Path 2: Neoplasms

Class 4

*Primary tumors develop from or within musculoskeletal ______ or _____in a localized area

Primary tumors develop from or within musculoskeletal soft tissue or bone in a localized area

What are the two types of primary tumors?

Benign & Maliganant

Ratio of Bengin to Malignant

Bengin 100: 1 Maligant

A benign neoplasm ____ always innocuous?

NOT always inoocuous

(A non-cancerous bone tumor isn’t always harmless)

A pathological fracture may occur in benign bone tumors due to what activity in the bone cell?

Resorption from osteoclasts

Some benign tumors may transform into _______.

Malignant

What location in the body do skeletal neoplasms often metastasize in through the blood stream?

Skeletal neoplasms often metastasize to the lung by the bloodstream

What is the ratio of incidence of men to women in primary musculoskeletal tumors?

men 3:1 women

Primary bone tumors are _____ tumors

Rare

What primary bone tumor accounts for 50% of tumors?

Multiple Myeoma (MM); from bone marrow

What is another term for secondary bone tumors? What is the ratio that it outnumbers primary tumors?

Metastatic bone tumors

Metastatic tumors outnumber primary tumors 10:1

What are the 4 types of Benign bone tumors?

1. Osteoid Osteoma

2. Chondroma

3. Nonossifying Fibroma (NOF)

4. Giant cells tumors

What type of bone tumors outnumber primary bone tumors?

Metastatic (secondary bone tumors)

metastatic 10:1 primary

T/F. Benign bone tumors require treatment

False

Is there a potential for malignant transformation in Osteoid Osteoma?

No potential to become cancerous

What drug usually relives pain for Osteoid Osteoma? What type of treatment is required if symptomatic.

Aspirin; surgical treatment

Where is Chondroma typically located in the bone?

Centrally within a bone

If the Chondroma lesion is asymptomatic do you require treatment?

no

If no treatment is needed in chondroma, what will be required as a follow up?

X-ray

What is the most common benign bone tumor in children?

Non-ossifying Fibroma

How much more often is non-ossifying fibroma in males compared to females?

Twice more often in males

If a child has NOF, will there be potential for cancer to develop?

No potential for cancer to develop in non-ossifying fibroma

What is the percentage of people in which giant cell tumors may reoccur locally?

50% of cases may recur

In some cases of giant cell tumor, a ______ may develop.

sarcoma

What are the 5 types of risk factors for malignant bone tumors?

1. Paget’s disease (increased one resorption & increased disorganized growth)

2. Li-Fraumeni syndrome (rare hereditary predisposition to cancer)

3. Chemotherapy drugs (cyclophosphamide)

4. Ionizing radiation over exposure

5. Hereditary retinoblastoma

What types of malignant bone tumors are more common in males?

1. Osteosarcoma - originate from osteoblasts

2. Chondrosarcoma - made of neoplastic chondrocytes

3. Ewing’s sarcoma - made of undifferentiated bone marrow cells (bone cells & soft tissue)

4. Multiple myeloma - develops from bone marrow cells

What accounts for half of all malignant solid bone tumors?

Osteosarcoma

Which part of the bone does osteosarcoma often involve?

Metaphysis of long bones

T/F, Osteosarcoma is NOT highly malignant

False. Osteosarcoma IS highly malignant

What malignant bone tumor does osteosarcoma originate from?

Origianted from osteoblasts

What malignant bone tumor is composed of neoplastic chondrocytes?

Chrondrosarcoma

What malignant bone tumor is composed by undifferentiated bone marrow cells?

Ewing’s sarcoma

What malignant bone tumor develops from bone marrow cells?

Multiple myeloma

What population and age group is Osteosarcoma most common in?

Male children, adolescent & young adults (under 30)

What are common symptoms of osteosarcoma?

Pain & mass

At what stage does lung metastasis occur in 90% of cases of Osteosarcoma?

Lung mestases occur early in 90% of cases

In the past for Osteosarcoma, surgery required amputation, what tx may allow for pulmonary metastases?

