONE SCAN.
  4. MRI cautiously.

DDx - Stress Fx, Osteomyelitis, Ewing’s Sarcoma/Osteoblastoma.

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22
Q

Treatment and Prognosis of Osteoid Osteoma?

A

Treatment:

  • 1st line = Non-Operative, Pain mgmt (50% respond to NSAIDs).
  • Operative if fails medical mgmt.

Prognosis:

  • Untreated…pain resolves in 3 yrs, lesion in 5-7 yrs.
  • Surgical resection w/resolution of Sx in days to wks.
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23
Q

Gradual, progressive, dull or achy pain with NO relief with NSAIDs?

A

Osteoblastoma.

**Neuro Sx if spine involved; also swelling, limping, muscle atrophy.

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24
Q

Osteoid Osteoma’s “Big Brother?”

A

Osteoblastoma

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25
Q

Describe an Osteoblastoma lesion?

A
  • *Benign; locally ‘AGGRESSIVE, EXPANSILE’ w/extension into soft tissue.
  • 66% cortically based.
  • NIDUS >2cm.
  • M>F, ped pop 10-30 yrs.
  • MC in Axial Skeleton (40%); cervical spine, sacrum.
  • Also seen in long bones (metadiaphysis, distal diaphysis).
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26
Q

Diagnostic imaging used for Osteoblastoma?

A
  1. Always start with XR! 2-3 views.
  2. F/U CT to fully evaluate.
  3. MRI – interpret w/caution, compare to XR/CT.
  4. Bone Scan – ‘hot’ focal uptake.
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27
Q

Treatment and Prognosis of Osteoblastoma?

A

Treatment:

  • *SURGERY is standard of care.
  • Excision vs Curettage; depends on location.

Prognosis:

  • 80-90% cure rate.
  • 10-20% recurrence….start process over again.
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28
Q

MC BENIGN bone tumor?

A

OsteoCHONDRoma.

-35-40% of benign bone tumors, 15% of ALL bone tumors.

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29
Q

An outward overgrowth of CARTILAGE and bone in the METAPHYSEAL bone at the GROWTH PLATE?

A

Osteochondroma.

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30
Q

Causes and Incidence/prevalence of Osteochondromas?

A

Causes:
-Salter Harris Fx, surgery, radiation therapy.

Incidence:

  • Ped. (Growing) population; 10-30yrs, usu. before 20.
  • M=F.
  • MC see NEAR JOINTS (Knee - 50%, Proximal humerus).
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31
Q

A PAINLESS BUMP that continues to get “bigger;” often ASYMPTOMATIC and found incidentally?

A

Osteochondroma.

  • *Sx are dependent on location, size, affects on surrounding structures.
  • Near a tendon = pain with activity.
  • Numbness/Tingling = near a nerve.
  • Change in blood flow/limb color = near vascular bundle.
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32
Q

Imaging studies used for diagnosis of Osteochondroma?

A
  1. XR! 2-3 view.
    - -pedunculated vs sessile.
  2. CT to better characterize in some areas (pelvis, scapula).
  3. MRI only if concerned for malignancy or if symptomatic to better delineate soft tissue anatomy.
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33
Q

Treatment and prognosis of Osteochondroma?

A

Treatment:

  • Conservative, observation.
  • If Sx, consider surgical excision.

Prognosis:
- <2% recurrence rate after surgical resection.

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34
Q

A benign, INTRAMEDULLARY neoplasm of hyaline cartilage? Incidence?

A

Enchondroma.

  • MC in 20-40yrs; skeletally mature pt’s.
  • M=F.
  • MC seen in appendicular skeleton in tubular bones, such as the HANDs and FEET (50%), proximal humerus, femur.
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35
Q

What must Enchondroma be differentiated from?

A

Chondrosarcoma.

36
Q

What is the pathophysiology of Enchondroma?

A

Growth plate remnants that are not resorbed and either persist or begin to grow in the medullary canal on the metaphyseal side of the growth plate.

37
Q

Clinical presentation of Enchondroma?

