chapter 27 bone, cartilage, and soft tissue sarcomas

what are some prognostic indicators for Ewing's sarcoma?

-amount of extension at the time of diagnosis -location of the tumor (pelvis has poor prognosis) -males have a worse prognosis than females

what are some radiographic appearances of primary bone tumors?

-permeative pattern -sunburst periosteal reaction (osteogenic sarcoma) -onion skin periosteal reaction (Ewing's sarcoma) -metastasis

osteogenic sarcoma: less than _____% are diagnosed as grade 1? _______% diagnosed grade 3 or 4.

1%; 85%

what are the 2 most common classifications in staging of soft tissue sarcomas?

1. musculoskeletal tumor society (MSTS) 2. American join commission on cancer (AJCC)

Only _____% are malignant giant cell tumors

10%

what % of bone tumors do Ewing's sarcomas account for?

16%

when are chondrosarcoma mostly diagnosed in adults?

30-60 years old

what % does osteogenic sarcoma account for all bone tumors?

35%

giant cell tumors account for ______% of primary bone tumors

5%

What is the staging system that is now used for primary bone tumors and it replaced what is what?

AJCC Update; replaced compartmentalization with tumor size

Strontium-89 causes minimal irradiation of normal tissue because it is a pure _____________ emitter. It is excreted in the urine within _______ hours.

Beta; 48

what was the staging system previously used for primary bone tumors?

Enneking system

Onion skin appearance is found in what tumor?

Ewing's sarcoma

chondrosarcoma: prognosis depends of 2 factors:

Histologic grade Location

it is important when treating an extremity for STS to have a strip of unirradiated tissue near the treatment volume to: I. maintain some lymphatic function II. avoid increased fracture possibilities III. avoid circumferential fibrosis

I and III

treatment for multiple myeloma involves: I. radiation therapy II. surgery III. chemotherapy

I and III

primary treatment for osteogenic sarcoma involves: I. radiation therapy II. surgery III. chemotherapy

II and III

Does MFH have a worse prognosis in the bone or soft tissue?

In the bone

Where are malignant fibrous histiocytoma found?

In the bone or soft tissue of any body part

where is the most common site of presentation of a soft tissue sarcoma?

Lower extremities

What are the subtypes of soft tissue sarcomas and what % do they make up?

MFH 28% Liposarcoma 15% Leiomyosarcoma 12% Synovial and unclassified 10% Malignant peripheral nerve sheath tumors 6%

May progress to complete loss of normal bony structure common in prostate cancer. Increased density.

Osteoblastic

Irregular and poorly defined margins. May produce a single large focus (blow out) common with breast cancer metastasis

Osteolytic

what disease has an excessive and abnormal bone resorption and formation?

Paget's disease

what is the most common STS in children peaking before age 20?

Rhabdomyosarcoma

bone scans are used to evaluate the:

Spread of metastatic disease

where does peripheral primitive neuroectodermal tumor start?

Start in the chest wall

what radiopharmaceutical may be used in treating metastatic bone disease resulting from primary prostate or breast cancer?

Strontium-89 Samarium-153

how is Ewing's sarcoma treated?

Surgery and Chemotherapy

A chondrosarcoma can also occur as a secondary malignancy developing in preexisting benign lesion.

True

Ewing sarcoma of bone is most common.

True

Patients are at risk for pathological fractures with metastatic bone disease.

True

When is radiation therapy used for Ewing's sarcoma?

Tumors are in areas where the use of surgery is limited. Pre and post operatively. Bone marrow transplant

during which of the following time frames is the incidence rate of primary bone cancer highest?

adulthood

The most important prognostic factor in Ewing's sarcoma is:

amount of extension at the time of diagnosis

all of the following are examples of primary bone tumors except: a. malignant histiocytoma b. rhabdomyosarcoma c. osteosarcoma d. multiple myeloma

b. rhabdomyosarcoma

the majority of giant cell tumors are

benign

what is the most common primary bone tumor in adults?

chondrosarcoma

the second most common type of primary bone tumors are ______________ and are of _____________ origin.

chondrosarcoma, cartilage

where is an osteogenic sarcoma most commonly found?

distal femur (especially around the knee)

what are some late effects of radiation therapy?

edema fibrosis growth deficits in children second malignancy sterility endocrine dysfunctions

pain is the most common presenting symptom of soft tissue sarcoma (STS).

false

What are some acute radiation side effects when chemotherapy is also used?

fever neutropenia mucositis nausea, vomiting diarrhea erythema

where are fibrosarcomas derived from?

fibroblasts (cells that produce connective tissue)

soft tissue sarcoma post operative radiation therapy: A skin strip of 1-3cm along the extremity is critical to prevent __________ and ______________.

fibrosis and lymphadema

where does multiple myeloma occur?

flat bones such as vertebra, skull, ribs and pelvis

what is the treatment goal for bone metastasis?

goal is pain relief and the prevention of fractures to weight bearing bones

what are some prognostic indicators for soft tissue sarcomas?

grade most critical predictor site, size resectability presence of metastasis local invasion of a pseudocapsule regional lymph node involvement local recurrence

overall, STS requires which dose of radiation to achieve local control and long-term survival?

high

Loss of calcium in the bone

hyperparathyroidism

when used with radiation therapy, which of the following reduces the loss of function and improves overall control?

limb-sparing surgery

In which anatomical location are primary bone lesions most common?

lower extremity

what is the most common site of metastasis from a primary bone cancer?

lung

Ewing's sarcoma is more predominant in

males than females

what is the most common bone disease?

metastatic bone disease

which of the following has the highest incidence of bone tumors?

metastatic bone lesions

Swiss cheese appearance is found in what tumor? And is considered osteolytic or osteoblastic?

multiple myeloma (osteolytic)

Patients previously treated with radiation therapy for retinoblastoma are at risk for what type of primary bone cancer?

osteogenic sarcoma

Typical sunburst pattern is found in what tumor?

osteogenic sarcoma

what is the most common osseous primary bone tumor in children?

osteogenic sarcoma (osteosarcoma)

infection of the bone or bone marrow

osteomyelitis

what is the most common form of primary bone cancer?

osteosarcoma

the most common presenting symptom of patients with bone tumors is:

pain

what is the most frequent symptom of metastatic bone disease?

pain developing gradually over time

what is the most common presenting symptom of a soft tissue sarcoma?

painless mass that grows over several weeks or months

what is the most important prognostic factor for osteosarcoma?

presence of metastatic disease

what are the 2 types of bone tumors?

primary and metastatic

why is radiation therapy not the treatment of choice for an osteogenic sarcoma?

radioresistant and would require doses resulting in severe tissue damage and subsequent amputation

in which anatomical location are metastatic bone lesions most common?

spine

where do fibrosarcomas most often occur?

spine, pelvis, and ribs

where does extraosseous Ewing tumors start?

start in the soft tissue surrounding bone

what is the treatment of choice for a chondrosarcoma?

surgery (surgical removal of the entire tumor mass with adequate bone and soft tissue margins)

what is the treatment of choice for a soft tissue sarcoma?

surgery, whether used alone or with radiation and/or chemotherapy

what is the treatment of choice for giant cell tumors?

surgical removal

Where do Ewing's sarcoma generally arise in?

the diaphysis of the bone

Where does MFH metastasis to?

the lungs

the most frequent symptom of metastatic bone tumors is pain over time.

true