Diagnosis and Management of Bone and Soft Tissue Tumors

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Diagnosis and Management of Bone and Soft Tissue
Tumors

• Henry DeGroot III, M.D.
• TUSM Core Lecture 2008

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Outline of Presentation

• Definitions and Basic Concepts
• Epidemiology, staging systems, growth patterns
• Common bone and soft tissue tumors
• Management strategy
• Bone, soft tissue, metastatic deposits in bone
• Delay in diagnosis
• Implications, causes, avoidance
• Treatment
• Prognosis
• World wide web resources

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Definitions and Basic Concepts
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Definition of Bone Tumor

• Hamartomas
• Reactive processes
• Post traumatic lesions
• Inflammatory lesions
• True neoplasms
• Benign
• Malignant

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Sarcoma A malignancy of connective tissue
derived from the mesoderm, muscle, tendon,
bone Tumor a growth or swelling Adjuvant
therapy treatments for sarcoma in addition to
surgery chemo, radiation given after
surgery Neoadjuvant therapy treatments given
for sarcoma before surgery chemo XRT Survival
Multiple definitions average 5-year
disease-free 10 year overall survival Margins
and resections are defined by the the plane of
dissection relative to the tumor and its reactive
zone
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Margins
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• Definition of types of resections
• Intralesional
• marginal
• wide
• radical

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Describing a margin an operative note Useful The
tumor was adjacent to (structures). The tumor
was removed with a _____ margin. Meaningless
we got around it OK en bloc clean
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Epidemiology, Grading SystemsGrowth Patterns
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Epidemiology Bone Sarcomas

• approx. 2700 primary malignant bone tumors / year
  in US (1 / 5 of adult/pedi cancers)
• benign bone tumors are at least 50X more common
• metastatic lesions in bone are 100X more common

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Epidemiology

• Bone tumors often have a strong affinity for
  certain age range
• Osteosarcoma
• 15 - 25
• Giant cell tumor
• 25 - 40
• Why?

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Epidemiology - Soft Tissue Sarcomas

• Incidence is 1.4 / 100,000 rises to 8 / 100,000
  in patients over 80
• 15 occur lt age 15, 40 gt55
• Most common in the large muscle groups of the
  extremities
• Benign soft tissue tumors 300 / 100,000 (gt100
  / 1)
• 9000 cases/ year USA

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Etiology and Pathogenesis

• Genetics
• Multiple DNA abnormalities have been identified
  - exact role unclear
• Deletion of the p53 and Rb tumor suppressor gene
  in OSA

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Genetic Markers in Tumors

• Myxoid Liposarc t(1216)
• Ewings Sarc t(1122)
• Synovial Sarc t(x18)
• Myxoid chondrosarc t(922)
• Rhabdomyosarc t(113) or t(213)

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Sarcoma Growth Patterns
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Bone Tumor Growth Patterns

• Latent pattern
• No growth or very slow growth
• Usually a benign lesion
• May require no treatment
• Active pattern
• Progressive growth
• Usually a benign but locally destructive lesion
  or low grade cancer
• Requires biopsy and removal
• Aggressive pattern
• Rapid, destructive growth
• Usually a high grade cancer or a metastatic
  lesion
• Requires complete cancer workup and multimodal
  treatment

GOOD
BAD
UGLY
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Latent

• Geographic destruction, narrow zone of
  transition, sclerotic rim

limited workup, may be observed
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Active

• Geographic destruction, narrow zone of
  transition, no sclerotic rim, minimal periosteal
  reaction, cortex expanded/thinned/locally absent

Complete work-up, will need biopsy and surgery
complete work-up, biopsy
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Aggressive

• Moth-eaten or permeative destruction, wide zone
  of transition, marked periosteal reaction,
  extensive cortical destruction, soft tissue mass

Full local and systemic work-up, biopsy last,
multimodal treatment
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Pathological Classification and Staging

• Classification
• By the tissue they are thought to resemble
• Surgical staging system for malignant tumors
• Enneking staging system bone
• AJCC staging system soft tissue

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AJCC Staging
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Enneking / MSTS Staging
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Common Bone and Soft Tissue Tumors
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Common Tumors