A
  1. ‘Asymptomatic.’
  2. Often incidental finding.
  3. Pain from impending (or complete) pathologic fracture, esp. in hands/feet.
38
Q

Imaging studies used for diagnosis of Enchondroma?

A
  1. XR – sharply defined edges.
    - characteristic location, r/o suspicious lesions.
    - NO gross bone destruction.
    - NO periosteal reaction.
    - NO soft tissue mass.
  2. ‘CT’ and MRI to differentiate from Chondrosarcoma.
  3. Tissue Bx only obtained intra-operatively if treating a pathologic Fx and no other suspicious features.
39
Q

Treatment and prognosis of Enchondroma?

A

Treatment:

  • OBSERVATION is standard of care.
  • Surgery for Fx (hands, feet…curettage and bone graft).

Prognosis:

  • Excellent.
  • Recurrence after surgery, 2-15%; suggests malignant transformation (5% of cases).
40
Q

A common (18-23%) BENIGN tumors that is locally AGGRESSIVE with significant bone destruction and local recurrence?

A

Giant Cell Tumor.

**Occasionally mets (bone, lungs 2-4%); not common.

41
Q

Incidence of Giant Cell Tumor?

A
  • F>M.
  • 3rd decade; pt’s in 20s.
  • Usually in EPIPHYSIS of long bones.
  • Around the KNEE (>50%); then distal femur, prox tibia, prox humerus, distal radius.
42
Q

Clinical presentation of Giant Cell Tumor?

A
  1. Pain…night pain associated w/tumor expansion.
  2. Swelling.
  3. Deformity.
  4. Joint effusion.
  5. Decreased ROM around affected joint; depends on size.
  6. Antalgic gait…may indicate pain.
43
Q

Diagnostic tools for Giant Cell Tumor?

A
  1. ‘XR’:
    - occurs w/closed growth plate, about articular surface.
    - typically lucent (dark), eccentrically located w/in the bone.
    - extensive bony destruction, cortical breakthrough, soft tissue expansion.
  2. ‘CT’:
    - absence of bone and mineralization in lesion.
  3. ‘MRI’
  4. ‘Tissue Bx’ – during surgery.
    - -multinucleated giant cells…NEED to be removed.
44
Q

Treatment of Giant Cell Tumor?

A
  1. ‘Surgery’ – excision vs curettage, reconstruction/stabilization.
  2. Radiation and embolization when surgery not feasible (Pelvis).
45
Q

Prognosis of Giant Cell Tumor?

A
  • Gen. GOOD.
  • Local recurrence in 20% of cases.
  • Pulm. mets causes death in 16-25% of reported cases.
  • occasional malignant transformation to sarcoma.
46
Q

BENIGN disease of ‘abnormal bone remodeling’?

A

Paget’s Disease of Bone.

*Usually MULTIFOCAL (polyostotic) vs Monostotic (single bone).

47
Q

What is Paget’s Disease of Bone?

A

‘Excessive bone resorption’ (osteoclast overactivity) and ‘abnormal new bone formation’ (osteoblast compensation) cause ‘woven bone that is weaker’ (structurally disorganized mosaic of bone).

48
Q

What are the 3 phases of Paget’s Disease of Bone?

A
  1. Lytic – osteoclast phase, bone turnover up to 20x the normal.
  2. Mixed – osteoblasts create new bone, abnormal w/irregular deposition of collagen fibers (instead of linear).
  3. Sclerotic – bone formation is disorganized (woven) and weaker than normal; allows marrow to be infiltrated by excessive fibrous connective tissue and blood vessels; hypervascular.
49
Q

Incidence of Paget’s Disease of Bone?

A
  1. Older adults, uncommon under 55.
  2. MC in Caucasian.
  3. M=F.
  4. MC sites…axial skeleton (pelvis and spine); long bones, skull.
    - -Femur > pelvis > tibia > skull > spine.
50
Q

Clinical presentation of Paget’s Disease of Bone?

