3e. Other Primary Neoplasms

Question 1

What is a Fibrosarcoma (FS)?

Answer 1

A RARE sarcoma in which the predominant cell type is a malignant fibroblast.

Question 2

MC age group for FS?

Answer 2

4-83…but not usually common in kids

Question 3

FS usually occurs where?

Answer 3

Major long bones (50% at knee)

Question 4

What is the average length of time of symptoms in FS before diagnosis?

Answer 4

2 years! Pain and swelling

Question 5

FS Plain film findings?

Answer 5

• Highly destructive, expanding, lytic lesion
• HUGE soft tissue mass
• Usually NO periosteal reaction

Question 6

FS Prognosis?

Answer 6

Poor prognosis, mets late to lung, liver, lymph, and brain

Question 7

Treatment of choice?

Answer 7

AMPUTATION

Question 8

What is Malignant Fibrous Histiocytoma (MFH)?

Answer 8

• MC soft tissue sarcoma in adults
• Indistinguishable from Fibrosarcoma
• Primitive mesenchymal cells
• Mets to lungs
• Painless, solid mass

Question 9

Where is MFH more common?

Answer 9

Lower extremity (50%)

Question 10

What is a Chordoma?

Answer 10

Rare, notochordal remnant neoplasm

Question 11

Where do Chordoma’s usually happen?

Answer 11

Spinal column (85% sacrococcygeal or spheno-occipital)

Question 12

Age range of Chordoma?

Answer 12

Any age, but 40-70 MC

Question 13

MC in lads or lasses?

Answer 13

Lads

Question 14

T or F: Metastasis is uncommon in Chordoma’s

Answer 14

T

Question 15

What are symptoms from a Chordoma usually from?

Answer 15

Mass effect

Question 16

Other than the Sacrococcygeal, what other places commonly have Chordoma’s?

Answer 16

Clivus, Vertebra (especially C2).

Question 17

What is the only primary malignancy to cross IVD?

Answer 17

Chordoma

Question 18

Prognosis for Chordoma?

Answer 18

It varies, many of the lesions can be difficult to resect (spinal column)

Question 19

Plain film findings for Chordoma?

Answer 19

• Lytic destruction
• Cortical expansion (may increase AP diameter of sacrum)
• Calcification in 50%
• Soft tissue mass common
• Ddx: lytic mets, chondrosarcoma, GCT, ABC, plasmacytoma

Question 20

What is Non-Hodgkins Lymphoma (NHL)?

Answer 20

A rare extranodal lymphoma, initially appearing as a solitary bone lesion

Question 21

Age range for NHL? (not the hockey league)

Answer 21

20-50

Question 22

…NHL is more common in guys, okay?

Answer 22

Why did you flip this card over?

Question 23

Prognosis for NHL?

Answer 23

Better than most bone primary malignancies

Question 24

Pain pattern for NHL?

Answer 24

• Local, intermittent pain

- Dull aching, not relieved by rest

Question 25

Are most patients with NHL healthy or unhealthy?

Answer 25

Generally healthy, but over 50% will have symptoms in a year (palpable mass or swelling)

Question 26

Plain film findings for NHL?

Answer 26

- Permeative destruction - Minimal periosteal response - Pathological Fx is very common - Femur, tibia, and humerus MC affected - Could have soft tissue mass

Question 27

What is Hodgkins Lymphoma of Bone (HLB)?

Answer 27

- Usually secondary to systemic Hodgkin's (10-20% of patient's with Hodgkin's) - Rarely primary in bone

Question 28

MC symptom of HLB?

Answer 28

- Pain

Question 29

Prognosis of HLB?

Answer 29

Treatment and prognosis related to stage of systemic disease

Question 30

What is Hodgkin's Lymphoma (HL)?

Answer 30

A type of lymphoma, which is generally believed to result from white blood cells of the lymphocyte kind

Question 31

Where is HL MC?

Answer 31

In the vertebral body ("ivory vertebrae", anterior scalloping possible)

Question 32

Are sclerotic or lytic HL lesions MC?

Answer 32

Lytic (especially in tubular bones)

Question 33

Periosteal response in HL?

Answer 33

Possible exuberant periosteal response

Question 34

What is Synovial Sarcoma (SS)?

Answer 34

An uncommon soft tissue sarcoma

Question 35

MC age range for SS?

Answer 35

30-50

Question 36

Where is SS MC?

Answer 36

Knee, hip, and ankle

Question 37

SS Plain film findings?

Answer 37

- Fine granular calcification in 1/3rd | - Secondary bone destruction in 10-20%

Question 38

SS Prognosis?

Answer 38

Moderately good

Question 39

Most useful imaging for SS?

Answer 39

MRI

Question 40

Treatment for SS?

Answer 40

Excision and/or radiation