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115 Cards in this Set

  • Front
  • Back
  • 3rd side (hint)
  • ppx:75% of pt < 20 yrs, + 6 M incrg knee pain & PE-recently noticed mass in knee. (prox hum, prox fem, dis fem, prox tib)
  • xray/ct:Expansive, eccentric and lytic lesion with bony septae & MRI- Fluid levels & Edema in surrounding tissue
  • risk factors-hx of prior radiation
  • >histo=high grade sarcoma with mitotic figures with intervening cellular areas & lakes of blood mixed with malignant cells

2.What is the most likely dx,


-DDX & MC compliation?


1.-dx=Telangietic Osteosarcoma


-DDx-ABC:Aneurysmal Bone Cyst


mcc=pathologic fx increased risk of recurrence

    • Gentics mutations-oncogenes: c-fos, c-myc, HER2/neu
      • tumor suppressor genes-Rb-1, p53,
  • px:75% of pt < 20 yrs, + 6 M incrg knee pain & PE-recently noticed mass in knee. (prox hum, prox fem, dis fem, prox tib)
  • xray/ct:Expansive, eccentric and lytic lesion with bony septae & MRI- Fluid levels & Edema in surrounding tissue
  • risk factors-hx of prior radiation

2.What is the most appropriate first step in management & dx,


DDX ->TX?


2.multi-agent neoadjuvant chemotherapy (ex. adriamycin, cis-platinum, methotrexate, and ifosfamide) x 8-12 wks


  • surgical resection (limb-salvage /amputation),
  • additional adjuvant chemo x 6-12 Mths.
  • dx=telangiectatic osteosarcoma
  • DDX-ABC's=intralesional curettage & BG
  • px:75% of pt < 20 yrs, + 6 M incrg knee pain & PE-recently noticed mass in knee. (prox hum, prox fem, dis fem, prox tib)
  • xray/ct:Expansive, eccentric and lytic lesion with bony septae & MRI- Fluid levels & Edema in surrounding tissue
  • risk factors-hx of prior radiation

3.most important predictor of good outcome DX?


& poor outcome,



  • 98% necrosis with chemo = good prognostic sign,
  • dx=telangiectatic osteosarcoma
  • poor=advanced tumor stage is most important indicator
  • increased lactate dehydrogenase (LDH) and alkaline phosphatase (ALP)
  • expression of multi-drug resistance (MDR) gene
dx DDX
tx
3 MCC
  1. -dx & DDX
  2. -tx
  3. -MCComplic
dx DDX
tx
3 MCC
  1. -dx=Telangietic Osteosarcoma


    -ddx-ABC:Aneurysmal Bone

  2. -multi-agent neoadjuvant chemotherapy, surgical resection, additional adjuvant chemotherapy for 6-12 months.
  3. -increased risk of recurrence with displaced, pathologic fx
multi-agent neoadjuvant chemotherapy, surgical resection, additional adjuvant chemotherapy for 6-12 months.
increased risk of recurrence with displaced, pathologic fx
LERNM-Lymphoma middle age, cd45, Ewings young ad, 11:22CD99; Rhabdo-kids desmin;...
  1. increased risk of recurrence with displaced, pathologic fx
  • px:20-50 yo f/male c/o thumb swelling and pain x6 M. He denies a hx of trauma or previous problems with the thumb.
  • xray/ct:benign-appearing, well-defined in diaphysis; calcified “popcorn stippled” no endosteal disruption or erosion.
  • (risk factors-calcified phleboliths)

  1. next best diagnostic study to confirm dx, (looking for) & DDx, MC comorbidity causing complication?
  2. if no pain#2 diagnostic study Dx?

1 skeletal survey r/o Ollier's dz (multiple enchondromatosis) & Maffucci's syndrm


ddx=Bone infarct, Low grade chondrosarcoma


  • angiomas=Maffucci's syndrome

2.Bone scan r/o chondrosarcoma , enchondroma & dx polyostotic dz


  • px:20-50 yo f/male c/o thumb swelling and pain x6 M. He denies a hx of trauma or previous problems with the thumb.
  • xray/ct:benign-appearing, well-defined in diaphysis; calcified “popcorn stippled” no endosteal disruption or erosion.
  • (risk factors-calcified phleboliths)
  • dx,

1What is the most appropriate first step in management & DDX & TX?


dx-enchondroma


-if NO Pain, tx=observance & repeat radiographs in 3-6 months


-if PAIN, tx= Curretage and bone grafting



ddx-bone infact tx-observ, Low grade chondrosarcoma tx- wide excision ray resection or partial hand amputation.