• Children soft tissue
• Children soft tis malignant
• Adult Soft tissue
• Adult soft tissue - malignant
• Most common b9 bone tumor
• Most common bone sarcoma
• Most common cancer in bone
• Most cm cancer arising in bone
• Most cm leg, ankle, foot sarc
• Most cm 4arm, wrist, hand sarc

• Hemangioma
• Rhabdomyosarcoma
• Lipoma
• MFH
• Osteochondroma
• Osteosarcoma
• Metastatic cancer
• Multiple myeloma
• Synovial sarcoma
• Epithelioid sarcoma

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SmallRoundBlueCellTumors

• A child with a SRBCT has
• O
• N
• E
• Long
• Long
• Road

• Osteomyelitis
• Neuroblastoma/PNET
• Ewing sarcoma
• Lymphoma
• Leukemia
• rhabdomyosarcoma

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 AGE DISTRIBUTION OF VARIOUS BONE TUMORS
 0 to 5 yr   LeukemiaMetastatic
neuroblastomaMetastatic rhabdomyosarcoma 10-25
yr   Osteosarcoma Ewing tumor Leukemia Lymphoma
 Eosinophilic granuloma  Enchondroma Fibrous
dysplasia Giant Cell Tumor Fibrous cortical defect
40-80 yr   Metastatic bone disease Myeloma Lymp
homa Chondrosarcoma Malignant fibrous
histiocytoma Paget sarcoma Postradiation
sarcoma  Paget disease Enchondroma Chordoma
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Bone Tumor Mimics / Tumor-Like Conditions
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Management Strategy
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If the diagnosis is certain the surgical
procedure follows from it.If the diagnosis is
uncertain then a biopsy is performed to make the
diagnosis
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Management Strategy Suspected Bone Tumors

• First order screen
• Tumor oriented history and physical
• Precise history of the pain
• Screening studies and labs (CBC/ diff, ESR, IPEP)
• Plain radiographs of the part
• Differential
• Latent, Active, or Aggressive?
• Consultation
• Mail films for review
• Referral

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Management Strategy - Bone Tumors

• Second order screen
• Bone scan / PET scan
• CT or MRI of the part
• If malignancy is suspected CXR / Chest CT
• If patient gt40 screen for metastasis

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Evaluation of a Patient With a Suspected
Metastasis - Primary Care Provider

• Complete history
• Careful search for prior cancer history, risk
  factors, usual suspects breast,lung,prostate,
  thyroid, kidney
• Careful examination
• Examine the whole area not just the nearby joint
• Remember about referred pain to the knee, low
  back or radicular pain as a sign of a pelvic or
  sacral tumor

• Henry DeGroot - Metastatic Bone Disease

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Soft Tissue Tumors - Challenges

• Overlap in clinical presentation of benign and
  malignant tumors
• Rate of misdiagnosis and mistreatment is high
• In review of 36 sports tumors - initially
  diagnosed as a sports injuries
• 70 of malignant tumors had had inappropriate
  arthroscopy or arthrography
• common diagnoses are ganglion, hematoma, lipoma,
  or bursitis

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Illustrative Case
75 year old male with bursitis - 3 drainage
procedures over several months - matter sent to
pathology from last attempt
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Diagnosis high grade MFH, w/u showed no other
site of disease Treatment Wide excision / STSG
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Evaluation of Soft Tissue Tumors History

• Benign tumors and musculoskeletal conditions
• Have been tender all along
• Cause locking, popping, and and/or effusion IN a
  joint
• The joint hurt to fully bend or straighten at
  first, but the motion is improving now
• Related to trauma, overuse, or osteoarthritis

• Malignant tumors
• Started out painless
• Cause a mass near the joint but not IN a joint
• The joint was fine in the beginning, but now it
  is a little bit stiff
• May be associated with radiation, burn scars,
  lymphedema

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Evaluation of Soft Tissue Tumors - Exam

• Malignant tumors are
• Bigger than 5 cm
• Grow and persist
• Not near the joint line
• Painless
• Deep to fascia (1/3 are SQ)
• Palpable as a definite mass
• Firm
• Fixed
• Do not transilluminate

