Systems Path Exam 2 Part 1: Bone & Soft Tissue Tumors

T/F: Metastatic spread to bone is MC than primary bone tumors.

True

What are the (2) risks for bone tumors?

1. Mutations --> RB or TP53

2. Inflammation (chronic)

T/F most bone tumors are benign

True

Benign tumors are also MC that bone cancers

What are the 3 MC primary bone cancers?

1. Osteosarcoma (multiple myeloma?)

2. Chondrosarcoma

3. Ewing sarcoma

Are Osteoid Osteoma & Osteoblastoma benign or malignant?

Benign

Who does Osteoid Osteoma & Osteoblastoma MC affect?

Male 2x more likely

Age 10-20s

Which is typically larger, Osteoid Osteoma or Osteoblastoma

Osteoblastoma (2-6cm)

T/F Osteoblastoma has localized nocturnal pain that is relieved by aspirin/NSAIDS

FALSE

Osteoid Osteoma

(O.B. has poorly localized pain and is unrelieved by aspirin)

What are the main differences between Osteoid Osteoma & Osteoblastoma?

O.O.: <2cm/ MC metaphysis of femur & tibia/ localzed nocturnal pain

O.B.: 2-6cm/ MC in vertebral processes/ poorly localized pain

Is osteosarcoma benign or malignant?

malignant and VERY aggressive

What is the MC primary bone cancer?

Osteosarcoma

Who does osteosarcoma MC affect?

adolescent males (1.5x)

10-20 years (75% under 20)

What is the MC location(s) of osteosarcomas?

Long bone metaphysis (especially around the KNEE)

What cancer is MC associated with periosteal reaction?

Osteosarcoma

(sunburst appearance or Codman triangle usually)

T/F Osteosarcoma does not cause pain or weaken bone

FALSE O.S. is very painful (rapid onset) and weakens bone

What are the 3 main types of cartilage forming tumors?

1. Osteochondroma (B)

2. Chondroma (B)

3. Chondrosarcoma (M)

Are cartilage-forming tumors MC benign or malignant?

Benign

Is osteochondroma benign or malignant?

benign (it's a cartilage capped outgrowth)

Where is osteochondroma MC located?

Long bone metaphysis near the growth plate

(Knee MC)

Who MC has osteochondroma?

Males (3x)

Ages 10-30

What is a chondroma that grows in the medullary cavity?

enchondroma (MC)

What is a chondroma that grows in cortical bone?

juxtacortical chondroma

Age range for chondromas is ...

20-50

What is Ollier Disease?

Multiple Enchondromas (a result of sporadic mutations)

AKA: enchondromatosis

T/F enchondromas are usually symptomatic

FALSE usually asymptomatic

What is the 2nd MC primary bone cancer?

Chondrosarcoma

Is chondrosarcoma malignant or benign?

Malignant

Who MC has chondrosarcoma?

40-60 years

males (2x)

Where is chondrosarcoma usually located?

- Intramedullary or juxtacortical

- pelvis, shoulder, ribs, prox. femur

What pathology can be describes as a "glistening" mass?

chondrosarcoma

(on Xray, also has popcorn/ stippled calcifications)

What is the difference between low and high grade chondrosarcoma?

High: large mass/ erodes cortex/ metastasize (esp. in lungs) --> significantly worse prognosis

Low: MC! slow growing/ small

Which cartilage-forming tumor is most likely to destroy a joint?

Chondrosarcoma

What are benign tumors/ proliferation of fibroblasts & macrophages?

Fibrous Cortical Defect (FCD) & Nonossifying Fibroma (NOF)

Which is larger: FCD or NOF?

FCD (>3cm)

How would FCD and NOF be described on X-Ray?

Radiolucent lesion with a thin radiopaque edge of sclerosis

(dark/thin circle with a bright/dense edge)

T/F: FCD and NOF are symptomatic.

FALSE: Usually Asymptomatic

(they are usually incidental findings on X-Ray)

Where are FCD and NOF MC found?

KNEE

What is a "failed bony differentiation" of osteoblasts and "arrested development"?

Fibrous Dysplasia (FD)

All Fibrous Dysplasia develop from ________

spontaneous GNAS mutations

What are the 3 types/ severities of Fibrous Dysplasia?

Monostotic = 1 bone (occurs later)

Polyostotic = multiple bones (occurs early)

Polyostotic + cafe-au-lait spots & endocrinopathy = McCune-Albright Syndrome

Which type of FD is MC?

Monostotic (70%)

What is the description of the hip in FD?

"Shepherd's crook" appearance on X-Ray

What 3 systems does McCune-Albright Syndrome affect?

1. skeletal- bony lesions

2. skin- cafe au lait spots

3. endocrine- early puvberty

Is Ewing Sarcoma differentiated or undifferentiated?

UNDIFFERENTIATED

What is the 2nd MC pediatric bone cancer?

Ewing Sarcoma

(#1 = osteosarcoma)

Who MC has Ewing Sarcoma?

10-20 years

caucasians (9x) males

(similar to osteosarcoma)

Where is Ewing Sarcoma MC?

Diaphysis of long bones

("onion skinning" in periosteal reaction)

Is Ewing Sarcoma benign or malignant?

Malignant

Giant-Cell Tumor of Bone is also known as _________.

Osteoclastoma

Where do Giant-Cell Tumors of Bone MC occur?

EPIPHYSIS*/METAPHYSIS

MC near knee

What ages usually get Giant-Cell Tumor of Bone?