  • px:20-50 yo f/male c/o thumb swelling and pain x6 M. He denies a hx of trauma or previous problems with the thumb.
  • xray/ct:benign-appearing, well-defined in diaphysis; calcified “popcorn stippled” no endosteal disruption or erosion.
  • (risk factors-calcified phleboliths)

poor outcome,


-DX?



poor-multiple enchondromas and soft-tissue angiomas ; risk of malignant transformation up to 100%


-enchondroma


dx DDX
tx
3 MCComplic
mn vertebra plana
  1. -dx & DDX
  2. -tx
  3. 3 MCComplic
dx DDX
tx
3 MCComplic
mn vertebra plana
  1. dx-enchondroma
  2. ddx-CSA. bone infact
  3. Malignant transformation ollier's <30% muffucci-100%
dx-enchondroma
ddx-CSA. bone infact
Malignant transformation ollier's
  • -10-20 yo, painful 2^to irritation of tendons
  • XR/CT:well-demarcated, shallow cortical defect
  • punctate mineralization (calcification)
  • histo bland hyaline cartilage, small chondroid cells in lacunar spaces

  1. dx
  2. DDx
  3. What is the most appropriate treatment for this tumor?

1dx=Periosteal chondromas


  • -ddx-chondrosarcoma
  • -tx=Marginal excision including the underlying cortex
dx DDX
tx
3 MCComplic
mn-
  1. dx
  2. tx
  1. Periosteal chondromas

  2. Marginal excision including the underlying cortex

dx DDX
tx
3 MCComplic
  1. -dx
  2. -tx
  3. -MCComplic
dx DDX
tx
3 MCComplic
  1. Chondromyxoid Fibroma,-intralesional curretage and bone grafting (or PMMA


    • Recurrence
      • occurs in 25% of cases
aneurysmal bone cyst (ABC) 
chondroblastoma 
non-ossifying fibroma  
chondroblastoma 
enchondroma  
chondrosarcoma 
intralesional curretage and bone grafting (or PMMA 
occurs in 25% of cases
based on histology image what is the Dx & def?
tx
describe histo

 
  1. -based on histology image what is the Dx & def?
  2. -tx
  3. -describe histo

synovial chondromatosis=proliferative disease of the synovium +cartilage metaplasia=>multiple intra-articular loose bodies
open or arthroscopic synovectomy and loose body resection -severe symptoms affecting range of motion
cartilage nodules a...
  1. -synovial chondromatosis=proliferative disease of the synovium +cartilage metaplasia=>multiple intra-articular loose bodies
  2. -open or arthroscopic synovectomy and loose body resection -severe symptoms affecting range of motion
  3. cartilage nodules are primarily visible on MRI
  4. discrete hyaline cartilage nodules in various stages of calcification and ossification
  5. -chondrocytes
    • mild atypia
    • binucleate cells
    • occasional mitoses
knee is most common location
  • knee is most common location
47-year-old man presents with right groin pain. radiograph, arthroscopy, and histology slide are shown:


dx & def
tx
MC MRI finding?

47-year-old man presents with right groin pain.


  1. -dx & def
  2. -tx
  3. -MC MRI finding?
synovial chondromatosis & benign metaplastic process which requires symptomatic treatment
open or arthroscopic synovectomy and loose body resection -severe symptoms affecting range of motion
knee-cartilage multiple intra-articular loose bodies 
  1. -synovial chondromatosis & benign metaplastic process which requires symptomatic treatment
  2. -open or arthroscopic synovectomy and loose body resection -severe symptoms affecting range of motion
  3. -knee-cartilage multiple intra-articular loose bodies

multiple intra-articular loose bodies

  • Hx: young adults 30-50 M>F, pain in 1 jnt->persists for months, or even yrs, does not ease w/exercise, steroid injection or heat tx,
  • PT => restriction of ROM
    • no weight loss
    • pain & swelling
      • pain worse with activity
    • PE:mechanical sx + stiffness
    • knee MC location
  • decreased ROM
    • + warmth, erythema, or tenderness

    1. -next best study to confirm dx
    2. -what is the next most appropriate step in Management?
    3. -outcome & tX?
  1. -if xray neg stippled calcification then 2-MRI synovial chondromatosis
  2. -arthroscopic synovectomy and loose body resection
  3. -treatment is symptomatic but may help prevent degenerative joint changes
  • occasional chromosome 6 abnormalities have been found
based on histology image what is the Dx & def?
tx?
describe histology
  1. -based on histology image what is the Dx & def?
  2. -tx?
  3. -describe histology
relatively small and uniform and found in sheets of malignant appearing cells with minimal cytoplasm and dark atypical nuclei
gland, nest, or cyst like cells
  1. -Synovial Sarcoma, Malignant soft tissue sarcoma which arises near joints, but rarely within the joint, cellular origin of synovial sarcoma is unknown, but it is not the synovial cell or any cell involved in the synovium

  2. -wide surgical resection with adjuvant radiotherapy + chemo

  3. -classical synovial sarcoma shows a biphasic appearance with two typical cell types

  4. spindle cells (fibrous type of cells)
    • relatively small and uniform and found in sheets of malignant appearing cells with minimal cytoplasm and dark atypical nuclei
  5. epithelial cells
    • gland, nest, or cyst like cells
  • -most common sarcoma found in young adults
  • most common malignant sarcoma of the foot
A 22-year-old female has a painful foot mass.  regional lymphadenopathy?


