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Flashcards in MSK Oncology Deck (20):
1

What lineage are MSK tumors from?

Mesenchymal cells

2

Sarcoma

Malignant tumor of mesenchymal cell origin

3

Where is most common site for soft tissue sarcomas?

Anterior quadriceps

4

2 most common soft tissue sarcomas.
2 others

Liposarcomas and undifferentiated fibrosarcomas are most common.
2 others include rhabdomyosarcoma and synovial sarcoma

5

How are soft tissue sarcomas treated?

Surgery and radiation

6

Rhabdomyosarcoma
Type
Age
Origin
Treatment

Most common soft-tissue sarcoma in kids.
Origin is skeletal muscle.
Treat w/ excision, radiation, and surgery.

7

Synovial sarcoma
Type
Age
Location
Morphology
Treatment

Soft tissue sarcoma
Commonly seen in young adults
Occurs in joints of hands / feet.
Cells appear to form synovial clefts.
Treat w/ surgery and radiation.

8

Where do most sarcomas metastasize?

Lungs

9

Are soft tissue or bony sarcomas more common?

Soft tissue

10

2 types of benign bone tumors

Unicameral bone cysts
Fibrous dysplasia

11

Unicameral bone cysts
Type
Morphology
Age
Location
Prognosis
Treatment (4)

•Benign bone tumor
•Well circumscribed lytic lesion in bone
•Often seen in humerus of kids. Developmental, starting close to growth plate. Smooth outline.
•Most spontaneously resolve w/ skeletal maturity. May require surgery if it persists.
•Treatment options: bone graft, injection, cryotherapy, and observation

12

Fibrous dysplasia
Type
Pathology
Age
Pathognomonic feature
Prognosis
Albright's Syndrome
Imaging
Histology
Treatment

•Bone hamartoma (benign neoplasm)
•Causes lytic bone deformity w/ fractures.
•Most common in kids.
•Pathognomonic features is that bone is being laid down by fibrous stroma rather than by rimming osteoblasts.
•Quality of bone often improves w/ age.
•Albright’s Syndrome – severe form in which pxs have precocious puberty, café au lait spots, and fibrous dysplasia.
•X ray shows lytic lesions w/ ground glass
•Histology – fibrous tissue b/w bone trabecular
•Medical tx is best. Surgery is reserved for chronic pain or increasing deformity.

13

4 types of malignant bone tumors

Osteogenic sarcoma, Ewing sarcoma, MM, metastasis

14

Osteogenic sarcoma
Type
Age
Cell activity
Imaging
Treatment

•Highly aggressive malignant bone tumor
•Often seen in kids.
•Cells make osteoid (aids in diagnosis).
•Imaging – Osteoblastic bone is formed in canal and a “SUNBURST” periosteal reaction surrounds the bone.
•Treatment is based on limb salvage surgical resection w/ preop and post chemotherapy.
• 90% of pxs can have legs salvaged. Resect bone and then transplant donated femur.
• In very young pxs that have lots of growth left, amputation is tx of choice.

15

Ewing Sarcoma
Type
Cell of origin
Age
Histology
Treatment

•Malignant bone tumor
•Primitive tumors w/ cells that are difficult to determine origin. Usually osteolytic.
•Seen in kids and young adults
•Histology shows sheets of small round blue cells that stain positive for glycogen.
•Treatment involves chemo, surgery, or radiation. Depends on site. Surgery done in expendable bones. Radiation done in bones that have lots of remaining growth.

16

Multiple Myeloma
Type
Isolated form
Urine findings
Imaging
Treatment

•Malignant plasma cell tumor in marrow. Almost always lytic.
•Called isolated plasmacytoma if only found in one bone. 80% progress to MM in 5 years.
•Urine proteins may be high
•Bone scan is usually cold since there is very little osteoblastic activity.
•Treat w/ chemo, immobilization, bisphosphonates (block osteoclasts to reduce risk of fracture). NOT surgery b/c it is a systemic disease.

17

Metastatic bone disease
Type of cancer
Most common sites
Blastic vs lytic types
Diagnosis
Treatment

•Often from carcinomas.
•Most common sites are vertebrae, pelvis, femurs, and humerus
•Prostate cancer is often blastic.
•Lung cancer and renal cell carcinomas are often lytic.
•Breast cancer may be blastic or lytic.
•All pxs w/ metastatic disease should get a bone scan. May be cold if tumor is not blastic.
•Treatment – radiation + chemo of primary tumor. Surgery reserved for pxs who do not respond. Prophylactic skeletal fixation done in pxs w/ fracture or who are about to fracture. Radiation decreases bone strength.

18

Malignant cancers by age

Osteogenic sarcoma and Ewing sarcoma are more common in kids.
Lymphomas are common in 2nd / 3rd decade.
MM is most common malignant bone tumor in pxs > 40 y/o

19

Achondroplasia mutation / effects

Dominant mutation in FGFR3 --> disruption of cartilage development and growth of long bones

20

Osteogenesis imperfects mutation
Presentation

Dominant mutation in type 1 collagen
Usually presents in infancy. Bowing / deformities / fractures of bones. Blue-gray sclerae.