A
  1. Often ‘Asymptomatic’ (70-90%), incidental w/Fx.
  2. Bone PAIN – dull, constant, deep.
    - -sometimes 2/2 to Fx.
  3. Arthritis.
  4. Excessive WARMTH 2/2 increased vascularity.
  5. DEFORMITY – long bone bowing.
51
Q

Diagnosis of Paget’s Disease of Bone?

A
  1. ‘Elevated Alk Phos’ (BSAP - bone specific alk phos).
  2. Serum Ca++ and Phosphate levels should be normal.
  3. ‘XR’ of painful area.
    - -lytic lesion w/sharp borders, thickened cortex and irregular appearance.
  4. ‘Bone Scan’ – whole body to assess extent of Paget’s.

**DO NOT do a full skeletal survey w/XR.

52
Q

Prognosis of Paget’s Disease of Bone?

A
  1. GOOD overall.
  2. High Morbidity – pain, Fx, deformity.
    - -Increased vascularity = excessive bleeding after Fx or surgery.
53
Q

Treatment of Paget’s Disease of Bone?

A
  1. ‘Bisphosphonates’ – standard of care w/serial monitoring of markers.
    - -Alendronate (FOSOMAX) is 1st line.
  2. Ca + Vitamin D Supplementation.
  3. Calcitonin.
  4. Surgery in cases of unstable Fx and severe arthritis.
    - -THA most common, IMN fixation preferred over ORIF.
    - -HO is common complication w/surgery.
54
Q

What is a deadly complication of Paget’s Disease of bone?

A

Sarcomatous degeneration:

  • sudden increase in pain or swelling.
  • malignant; rapid and fatal.
  • 5-10% of cases w/extensive skeletal involvement.
  • Male > Female.
  • Best evaluated by MRI.
55
Q

2nd MC (25%) MALIGNANT, primary bone tumor made of cartilage?

A

Chondrosarcoma.

56
Q

What are the 2 types of Chondrosarcoma?

A
  1. Primary:
    - -Low-grade (1 and 2, 85%), High-grade (3, 15%), Dedifferentiated (most Malignant).
  2. Secondary:
    - -Arises from Benign lesions (Osteochondroma, Enchondroma, etc).
57
Q

Incidence of Chondrosarcoma?

A
  • ‘Older Adults’ (>40yrs).
  • Males > Females.
  • Predilection for LONG BONES (45%), AXIAL skeleton (pelvis 25%)…MC Hip and Pelvis.
58
Q

Clinical presentation of Chondrosarcoma?

A
  1. PAIN - deep, dull, achy pain.
  2. Slowly growing mass.
  3. Bowel/Bladder obstruction 2/2 to mass effect in pelvis.
  4. Pathologic Fx (50% of Dedifferentiated cases).
59
Q

Imaging studies used for diagnosis of Chondosarcoma?

A
  1. ‘XR’:
    - -lytic or blastic lesion, cortical changes.
    - -intralesional “POPCORN” mineralization.
    - -RINGS and ARC, multilobulated.
  2. ‘MRI’ – to assess marrow, soft tissue involvement.
  3. ‘CT’ – determine cortical involvement.
    - -endosteal ‘scalloping’ (signifies Chondrosarcoma from Enchondroma).
    - -90% cortical breach.
  4. ‘Bone Scan’ – “hot” in all grades, helps establish mets.
60
Q

Treatment of Chondrosarcoma?

A
  1. Cartilage lesions typically resistant to Chemo/radiation.
  2. Surgery:
    - -Curettage of low-grade extremity lesions.
    - -Excision for grade 2 or 3 lesions, or grade 1 in pelvis.
61
Q

Prognosis of Chondrosarcoma?

A

*Histology correlates with prognosis.

  • 5 yr survival rates:
  • -Grade 3 has a 25% recurrence rate, >30% mets.
62
Q

What is the most common type of primary malignant bone tumors in children and young adults.?

A

Osteosarcoma.

63
Q

Where does Osteosarcoma occur? Most common sites?

A

Occurs in ‘Metaphyseal Long Bones,’ where new tissue forms as CHILDREN grow.