What is the most likely diagnosis & def?
tx
tumor stains pos 4? 

A 22-year-old female has a painful foot mass. regional lymphadenopathy


-XR/CT- calcification in the soft tissue mass ?


  1. -What is the most likely dx & def?
  2. tx
  3. tumor stains pos? 4
vimentin 
epithelial membrane antigen
sporadic S-100 

epithelial cells stain positive for keratin
  1. -Synovial Sarcoma, Malignant soft tissue sarcoma which arises near joints, but rarely within the joint

  2. wide surgical resection with adjuvant radiotherapy, chemotherapy may improve both local control and overall survival

  3. vimentin
  4. epithelial membrane antigen
  5. sporadic S-100

epithelial cells stain positive for keratin

  • chromosomal translocation t(X;18) is observed in more than 90% of cases

  • translocation forms the SYT-SSX1, 2, or 4 fusion protein

29-year-old male presents with deep pain & swelling of the Rt foot. He first noticed pain and swelling 1 year ago, which has been steadily increasing over this time. He denies any recent trauma, travel or systemic symptoms. XR- calcification in the soft tissue mass


dx?


  1. -next best study to confirm dx (2) DX?
  2. -what is the next most appropriate step in Management?
  3. most important predictor of good outcome vs poor?

1-Synovial Sarcoma,


-genetic/karyotyping, blood/BM chrom translocation t(X;18)


  • fusion protein=>SYT-SSX1, 2, or 4
  • Immunostaining ->
      • vimentin, epithelial membrane antigen EMA
      • sporadic S-100, keratin

      1. wide surg resection with adjuvant radiotherapy,+ chemo
      2. stage with LN Bx, pre-surgery or post-surgery->, chemotherapy
      3. poor-lung mets MC large, deep, and high grade sarcomas
      4. good-low histologic grade, no mets
  • overall prognosis is poor
    • 5 year survival is approximately 50%
    • 10 year survival is approximately 25%

mn-epiphyseal tumors

  1. chondroblastoma,
  2. giant cell,
  3. clear cell sarcoma
  • occasional chromosome 6 abnormalities have been found, DX

Synovial Chondromatosis

multiple intra-articular loose bodies 

multiple intra-articular loose bodies

def Metaplasia, in contrast to neoplasia, is a process? ex? tx?

  • where one cellular phenotype transforms into another cellular phenotype, each non-cancerous
  • examples are the synovial transformation to chondroid tissue in synovial chondromatosis
  • symptomatic treatment
-multiple intra-articular loose bodies 


-Synovial Chondromatosis

-multiple intra-articular loose bodies


-Synovial Chondromatosis

In a older adult with loss of joint space treatment of painful synovial chondromatosis of the hip is? young adult tx?


  1. arthroplasty is recommended.
  2. Arthroscopic removal of loose bodies

chromosomal translocation t(X;18) is observed in more than 90% of cases, dx?

Synovial Sarcoma

translocation forms the SYT-SSX1, 2, or 4 fusion protein, dx

Synovial Sarcoma

biphasic appearance with two typical cell types

biphasic appearance with two typical cell types

  • which sarcomas, the lung is most common site of metastasis?

Synovial Sarcoma

biphasic appearance with two typical cell types

biphasic appearance with two typical cell types

soft tissue sarcomas which can metastasize to lymph nodes, mn

RACES


  1. rhabdomyosarcoma
  2. Angiosarcoma
  3. Clear cell sarcoma
  4. epitheliod sarcoma
  5. synovial sarccoma

  • most common sarcoma found in young adults (15-40 years)
  1. synovial sarccoma
biphasic appearance with two typical cell types

biphasic appearance with two typical cell types

it is the most common malignant sarcoma of the foot

  1. synovial sarccoma
biphasic appearance with two typical cell types

biphasic appearance with two typical cell types

-based on histology image what is the Dx & def?
-tx?
-describe histology
  1. -based on histology image what is the Dx & def?
  2. -tx?
  3. -describe histology
-Malignant Peripheral Nerve Sheath Tumor,  peripheral nerve or neurofibroma aka. neurofibrosarcoma or malignant schwannoma
-wide surgical resection + radiation, in general, treated as high-grade sarcoma, chemotherapy not useful
spindle cells ...
  1. -Malignant Peripheral Nerve Sheath Tumor, peripheral nerve or neurofibroma aka. neurofibrosarcoma or malignant schwannoma
  2. -wide surgical resection + radiation, in general, treated as high-grade sarcoma, chemotherapy not useful
  3. spindle cells with wavy nuclei resembling fibrosarcoma
  4. -
positive S100 stain
keratin staining is negative
- The MRI shows the classic "target sign" on fluid sensitive imaging.
  • Immunohistochemistry
    • positive S100 stain
    • keratin staining is negative
    • - The MRI shows the classic "target sign" on fluid sensitive imaging.
soft-tissue mass
most arise from large nerves (sciatic, brachial plexus)
motor and sensory deficit of the affected nerve
-A 45-year-old man presents for evaluation of 6 months of radicular buttock pain.
-next best study to confirm dx (2)
-what is ...
  • soft-tissue mass