• Benign tumors are
• Small
• Grow and shrink
• Near the joint line
• Painful
• Superficial
• Not a discrete mass
• Fleshy and soft
• Mobile
• Transilluminate

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Management of Soft Tissue Tumors

• Small (3 cm if superficial, 5 cm if deep), has
  benign exam
• Observe, document , recheck 6 weeks, ask the
  patient to report any growth, document findings
  and plan in chart
• Still small? Re-check in 3 months
• Big, or has features of possible malignancy
• Plain radiograph of the area (soft tissue
  technique)
• MRI of the lesion - send the MRI (films) to and
  orthopedic oncologist
• Ask the radiologist to image entire lesion in all
  sequences
• Avoid drive-thru MRI units
• Referral for exam / possible work up

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Metastatic Deposits in Bone
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Common Mets to Bone

• B
• L
• T
• P
• R

• Breast
• Lung
• Thyroid
• Prostate
• Renal

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• Breast
• Lung
• Prostate
• Kidney
• Thyroid
• GI

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Metastases are common

• Bone metastasis in the first presentation of
  carcinoma in 23 of patients (Conroy)
• An estimated 2 -2.5 million patients with
  metastatic cancer are alive today, more than half
  a million women with metastatic breast cancer

• Henry DeGroot - Metastatic Bone Disease

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Metastatic deposits in bone

• Localize in areas where red marrow persists
  (blood flow)
• Spine, pelvis, proximal long bones, ribs, skull
• Acrometastasis is often from lung cancer
• Batsons plexus is the connection
• Seed and soil factors

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Cancer --- Pain

• Early - insidious, activity related, history of
  minor trauma
• Mid - no longer relieved by rest, bothersome at
  night
• Late - Severe pain, narcotics necessary,
  pathological fracture

• Henry DeGroot - Metastatic Bone Disease

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Other Clinical Manifestations

• Burning pain, numbness, loss of continence,
  weakness
• Radicular pain in the thorax or abdomen is a
  harbinger of spinal cord compression from
  metastatic disease
• Pain from hip area may be referred to the knee
• Lumbar and pelvic tumors may mimic sciatica
  pain and lead to a false diagnosis of HNP

• Henry DeGroot - Metastatic Bone Disease

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Evaluation of a skeletal metastasis of unknown
origin

• A complete and thorough workup as noted above has
  been completed and no primary site is evident
• Less than 3 of patients with skeletal metastasis
  fall in this group
• Remember to think about a primary sarcoma of bone

• Henry DeGroot - Metastatic Bone Disease

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Work Up of a Suspected Metastasis of Unknown
Primary

• Careful history and physical
• 2 plain orthogonal radiographs
• T-99 bone scan
• Chest, abdomen, pelvis CT-oralIV contrast
• Screening labs-CBC, ESR, CHEM27 (Ca, AØ,
  SPEP/UPEP, PSA, U/A

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Evaluation of a skeletal metastasis of unknown
origin

• most likely site is lung or kidney
• Workup consists of CT of chest, abdomen, pelvis -
  may lead to correct diagnosis in 85
• Simon and Bartucci Cancer, 58, 1088-1095, 1986

• Henry DeGroot - Metastatic Bone Disease

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Biopsy is Not a No-Brainer!

• Henry DeGroot - Metastatic Bone Disease

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Hazards of Biopsy

• Biopsy has many potential complications
• Nondiagnostic material
• Errors in interpretation of histological material
• Contamination of uninvolved compartments or
  spread of tumor in hematoma

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Whats the Frozen Section For?
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Delay in Diagnosis
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Diagnostic Delay - Case Example

• 65 y/o man with months of progressive shoulder
  pain

• The back of the shoulder was not examined
  -several shots given for tendonitis of shoulder
  over 6 month period

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Illustrative Case Example

• Large destructive mass in scapula noted when pain
  persisted

• Diagnosis renal cell carcinoma with single focus
  of bony metastasis
• Curative surgery possible

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Legal Implications of Diagnostic Delay

• 338 negligence cases for delay in diagnosis of
  cancer
• Number one cause is breast (35)
• 15 years younger than mean for that diagnosis
• Delay 1 -3 months - 65 chance
• Delay gt 6 months - 30 chance