  • Most common sites…near joints:
  • Distal Femur (40%).
  • Proximal Tibia (16%).
  • Proximal Humerus (15%).
64
Q

Where is the most common place that Osteosarcoma metastasis to?

A

Lungs…15-20% of pt’s w/mets.

65
Q

Incidence of Osteosarcoma?

A
  • PED population, MC in ages 10-25 yrs.
  • Often occurs during growth spurts in TEENAGE years.
  • Boys > Girls, AA > Caucasian.

**2nd peak in older adults >60.

66
Q

History and Physical findings of Osteosarcoma?

A
  1. MILD PAIN for wks to months; insidious onset.
  2. Bone pain.
  3. Limp.
  4. SWELLING or soft tissue mass, often near joint limiting ROM.
  5. Unexpected Fx; rare.
  6. Systemic Sx (rare) – fevers, night sweats, SOB, etc.
  7. Rarely see LAD.
67
Q

Diagnostic studies in Osteosarcoma?

A
  1. Labs mostly normal, elevated serum Alk Phos in more advanced disease.
    - -High ALP correlation to Pulm mets.
    - -High LDH correlation to poorer prognosis.
  2. ‘XR’ - 2 views.
  3. MRI to assess local spread – intraosseous extension and degree of soft tissue involvement.
    - -also helps eval the growth plate; 75-88% of metaphyseal tumors cross the growth plate into epiphysis.
  4. CT, PET, Bone scan help in staging and surgical planning.
  5. TISSUE BIOPSY!!
    - -needs to be done by an Ortho Oncologist; the one who will be doing the resection.
68
Q

Treatment and prognosis of Osteosarcoma?

A

Treatment:

  • Only cure is SURGICAL, aggressive resection and often amputation.
  • CHEMOTHERAPY before and after surgery.
  • Not responsive to radiation therapy.

Prognosis:
-5 yr survival after adequate treatment…70%.

69
Q

What is the 2nd MC primary malignant bone tumor in kids…approx. 15% of primary bone tumors?

A

Ewing’s Sarcoma.

70
Q

Where does Ewing’s Sarcoma occur? MC sites?

A
  1. Location = Usually in the DIAPHYSIS (shaft) of LONG bones and in FLAT bones (pelvis, scapula).
  2. Sites:
    - -Long bones, 55% = Femur, Tibia, Humerus.
    - -Flat bones, 40% = Pelvis, scapula, ribs.

***But it can start anywhere in the body; 85% in bones, 15% in soft tissues.

71
Q

Where does Ewing’s Sarcoma typically arise from and MC sites of mets?

A

*Arises from the medullary cavity.

  • Mets to bone marrow and lungs:
  • -BM: thrombocytopenia = petechiae, purpura.
  • -Lungs: asymmetric breath sounds, rales, etc.
72
Q

Incidence of Ewing’s Sarcoma?

A
  1. Very rare; 1 per 1 million per year.
  2. Mostly PED pop, but can affect any age.
  3. MC in 5-25 yrs.
  4. Peaks in LATE TEENAGE yrs; 65% in 2nd decade.
  5. Boys > Girls.
  6. White > AA (9x higher).
73
Q

Clinical presentation of Ewing’s Sarcoma?

A
  1. PAIN – typically, 1st symptom.
  2. “Painful bump that keeps getting bigger” – rapidly growing.
  3. Persistent pain, NIGHT PAIN, exacerbated by exercise.
  4. Localized SWELLING.
  5. Low fever.
  6. Limping.
74
Q

Imaging studies used to diagnose Ewing’s Sarcoma?

A
  1. ‘XR’ – start with a simple XR!
    - -“permeative bone destruction,” lamellated new bone formation, CODMAN triangle.
    - -mottled, moth eaten, onion skinning, raised periosteum in triangle shape.
  2. MRI to further eval bone and soft tissues.
  3. CT to eval for Mets.
  4. ‘Tissue Biopsy’
    - -by specialist ONLY.
    - -Histology has a homogenous appearance w/small, round blue cells.
75
Q

Treatment for Ewing’s Sarcoma?