  • most arise from large nerves (sciatic, brachial plexus)
  • motor and sensory deficit of the affected nerve
  • -A 45-year-old man presents for evaluation of 6 months of radicular buttock pain.
  • -next best study to confirm dx (2)
  • -what is the next most appropriate step in Management?
  • most important predictor of good outcome vs poor?
  1. -Immunohistochemistry
    • positive S100 stain
    • keratin staining is negative->Malignant Peripheral Nerve Sheath Tumor
  2. -wide surgical resection ( should include entire affected nerve) + radiation, treated as high-grade sarcoma, perform preoperative adjuvant radiation chemotherapy not useful
  3. -poor-most cases associated with NF-1, 30% (5-year survival)

Genetics


  • most cases associated with NF-1
  • Prst:soft-tissue mass

  • most arise from large nerves (sciatic, brachial plexus)
  • motor and sensory deficit of the affected nerve
  • -histo-spindle cells with wavy nuclei resembling fibrosarcoma
  • -serial MRI may show enlargement of previous benign nerve sheath lesion suggesting malignant transformation
  • -Bone scan, mildly positive

  1. -What is the most likely dx & def?
  2. -tx
  3. -tumor stains pos? 4
  1. Malignant Peripheral Nerve Sheath Tumor, neurofibrosarcoma or malignant schwannoma
  2. wide surgical resection + radiation, include entire affected nerve
  3. Immunohistochemistry
    • positive S100 stain
    • keratin staining is negative
 MRI shows the classic "target sign" on fluid sensitive imaging

MRI shows the classic "target sign" on fluid sensitive imaging

  1. -mn-small round blue cell
  2. -age group, factoid

LERNM


  1. -Lymphoma middle age, cd45,
  2. -Ewings young ad, 11:22CD99;
  3. -Rhabdo-kids desmin;
  4. -Neuroblastoma-infants NSB,NB84;
  5. -Myeloma (older)-SPEP/UPEP, IHC.IgG/A

MC CA that develops as a result of chronic draining wounds from osteomyelitis sinus tracts or pressure sores or burn scar?

Marjolin’s ulcers=> squamous cell carcinoma

can arise following irradiation and poly-vinyl chloride exposure., tumor in the hand often has central necrosis that is manifested with overlying skin changes.?


-most appropriate step in Management? DX?


tx?

-angiography helps determine the extent of the lesion and may show metastasis" and MR imaging is valuable for evaluating the size and extent of the lesion.


-angiosarcoma.


  • -wide surgical resection
    • indications
      • treatment of choice
      • relatively insensitive to chemotherapy and radiation
    • -amputation
      • indications
        • to achieve local control of disease
        • very aggressive tumor

specimen stains positive for CD31 endothelial marker

  • most common subungual malignancy
  • MC located dorsum of hand and forearm
  • primary risk factor is excessive exposure to ultraviolet radiation

squamous cell carcinoma

classic clinical presentation is the triad of fingertip pain, tenderness, and sensitivity to the cold

Glomus Tumor

  • clinical presentation is the triad of fingertip pain, tenderness, and sensitivity to the cold
  • Physical exam
    • small bluish nodule
    • often difficult to see, especially in the subungual location
    • nail ridging or discoloration is common

Glomus Tumor

  • a syndrome characterized by multiple intramuscular myxomas associated with monostotic or polyostotic fibrous dysplasia

Mazabraud's syndrome

  • MRI
  • homogeneous appearance
  • bright T2 signal
  • dark T1 signal
  • intramuscular location of _____ is important to differentiate from ______ which occurs in anintermuscular location

myxomas


myxoid liposarcoma,

1) intramuscular location, 2) relatively homogenous appearance on MRI,


3) bright T2/dark T1 signal - suggesting a myxoid tumor,


4) bland, hypo-cellular myxoid appearing histology that does not show cellular atypia in the relatively homogenous cells without nuclear atypia, mitotic figures, or high nuclear to cytoplasmic ratio.