20-40

What is the MC site of metastatic spread?

The spine

(axial skeleton)

T/F: Secondary metastasis to bone is MC than primary bone cancer?

TRUE

Describe bony metastasis in adults (and their patterns).***

1. prostate = blastic

2. breast = lytic

3. lung = mixed

What are the 3 most important risk factors in low back pain of cancerous origin?

1. over 50 y/o

2. history of cancer

3. cachexia

T/F: Primary Joint Tumors are MC than Tumor-Like Lesions.

FALSE: Tumor-like lesions are MC than true neoplasms

What is a Ganglion Cyst?

Accumulation of degenerative tissue around/near an injured joint or tendon.

It is common and asymptomatic.

Small & "myxoid"

What is synovial herniation associated with joint degeneration?

Synovial cyst

What are the two types of synovial cysts?

1. Popliteal = Baker Cyst

2. Spinal synovial cyst

An individual with a spinal synovial cyst is most likely to experience which of the following?

A. Headaches

B. Lumbar spinal stenosis

C. Bilateral UE erythema

D. Gangrene in the LE

B. Lumbar spinal stenosis

T/F: Benign tumors are 100x MC than Malignant.

TRUE

EXCEPTION: skeletal muscle tumors

What are the MC locations for soft tissue tumors? (Hint: deep)

Thigh

Retroperitoneum

What is a benign adipocyte tumor?

Lipoma

What is a malignant adipocyte tumor?

Liposarcoma

(MC in retroperitoneum or thigh)

What is the MC soft tissue tumor?

Lipoma

What is Nodular Fasciitis?

Benign fibroblast tumor

(it is firm)

What is heterotopic ossification of fibroblasts DUE TO INJURY?

Myositis Ossificans

in Myositis Ossificans, fibroblasts become ________.

Osteoprogenitor cells

What is a benign tumor of fibroblasts?

Fibromatoses

(Superficial or Deep)

Which type of fibromatoses is more aggressive?

deep (but remember, BOTH are invasive)

What are the 2 common forms of Superficial Fibromatoses?

Pyronie Disease - Penis, MC 40-70

Dupuytren Contracture - hands/ ulnar forearm. (MC old European males.)

What is a malignant tumor of fibroblasts?

Fibrosarcoma

T/F: Fibrosarcomas are encapsulated.

FALSE: Unencapsulated

*herring bone histology

What is an aggressive malignancy of skeletal muscle tumors?

Rhabdomyosarcoma

Who does Rhabdomyosarcoma MC occur in?

MC pediatric soft tissue sarcoma

What are the two smooth muscle tumors? Are they malignant or benign?

Leiomyoma = benign

Leiomyosarcoma = malignant (MC adult females)

What is synovial sarcoma? Who is it most commonly seen in?

Soft tissue cancer that's aggressive & painless. (MALIGNANT)

MC in 20-40 y/o.

Where would you expect an enchondroma to be discovered?

hands & feet

Where would you expect an osteoblastoma to be discovered?

spine (vert. processes)

Who is most likely to be diagnosed with an osteosarcoma?

A. a 4-year-old

B. a 14-year-old

C. a 44-year-old

D. a 64-year-old

B. 14 y/o

(MC boys)

The 64 y/o would be predisposed if they have something like Paget's

A Codman triangle indicates that a tumor is _________.

A. causing a periosteal reaction

B. invading the marrow

C. metastasizing

A. causing a periosteal reaction

Who is most likely to develop a chondrosarcoma?!?!

A. an infant

B. a child

C. an adolescent

D. an older adult

D. an older adult

Where is a chondrosarcoma most likely develop?

A. Proximal tibia

B. Proximal humerus

C. Proximal phalanx

D. Proximal metacarpal

B. Proximal humerus

Where is an osteosarcoma most likely to develop?

A. Proximal tibia

B. Pelvis

C. Distal humerus

D. Craniofacial bones

A. Proximal tibia

Who is most likely to develop Ewing sarcoma?

10-20 y/o boys

Caucasian highest risk.

An osteoblastoma is a _______ that develops within the _______.

A. benign, humerus

B. benign, spine

C. malignant, tibia

D. malignant, femur

B. benign, spine

An osteoid osteoma is most likely to develop within the ________.

A. lower extremity

B. upper extremity

C. pelvis

D. spine

A. lower extremity

A periosteal reaction is an indication that the tumor is likely ______.

A. benign

B. malignant

B. malignant

An enchondroma is most likely to develop within the ___________.

A. transverse process of a vertebral body

B. proximal humerus

C. distal femur or proximal tibia

D. proximal phalanx of the hand

D. proximal phalanx of the hand

Which of the following is more common?

A. metastasis to bone

B. primary bone cancer

A. metastasis to bone

Where is an osteochondroma most

likely to be discovered?

A. anterior vertebral body

B. distal femur or proximal tibia

C. metacarpal

D. proximal humerus

B. distal femur or proximal tibia

What is the most common bone cancer of adulthood?

A. Osteosarcoma

B. Ewing sarcoma

C. Osteochondroma

D. Chondrosarcoma

D. Chondrosarcoma

A fibrous cortical defect is _________.

A. likely to cause a pathologic fracture

B. most likely to self-resolve

C. likely to transition into cancer

D. a common cause of AVN

B. most likely to self-resolve

McCune-Albright syndrome is a formof __________.

A. dwarfism

B. fibrous dysplasia

C. osteoid osteoma

D. fibrous cortical defect

B. fibrous dysplasia