A
  1. Chemo to shrink tumor.
  2. Surgery to remove.
  3. Radiation to destroy any remaining cancer cells.
76
Q

Prognosis of Ewing’s Sarcoma?

A
  1. 70% cure rate if localized disease.
  2. If diagnosed after METS or spread, survival is <30%.
    - -better prognosis if mets to lungs only.
  3. Distal tumors > central (pelvis).
77
Q

What makes up 1% of ALL malignancies and is the MC primary bone malignancy in adults?

A

Multiple Myeloma

78
Q

Neoplastic proliferation of PLASMA CELLS that presents with skeletal lesions?

A

Multiple Myeloma.

79
Q

What is the pathophysiology of Multiple Myeloma?

A

Cancer cells accumulate in bone marrow – crowd out healthy blood cells (plasma cells are made in red marrow and produce Abs/IGs) – the neoplastic cells or abnormal plasma cells produce ineffective IGs/antibodies (proteins) and decrease the production of RBCs, WBCs, Platelets.

80
Q

What are the common IGs produced by neoplastic cells of Multiple Myeloma?

A
  1. Heavy Chains - IgG (MC; monoclonal protein spike), IgA, IgM.
  2. ‘LIGHT CHAINS - Bence Jones Proteins.’
81
Q

Incidence of Multiple Myeloma?

A
  1. > 40 yrs, median age 60.
  2. M > F.
  3. MC in AAs.
82
Q

Where is Multiple Myeloma most commonly found?

A

In the ‘AXIAL SKELETON – Vertebrae.’

–ribs, skull, shoulder girdle, pelvis, long bones.

83
Q

Risk factors associated with multiply myeloma?

A
  1. Age >50, men, AA.
  2. MM often preceded by MGUS > SMM.
    - -MGUS: Monoclonal gammopathy of undetermined significance.
    - -SMM: Smoldering MM (often asymptomatic, observation until progresses to active MM).
84
Q

Clinical presentation of Multiple Myeloma?

A
  1. ‘Localized BONE PAIN’ – intermittent progressing to constant.
    - -low back pain…‘Lumbar Spine’ MC site.
  2. Pathologic Fx.
  3. FATIGUE/Weakness.
  4. Frequent infections (resp or urinary) due to weakened immune system.
  5. Spinal Cord Compression – back pain, weakness, numbness, dysesthesias in legs (painful burning or pricking).
85
Q

Imaging studies in Multiply Myeloma?

A
  1. ‘XR’ with “moth eaten” lytic lesions.
    - -only visible after >50% destruction occurs.
    - -‘Obtain Skeletal XR Survey’ … bone scans are often cold.
  2. MRI more sensitive for pelvis/spine.
86
Q

Labs used for diagnosis of Multiple Myeloma?

A
  1. CBC, CMP, ESR, CRP
    - -Neutropenia, ‘ANEMIA,’ Thrombocytopenia (depressed CBC).
    - -Elevated Creatinine, Ca++, ESR.
    - -‘SPEP with M Spike.’ – PANCE.
  2. Urine: 24 hr urine.
    - -‘PROTEINURIA’ (>1g/24hrs).
    - -‘UPEP = Bence Jones Proteins (IgG light chains).’ - PANCE.
  3. ‘Bone Marrow Aspirate and Biopsy’
    - - >10% plasma cells = MM.
  • SPEP - serum protein electrophoresis.
  • UPEP - urine protein electrophoresis.
87
Q

Treatment and prognosis of Multiple Myeloma?

A

Treatment:

  • -Incurable.
  • -Multi-agent CHEMO, +/-stem cell ‘transplant,’ +/- ‘Bisphosphonates.’
  • -‘SURGICAL STABILIZATION +RADIATION’ for symptomatic lesions and impending Fx or Spinal Cord Compression.
  • -‘Corticosteroid’ Injections for Spinal compression.

Prognosis:

  • -Median Survival…3 yrs from diagnosis.
  • -5 yr survival rate of 30%.
  • -10 yr survival rate 11%.