  1. dx
  2. tx
  3. syndrome characterized by multiple intramuscular myxomas associated with monostotic or polyostotic fibrous dysplasia
  4. intermuscular location important to differentiate from a ____
  1. -Intramuscular Myxomas
  2. -. local surgical excision is the treatment of choice and recurrence is extremely rare observation indications
    • for asymptomatic lesions
  3. -Mazabraud's syndrome
  4. myxoid liposarcomas

Several methods exist to predict the risk of pathologic fracture. These include (3)


  1. -the presence of significant functional pain
  2. > 50% destruction of cortical bone
  3. formal staging systems
    • Harington's criteria
    • Mirel's criteria

Harington's criteria vs Mirels criteria compare contrast


- in Mirels criteria
score___suggests prophylactic fixation

Harington's criteria


  • > 50% destruction of diaphyseal cortices
  • > 50-75% destruction of metaphysis (> 2.5 cm)
  • Permeative destruction of the subtrochanteric femoral region
  • Persistent pain following irradiation
  • Mirels criteria
    score > 8 suggests prophylactic fixation


    Score


    -Site


    -Pain


    -Lesion


    -Size


  • following surgery refer the patient to radiation oncology for post-operative radiotherapy treatment to
    • (3)
  1. -decrease pain
  2. -slow progression
  3. -treat remaining tumor burden not removed at surgery

In terms of life expectency,___& ___have the worst median survival (<6 months) and 5 year survival (<5%) when bone metastases are present.

lung cancer and melanoma

if the diagnosis of malignancy or aggressive tumor is favored, or a soft-tissue mass greater than 5cm in diameter is present, ____prior to biopsy.

that these should be referred to an orthopedic oncologist

clinician must be able to classify the patient as having (4)

  1. - non-progressive primary benign bone tumor
  2. -progressive primary benign bone tumor
  3. -primary malignant bone tumor,
  4. -metastatic bone tumor.

If the clinical and radiographic information favors a diagnosis of malignant or aggressive benign bone tumor, then___.

-then clinician should refer the patient to an experienced orthopaedic oncologist without performing additional diagnostic tests or a biopsy. If a soft-tissue mass is 5 cm in diameter or larger on physical examination, and especially if it is deep to the fascia, the patient should also be referred to an orthopaedic oncologist, without additional evaluation or biopsy, because of the relatively high probability that the mass is malignant.


If a soft-tissue mass is 5 cm in diameter or larger on physical examination, and especially if it is deep to the fascia, then bx yes no? ____, why?

- patient should also be referred to an orthopaedic oncologist, without additional evaluation or biopsy,


-because of the relatively high probability that the mass is malignant.

diagnostic strategy was simple and highly successful for the identification of the site of an occult malignant tumor before biopsy in patients who had skeletal metastases of unknown origin. tx plan? 7 steps

-1:medical history & physical examination;=occult primary site of the malignant tumor in three patients (8 per cent), breast, bladder, kidney


-2:routine laboratory analysis;


-3:plain xr of the involved bone


4;XR chest-carcinoma of the lung (43 %).


-5: whole-body technetium-99m-phosphonate bone scintigraphy;


-6:CT-C-15%/A/P-13% K/L/Colon


-7:biopsy of the most accessible osseous lesion-8%

On the basis of the biopsy alone, we were unable to identify the primary site of the malignant tumor in ___of the patients.

(65 per cent)

-diagnostic strategy was simple and highly successful for the identification of the site of an occult malignant tumor before biopsy in patients who had skeletal metastases of unknown origin


-what % success?

(85 per cent)

the___ is the most common site for all boney metastasis,

spine

the _____ is the most common site for pathologic fracture secondary to metastasis to bone.

proximal femur

A 43-year-old women presents with a long-standing history of pain at the tip of her right non-dominant middle finger. Pain is exacerbated by cold weather. She denies systemic symptoms or preceding trauma. She has seen a number of physicians but no...

A 43-year-old women presents with a long-standing history of pain at the tip of her right non-dominant middle finger. Pain is exacerbated by cold weather. She denies systemic symptoms or preceding trauma. She has seen a number of physicians but no formal diagnosis as been made. Physical examination reveals no distinct mass or color change. Pain is reproduced by palpating over the ulnar nail bed of her distal middle finger.


-xray=>pressure erosion of the underlying bone and an associated deformity of the bone cortex


  1. -next best study to confirm dx (2)
  2. -what is the next most appropriate step in Management?
  3. most important predictor of good outcome vs poor?
- MRI images, respectively. There is a well-defined nodular lesion in the distal middle finger. The small lesion (usually <7mm) is mildly hyperintense on proton density-weighted image and markedly hyperintense on T2-weighted scans.
  1. -Glomus Tumor-
  2. MRI images, respectively. There is a well-defined nodular lesion in the distal middle finger. The small lesion (usually <7mm) is mildly hyperintense on proton density-weighted image and markedly hyperintense on T2-weighted scans.
triad of fingertip pain, tenderness, and sensitivity to the cold. 

triad of fingertip pain, tenderness, and sensitivity to the cold.

-based on histology image what is the Dx & def?
-tx?
-describe histology
  1. -based on histology image what is the Dx & def?
  2. -tx?
  3. -describe histology
  1. dx-glomus tumor, glomus body is a perivascular temperature regulating structure frequently located at the tip of a digit or beneath the nail
  2. excision with no recurrence
  3. well-defined lesion lacking cellular atypia or mitotic activity
    • small round cells with dark nuclei
    • associated small vessels in a hyaline/myxoid stroma
  4. can show gland-like or nest structures, separated by stromal elements

triad of fingertip pain, tenderness, and sensitivity to the cold.

