MSK Oncology

Question 1

What lineage are MSK tumors from?

Answer 1

Mesenchymal cells

Question 2

Sarcoma

Answer 2

Malignant tumor of mesenchymal cell origin

Question 3

Where is most common site for soft tissue sarcomas?

Answer 3

Anterior quadriceps

Question 4

2 most common soft tissue sarcomas.

2 others

Answer 4

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

Question 5

How are soft tissue sarcomas treated?

Answer 5

Surgery and radiation

Question 6

Rhabdomyosarcoma
Type
Age
Origin
Treatment

Answer 6

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

Question 7

Synovial sarcoma
Type
Age
Location
Morphology
Treatment

Answer 7

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.

Question 8

Where do most sarcomas metastasize?

Answer 8

Lungs

Question 9

Are soft tissue or bony sarcomas more common?

Answer 9

Soft tissue

Question 10

2 types of benign bone tumors

Answer 10

Unicameral bone cysts

Fibrous dysplasia

Question 11

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

Answer 11

• 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

Question 12

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

Answer 12

• 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.

Question 13

4 types of malignant bone tumors

Answer 13

Osteogenic sarcoma, Ewing sarcoma, MM, metastasis

Question 14

Osteogenic sarcoma
Type
Age
Cell activity
Imaging
Treatment

Answer 14

• 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.

Question 15

Ewing Sarcoma
Type
Cell of origin
Age
Histology
Treatment

Answer 15

• 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.

Question 16

Multiple Myeloma
Type
Isolated form
Urine findings
Imaging
Treatment

Answer 16

• 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.

Question 17

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

Answer 17

• 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.

Question 18

Malignant cancers by age

Answer 18

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

Question 19

Achondroplasia mutation / effects

Answer 19

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

Question 20

Osteogenesis imperfects mutation

Presentation

Answer 20

Dominant mutation in type 1 collagen

Usually presents in infancy. Bowing / deformities / fractures of bones. Blue-gray sclerae.