Surgery may allow pulmonary metastases

Currently for Osteosarcoma, there is no amputation and it accounts for how much survival?

90% survival with no amputation

What type of tx is ineffective in osteosarcoma?

Radiotherapy is ineffective

What type of cell is responsible for chondrosarcoma?

Chondrocytes

What is the second most common solid bone cancer in adults?

Chondrosarcoma (less metastatic than osteosarcoma)

What is the most common presenting complaint of chondrosarcoma?

Pain

What is the treatment for chondrosarcoma?

Complete surgical removal of tumor → “Chondro needs a complete surgery not chemo”

What is the second most common primary bone malignant tumor in children, adolescent and young adults?

Ewing’s Sarcoma

What age group does 80% of Ewing’s sarcoma occur in?

People under 20

What are the treatments for Ewing’s Sarcoma?

Chemotherapy (to eradicate metastases)

Radiotherapy (local tumors are responsive)

Immunotherapy

Embolization

Surgery (limb sparing)

T/F Ewing’s sarcoma is a highly malignant tumor

True

T/F. In Ewing’s Sarcoma, there is a high recurrence and metastases for malignant tumor

True

Giant cell tumor and has a ____ rate of recurrence and a potential for _______transformation

High rate; malignant

Giant cell tumor is more often seen in the ______ demographic, with 20% of population affected.

Chineese

Multiple Myeloma is a ______-cell derived. or a ____-cell ______

plasma; plama-cell dyscrasia (abnormal)

In MM, most patients die within______ after diagnosis

1-3 years

In multiple myeloma (MM), there is an overgrowth of _____by neoplastic cells

bone marrow

(neoplastic cells are abnormal cells that are excessive forming a mass tumor or neoplasm)

Most patients of MM are older than ___.

45

Multiple Myeloma is also called what condition

Monoclonal (group of blood disorders)

In multiple myeloma the surrounding _____ is destroyed and _____ are seen in the blood-forming bones.

bone to the marrow; punched out holes

The cause of death in MM is usually _______

kidney failure

Hypercalcemia from _______ cases calcium deposition in kidneys and organs.

Osteolysis

Osteolysis occurs when balance between osteoblasts and osteoclasts occur.

Fibromas, lipomas, rhabdomyomas, neurofibromas, schwannomas are all _______

Benign soft tissue tumors

What is the most important soft tissue tumor in children?

Rhabdomyosarcoma

Where is Rhabdomyosarcoma often located?

In exremedies and trunk

What is the second most common sarcoma in adults?

Liposarcoma

Lipoma is composed of _____adipocytes

malignant

Osteoid Osteoma

A rare benign vascular osteoblastic lesion

Benign cartilage tumors (3)

Chondrosarcoma
Endosarcoma
Chondroblastoma

(malignant: Chondrossarcoma)

Benign tumors affecting blood vessels

Angioma
Hemangioma

Bengin tumors affecting smooth muscle

Leioma

(malignant: Leiosarcoma)

Malignant bone tumors of bone marrow

Multiple Myeloma

Ewings Sarcoma

Leukemia

What is an Osteoid Osteoma?

Benign osteoblastic lesion - growth of new bone on existing bone. Located int he cortex of long bones

Cortex of long bones

Males < 25

Aspirin

Surgery if symptomatic

NO potential to become malignant

Osteoid Osteoma - cell type, location & tx

Osteoblastic cells. Cortex of long bones

Aspirin; surgical removal if symptomatic

No maligant potential

What is Chondroma and when do you treat it?

Benign cartilage tumor located centrally within bone (epiphyseal plate)

No tx if asymptomatic - just X-ray follow up.

Can transform to chondrosarcoma

What is Non-ossifying Fibroma (NOF)?

Fibrous tissue replaces normal bone during development. Most common benign tumor in children. NEVER becomes cancerous - resolves at skeletal maturity

Osteoid Osteoma - cell type, location & tx

Fibroblasts, no tx needed (resolves with maturity)

Giant cell tumor - why is it now a low-grade sarcoma

High local reoccurrence (50% cases) & potential for malignant transformation

Classically considered benign, now reclassified

Giant cell tumor - location, demographics, features?