-based on histology image what is the Dx & def?
-tx?
-describe histology
  1. -based on histology image what is the Dx & def?
  2. -tx?
  3. -describe histology
  1. -Angiosarcoma, malignant and very aggressive tumor that derives from endothelium of blood vessels
  2. -wide range of treatments have been proposed including resection, forequarter amputation, adjunctive radiation, and chemotherapy depending on the extent, metastasis, and aggressiveness of the lesion.
  3. -vascular channels with neoplastic cells that have abundant eosinophilic cytoplasm and large nuclei with mitotic figures.
 -


overlying skin changes

-CD31 is a sensitive marker for angiosarcoma

-


  • hallmarked with
    • overlying skin changes

-CD31 is a sensitive marker for angiosarcoma

34-year-old male with an eleven-year history of hand exposure to polyvinyl chloride presents with hand pain, but no constitutional symptoms. Physical exam shows lesions involving the nail-folds of the thumb, long, and ring finger as well as several soft-tissue masses in the palm.


-histo-malignant cells associated with vascular structures


-xr- eccentric, purely lytic, metaphyseal and diaphyseal lesions, with no visible matrix mineralization


  1. -next best study to confirm dx
  2. -what is the next most appropriate step in Management?
  3. -outcome?
  1. -"angiography-determine the extent of the lesion and may show metastasis"
  2. -MR for evaluating the size and extent of the lesion.
  3. -CD31 is a sensitive marker for angiosarcoma
  4. resection, forequarter amputation, adjunctive radiation, and chemotherapy depending on the extent, metastasis, and aggressiveness of the lesion.
  5. -poor, high local failure rate and amputation is often required
  6. propensity for lymphatic spread
  7. metastases to lung is common
  • Prophylactic fixation is preferred to fixation of actual pathological fracture due to (3)
    • shorter operative time
    • decreased morbidity
    • quicker recovery

if biopsy suggests primary neoplasm of bone (like sarcoma)


-next steps 5

  1. - may benefit from neoadjuvant chemo/radiotherapy
  2. close wound
  3. refer to local sarcoma center
  4. prior to surgical stabilization,
  5. surgical treatment of primary sarcoma will contaminate entire bone with sarcoma and affect ability to perform limb-salvage surgery

if biopsy suggests primary neoplasm of bone (like sarcoma) then Radiation therapy


  • indications(

low Mirels' score

  • goals of fixation

Surgical fixation primary neoplasm of bone (like sarcoma) 3

  • maximize ability for immediate mobilization and weight-bearing
  • protect the entire bone in setting of systemic or metastatic disease
  • optimize implant choice in the context of the patient's overall prognosis
  • femur with
    • peritrochanteric lesions
      • tx?
    • femoral neck and head lesions
      • tx?
  • intramedullary nail
  • hemiarthroplasty

mn-metaphyseal tumor

  • mn MOCA-

  1. Mets, MFH,
  2. OSA,
  3. CSA,
  4. ABC

mn-diaphyseal tumor

  • A,E,I,O,U,Y+METS

  1. A-Adamantanoma,
  2. E-EG & Enchondroma
  3. I-Infection,
  4. O-OO/OB,
  5. U-Ewings Sar,
  6. Y-mYeloma, lYmphoma, fibrous dYsplasia

  • METS-PT Barnum Likes Kids Yes Yes
  • Prostate-PSA; Thyroid-us; Breast-Mamo; Lung-cxr; Kidney CT abd mYeloma-SPEP/UPEP, skeletal survey; lYmphoma-CT C/A/P
  1. mn-spine

A&P And -Anterior


My Mets Love Home Games:


-My-myeloma,


-METS-mets


-L-LCH, Langerhand histocytosis


-H-hemangiomas,


G-GCT:


:POO Ass-Post-OO, OB ABC

2 mn-tumors in sacrum

-midline-chordoma


-MAGE-


-METS, MFH


-ABC,


-GCT,


-Eccentric-

mn- STS LN mets

RACES through the Lymph


--Rhabdo,


-Angiosarcoma,


-Clear Cell Sarc,


-Epithelioid,


-Synovial

  • Prognosis of bone cancer depends on the following variables (5)
    1. -overall stage of disease, extent to which a cancer has developed by spreading. chest imaging to look for pulmonary metastases + bone scan
    2. -presence of metastasis
    3. -skip (discontinous) lesions within the same bone
    4. -histologic grade
    5. -tumor size

two systems - one for malignant lesions and one for benign lesions based on what?