• Henry DeGroot - Metastatic Bone Disease

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Causes of Diagnostic Delay - the Fairy Tale and
the Pigeon Hole

• The patients history sounds like the typical
  story of a minor injury or sprain - (the fairy
  tale)
• Doctor, my hip is all swelled up but I think I
  bumped it against the dining room table
• I think I pulled it two weeks ago when I was
  shoveling snow

• Henry DeGroot - Metastatic Bone Disease

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Causes of Diagnostic Delay - The Fairy Tale and
the Pigeon Hole

• The doctor buys the story at face value and is
  lulled into making the diagnosis of a sprain,
  strain, muscle pull, contusion, hematoma, etc.
  - (the pigeon hole)
• The patient accepts the explanation and goes home
  to wait for a few months for the swelling to go
  away and the pain to get better, and then
  reappears in the emergency room with a
  pathological fracture

• Henry DeGroot - Metastatic Bone Disease

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Denial is not just a River in Egypt
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Another Way to Get Into Hot Water with Soft
Tissue Tumors

• Oh by the way would you look at this bump
• Physician orders an x-ray of the bump but doesnt
  check it
• Physician suggests a consultation to check the
  bump but doesnt set it up

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Look Out for the Red Flags

• Progressive growth of a soft tissue lesion
• Persistence gt 6 weeks
• The proposed diagnosis does not fully explain the
  patient's symptoms
• The expected improvement has not occurred the
  patient is worse

• Henry DeGroot - Metastatic Bone Disease

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Treatment
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Treatment --- Depends of Behavior

• Observation
• Medications
• Injections
• Percutaneous ablation
• Surgical removal alone
• Multimodal treatment
• Chemotherapy, surgery, radiation

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Observation - Medication - Injection

• Tumors that do not require surgery
• Enchondroma, non-ossifying fibroma
• Treatment with NSAI medications
• osteoid osteoma
• average 33 months required (Simon, JBJS 1992)
• Injectable Treatments (UBC)
• Cortisone, Marrow, Calcium sulfate,
  Hydroxyapatite cement

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Percutaneous Treatments

• Computed tomography-guided percutaneous removal
  of osteoid osteoma
• Location of nidus by CT
• Trephine or core reamer used to remove the nidus
• Percutaneous microwave radiothermy
• applied via needle probe to heat and kill the
  nidus (0.6 mm)

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Persistent Hematoma
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Surgical Removal

• Definition of types of resections
• Intralesional
• marginal
• wide
• radical

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Options for Reconstruction
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Upper Extremity Soft Tissue
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Limb Salvage - Indications

• The tumor can be removed with an adequate margin
• The residual limb will be worth saving
• Three strikes rule
• Neurologic integrity
• Bone structure integrity
• Vascular integrity
• Soft tissue (coverage) integrity

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Distal Femur Bone Sarcoma
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Bone - osteosarcoma
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Alternatives to amputation
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Oncological Results

• Overall prognosis is most strongly related to
• Low vs high grade (100 vs 64 5yas)
• Size lt10 cm vs gt10cm (88 vs 29 5yas)
• Negative vs positive margins ( 89 vs 58 5yas)
• Prognosis is not strongly affected by
• Chemotherapy in soft tissue sarcoma
• Limb salvage vs amputation

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Quality of life Limb Salvage vs Amputation

• Patients with amputations are more active, least
  worried about limb injury, but have the most
  impaired ambulation
• Patients with knee arthrodesis perform the most
  demanding physical and recreational activities,
  but have difficulty sitting
• All patients who survive sarcoma have a
  significant decrease in QOL measures

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Quality of life Amputation vs Limb Sparing

• Overall functional scores are higher for patients
  with limb sparing
• Both patients with amputations and with limb
  sparing report an overall diminution of QOL, but
  studies have failed to show a consistent
  difference between the two groups.
• Amputees are more likely to say they feel
  unattractive or report difficulty finding a
  partner or developing sexual relationships

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Prognosis

• Benign tumors
• latent -no morbidity
• active - local morbidity
• aggressive - potential severe local morbidity and
  death
• Malignant tumors
• Prognosis strongly related to stage, not to cell
  or tumor type

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Thank You