Mostly around knee, distal radius.

Epiphysis of long bones

Adults 20-55 (peak 30s)

Pulmonary mets grow slowly

Osteosarcoma - who gets it, where and how bad?

Males under 30. Metaphysis of long bones - 50% at knee

Highly malignant

About half of all malignant solid bone tumors

What changed osteosarcoma survival from 20% to 70-80%?

Before 1970: amputation (low survival). Now: adjunctive chemo FIRST, then limb-sparing surgery. Radiation = zero effect

Osteosarcoma - link to Paget’s disease?

Older adults can develop osteosarcoma as a complication of Paget’s disease. Causes disordered bone remodeling and predisposes to malignant transformation.

Same tx applies

Radiation fails

Osteosarcoma

Chondrosarcoma

Multiple Myeloma

Chemo Fails

Chondrosarcoma

Multiple Myeloma

BUT Ewing’s = very responsive

Chondrosarcoma - demographics, location, treatment

Men 40-60. Pelvic & shoulder girdles. Slow-growing. Arises from pre-existing Chondroma or Paget’s disease. Surgery only, chemo & radiation = no work

Why are chemo and radiation useless for Chondrosarcoma?

Chondrosarcoma is slow-growing (malignant), so chemotherapy (which targets rapidly dividing cells) has limited impact. Radiation is also ineffective. Complete surgical resection is the only treatment.

Ewing’s Sarcoma - who gets it, where, how does it present?

People under 20. Pelvis & lower extremities / tubular bones (femur, tibia). Progressive pain, pathological fractures. Often MISDIAGNOSED with osteomyelitis.

Second most common primary bone malignant tumor in children, adolescent & young adults

Ewing’s Sarcoma

What treatments work for Ewing’s sarcoma?

All modalities work: chemotherapy + radiotherapy (very responsive locally) + surgery + immunotherapy + embolization

What malignant bone tumor has a high recurrence & metastases?

Ewing’s sarcoma = Highly maligant tumor

Why is Ewing’s misdiagnosed as osteomyelitis?

Both cause bone pain in young patients. Ewing’s pain worsens after sports injury. Biopsy distinguishes them - Ewing’s show undifferentiated small round cells

What are the risk factors for malignant bone tumors?

Paget’s disease, Li-Fraumeni syndrome, chemotherapy drugs (cyclophosphamide), radiation over-exposure, hereditary retinoblastoma

Never malignant (2)

NOF (Non-ossifying fibroma)

Osteoid Osteoma

Chondroma CAN transform

How does multiple myeloma spread?

ONE plasma cell undergoes malignant transformation → clonal expansion → overgrowth of bone marrow by neoplastic cells. It is called a monoclonal condition

Multiple Myeloma - demographic, prognosis

Most patients die within 1-3 years after diagnosis

What is the Bence jones protein?

Immunoglobulin chains produced by malignant plasma cells that appear in urine. Classic diagnostic marker for Multiple Myeloma
(Antibody in urine → Excess protein → kidney damage)

What are punched-out lesions and what causes them?

Lytic holes in bone especially in skull & pelvis. Caused by overactivation of osteoclast - bone is destroyed faster than it can be rebuilt (osteopenia = low bone mineral density)

Multiple Myeloma cause of death

Kidney (renal) failure from protein overload & hypercalcemia (calcium deposition that damages kidneys. Chemo is ineffective

What is Rhabdomyosarcoma and who gets it?

Most important soft tissue tumor in children. Striated muscle cells. Located in extremities and trunk. In adults, mostly originates in extrinsic eye muscles

Liposarcoma vs. Lipoma - how do you tell them apart?

Lipoma = benign fat tumor. Liposarcoma = malignant adipocytes. Low-grade liposarcoma can look similar to benign - biopsy needed. 2nd most common sarcoma in adults.

Synovial sarcoma is a _________.

Name cell origins

Malignant soft tissue sarcoma
Mesenchymal stem cell

Angiosarcoma is a _________.

Name cell origins

Malignant soft tissue sarcoma

blood vessels & endothelium