  • malignant lesions are defined using Roman numerals (e.g. I, II, III)
  • Stage


    Grade


    Site (1)


    Metastasis

  • benign lesions are defined using Arabic numbers (1,2,3)
  • Stage


    Grade


    Size


    Depth


    Node


    Metastasis


    5 yr. survival

  • e.g. osteosarcoma most commonly presents as stage___

IIB

prerequisites& prior to a biopsy & purpose

purpose: is to confirm a suspected diagnosis


  • CBC, platelets, coagulation studies
  • cross-sectional imaging to evaluate local anatomy
  • treatment center performing biopsy must be capable of proper diagnosis and treatment

4 Indications for Biopsy

Indications for Biopsy


    1. A-Aggressive bone or soft tissue lesions
    2. B-soft tissue lesions Bigger > 5cm, deep to fascia, or overlying bone/neurovascular structures
    3. C-C(s)olitary bone lesions in a patient with history of Carcinoma
    4. -D-in Doubt Dx in a symptomatic pt
  • When a biopsy is NOT indicated 3
  • When a biopsy is NOTindicated
    1. -Asymptomatic latent bone lesions
    2. -Symptomatic active Bone lesions which appear entirely Benign on imaging
    3. -soft tissue lesion which are completely benign on MRI don't necessarily need a biopsy (e.g. lipoma, hemangioma)

MC Types of Biopsy frequently used for sarcoma

Core biopsy

 radiograph that shows a ill-defined lytic lesion with cortical expansion with calficifications suggest what type of process & most likely dx?? 

radiograph that shows a ill-defined lytic lesion with cortical expansion with calficifications suggest what type of process & most likely dx??

-lytic appearance with cortical expansion suggests a malignant and aggressive process.


-calficifications suggest a chondroid tumor


high grade chondrosarcoma.

A 53-year old female presents to your community hospital with right shoulder pain of 2 weeks duration, night sweats, and loss of appetite. Her past medical history is significant for hypertension only. A radiograph is performed and is shown in Fig...

A 53-year old female presents to your community hospital with right shoulder pain of 2 weeks duration, night sweats, and loss of appetite. Her past medical history is significant for hypertension only. A radiograph is performed and is shown in Figure A. What is the most appropriate next step in management.

Referral to an orthopaedic oncologist

Indications of External beam irradiation 3 with ex's

  1. -Definitive control (primary malignant bone tumors)-Ewing sarcoma/primative neuroectodermal tumor
  2. Adjuvant to surgical excision-soft tissue sarcomas-
  3. -Palliative care and impending fracture fixation-metastatic bone disease

tumors that are not radiosensitive

GI and renal tumors

  • ___ Gray: tissue will likely not heal

> 60

  • ___Gray: usually leads to uncomplicated tissue healing

< 45

  • risk factors for post radiation fracture
    • -Hx-2,
    • -Bone 3-
    • -other1-
  • -Hx-age & female Sex
  • -Bone -1 anterior femoral compartment resection
  • -bone -2 periosteal stripping
  • -bone 3- osteoporosis
  • -radiation dose

A 63-year-old female sustains a subtrochanteric femoral fracture after a fall in her home. Five years ago she underwent resection of a left thigh leiomyosarcoma with adjuvant radiotherapy. Which of the following is not a known risk factor for development of pathologic fracture post radiotherapy?


1. Female


2. Age


3. Dose of radiotherapy


4. Periosteal resection during sarcoma removal


5. Adjuvant chemotherapy

(5)chemotherapy as an adjuvant treatment has not been shown to increase the risk of post-radiation fracture.

A 50-year-old female presents to your office with worsening knee pain of 6 months duration. Radiographs are shown in Figure A and B. She is otherwise healthy and very active. She insists that something be done today to treat her symptoms. Your ini...

A 50-year-old female presents to your office with worsening knee pain of 6 months duration. Radiographs are shown in Figure A and B. She is otherwise healthy and very active. She insists that something be done today to treat her symptoms. Your initial treatment would include


1. Intra-articular cortisone injection now and referral to sarcoma center


2. Staging including chest imaging and bone scan


3. Neoadjuvant chemotherapy


4. Neoadjuvant radiotherapy


5. Limb salvage with mega-prothesis


2-hx & XR suggest a potentially malignant process in this pt


-prior to instituting any tx, she needs complete staging.


-Starting treatment prior to full oncologic workup (even if the radiographs suggest a giant cell tumor) including chest imaging to look for pulmonary metastases and a bone scan can have devastating consequences.


-If her pain is so severe that something needs to be done today, admit her for parenteral narcotics and tumor work-up. Pending her work-up, she may need chemo/radiotherapy, referral to a sarcoma center, and limb salvage surgery.

define test for Starting treatment ?

Staging= chest imaging to look for pulmonary metastases + bone scan

history and radiographs suggest a potentially malignant process ---next 2 steps in management?


-most important NOT to do?

  1. -complete staging =chest imaging to look for pulmonary metastases + bone scan
  2. -full oncologic workup/called the metastatic workup,
  3. -prior to instituting any treatment,

metastatic lesion of unknown primary workup ?

  1. CT-C/A/P=85 %
  2. blood examination and liver function tests
  3. bone scan
metastatic lesion of suspected based on hx & PE primary workup what is Dx and w/u ?


-BRCA1 or BRCA2 genes in African-American M


 

metastatic lesion of suspected based on hx & PE primary workup what is Dx and w/u ?


-BRCA1 or BRCA2 genes in African-American M


prostate-PSa

metastatic lesion of suspected based on hx & PE primary workup what is Dx and w/u ?


-BRCA genes & p53 in F

metastatic lesion of suspected based on hx & PE primary workup what is Dx and w/u ?


-BRCA genes & p53 in F

F=breast-mamograph

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-had head or neck radiation treatments in childhood

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-had head or neck radiation treatments in childhood

thyroid- U/S

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-Cancer survivors who had radiation therapy to the chest & arsenic in drinking water

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-Cancer survivors who had radiation therapy to the chest & arsenic in drinking water

lung- CXR

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-cadmium (a type of metal), some herbicides, and organic solvents, particularly trichloroethylene. & American Indians/Alaska Natives have slightly higher rat...

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?


-cadmium (a type of metal), some herbicides, and organic solvents, particularly trichloroethylene. & American Indians/Alaska Natives have slightly higher rates than do whites

kidney- CT abdomen

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ? 

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ?

myeloma-


-SPEP/UPEP


-skeletal survey

metastatic lesion of suspected based on hx & PE primary workup , what is Dx and w/u ? - weakened immune systems & infection with the Epstein-Barr virus (EBV)

lymphoma- CT-C/A/P

Chemotherapy for cancer


  • -Mechanism of action
    • -which cells are primary affected?

-induces apoptosis


-rapidly dividing cells like the lining of the


  • -gut,
  • -bone marrow,
  • -hair,
  • -skin

Chemotherapy good prognostic sign & very poor prognosis

  1. ->98% necrosis with chemotherapy is good
  2. -expression of multi-drug resistance (MDR) gene
  • Chemotherapy Integral component of treatment along with surgical resection in ?
  • NOT used in?
    • ---osteosarcoma (intramedullary and periosteal)
    • -Ewing's sarcoma/primative neuroectodermal tumor
    • - MFH malignant fibrous histiocytoma
    • -dedifferentiated chondrosarcoma
    • _NOT=chemotherapy for soft tissue sarcoma is controversial

Doxorubicin side effects


  • aka
  • -Adriamycin.
  • -dose-limiting toxicity of doxorubicin is cardiomyopathy. aka cardiac toxicity

Bleomycin side effects

Bleomycin is associated with pulmonary fibrosis.

Cyclophosphamide side effects

Cyclophosphamide is associated with myelosuppression and urotoxicity.

Bone scan is cold

Multiple myeloma
Melanoma

Unknown origin•tumor 4 ex's?

Unknown origin•


- Giant cell tumor
• -Histiocytoma•


-Adamantinoma•


-Ewing's tumor

Epithelial Glands seen on histology 3

Epithelial Glands seen on histology 3

Epithelial Glands seen on histology


• Synovial sarcoma (biphasic)
• Metastatic carcinoma
• Glomus tumor

Bimorphic histology


•3

Bimorphic histology


•3

Bimorphic histology


• Dedifferentiated chondrosarcoma
• Synovial sarcoma
• Osteosarcoma with chondroblastic features

Multi-nucleated Giant cells present 3


•

Multi-nucleated Giant cells present 3


Giant cell tumor
• Chondroblastoma
• Aneurysmal bone cyst

Hemosiderin pigmentation


• 3

Hemosiderin pigmentation


• 3

Hemosiderin pigmentation


• -NOF
• -PVNS
• -UBC

CD99•

CD99•

Ewing's

CK125• dx

CK125• dx

CK125• Ovarian CA

CD138•?dx

CD138• Myeloma

Desmin•dx?

Desmin•dx?

Desmin• Rhabdomyosarcoma

CK7•

CK7•

CK7• Breast CA
• Lung CA

Vimentin


• 3

Vimentin


• 3

Vimentin


• synovial sarcoma, rhabodymosarcoma, and leiomyosarcoma

Bisphosphonate therapy 4

-Bisphosphonate therapyMetastatic bone disease (with wide resection and radiation)
-Myeloma (with chemotherapy)
-Paget's disease (with observation)
-Fibrous dysplasia (with observation)

Chemotherapy alone 2

Chemotherapy aloneLyphoma
Multiple myeloma

Wide Resection Alone


•5

Wide Resection Alone


• -Chondrosarcoma
•- Parosteal osteosarcoma
• -Chordoma
• -Adamantinoma
• -Squamous cell (if no mets)

Wide Resection + Irradiation - 2

-Metastatic Bone Disease
•- Soft tissue sarcoma-high grade (angiosarcoma, synovial sarcoma, liposarcoma, desmoid tumor, MFH/fibrosarcoma)

mn vertebra plana
  1. mn vertebra plana

-M-Metts, mylemoma


-E-EG Ewings


-L-Lymoha


-T-trauma TB