Exam 3: Malignant Tumors (Part 2)

Primary malignant neoplasia accounts for ____ of all malignancies

30%

What are the MC primary malignant neoplasia's (in order)?

1. multiple myeloma

2. osteosarcoma

3. chondrosarcoma

4. ewing's sarcoma

What are the MC primary malignancies in KIDS?

1. osteosarcoma

2. ewing's sarcoma

What are the MC primary malignancies in ADULTS?

1. multiple myeloma

2. chondrosarcoma

Solitary Plasmacytoma

-localized form of plasma cell proliferation

-vertebra, pelvis, skull, sternum, ribs

-typically presents as soap bubbly, highly expansile lesion

-spares posterior elements

-70% develop into diffuse form

Multiple Myeloma

-malignant proliferation of plasma cells that infiltrate the bone marrow

-MC primary malignant bone tumor

-accounts for 27% of biopsied bone tumors

->10% of hematologic malignancies

Multiple myeloma is the _____ most prevalent blood cancer

3rd

Multiple myeloma clinical features/age and sex:

-usually 5th-8th decade of life

-75% of patients are between 50-70 years old

-unknown etiology

-one of the round cell tumors

-male to female ratio = 2:1

-2x as common in african americans and least common in asian americans

Multiple myeloma clinical features/signs and symptoms:

-pain is the initial cardinal symptom

-unexplained weight loss, cachexia

-respiratory bacterial infections

-anemia

-renal disease

-respiratory disease

-deossification of bone

-production of abnormal serum and urinary proteins

What causes renal disease in patients with multiple myeloma?

excess calcium in the blood due to deossification of bone is harsh on kidneys

What abnormal serum/urinary proteins are present in multiple myeloma?

bence jones proteins

Laboratory findings associated with multiple myeloma:

-elevated ESR

-thrombocytopenia

-rouleaux formation

-increased serum Ca+

-elevated plasma proteins

-hyperglobuinemia with reversed A/G ratio

-PEP SPIKE

-monoclonal globulin production (IgG, IgA, etc)

-bence jones proteinuria

-possible elevated uric acid

Common tetrad of multiple myeloma (CRAB)

-calcium elevated

-renal failure/dysfunction

-anemia

-bone lesions

Skeletal locations of multiple myeloma:

-vertebra, thoracic and lumbars

-pelvis, skull and ribs

-clavicle and scapula

-femur and humerus

-areas rich in red marrow

Radiographic features of multiple myeloma:

-circumscribed osteolytic defects (punched out)

-bone scans may be normal

-MRI sensitivity for marrow changes

-osteoporosis/osteopenia (MC)

-osteolytic defects (rain drop skull)

-osteoblastic lesions in

Rain Drop Skull

multiple lytic defects results in punched out appearance in the skull from multiple myeloma

Treatment/prognosis of multiple myeloma:

-palliative treatment

-chemotherapy and/or radiotherapy

-hydration and ambulation

-5 year survival in 20%

Osteosarcoma

-primary malignant tumor of undifferentiated connective tissue which forms neoplastic osteoid

-2nd MC primary bone tumor overall

-MC primary bone tumor in kids/young adults

-unknown etiology

Osteosarcoma clinical features/age and sex:

-bimodal distribution of 10-25 years old and > 60 years old

-male to female ratio = 3:2

-patients tend to be taller than their peers

What causes patients with osteosarcomas to be taller than their peers?

osteosarcomas attack areas of rapid growth (knees) and can cause an increase in height

Osteosarcoma clinical features/signs and symptoms:

-painful swelling 85% of the time

-antecedent history of trauma

-pain initially insidious and transient becoming severe and persistent

-minor restriction of joint motion

-possible limp

-fever may occur

What are the different forms of osteosarcomas?

-central (MC)

-parosteal

-secondary

-multicentric

-extraosseous

Skeletal location of osteosarcoma:

-distal femur (MC), proximal tibia and proximal humerus

-75% occur in the METAPHYSIS next to the growth plate

-cylindrical bone involvement

Laboratory findings and pathological features of osteosarcoma:

-sclerotic = 50%

-lytic = 25%

-blastic = 25%

-metastasis to lungs is common

-elevated alkaline phosphatase

Radiographic features of an osteosarcoma:

-typically METAPHYSEAL region of long bones, especially around the knee and proximal humerus

-ill defined dense ivory or sclerotic lesion >5cm filling the medullary space

-moth eaten, permeative medullary and cortical destruction is common

-spiculated/sunburst periosteal reaction and codman's triangle

-large soft tissue mass with tumor new bone

-pathological fractures

Advanced imaging and findings of an osteosarcoma:

-NUC shows increased activity, hypervascularity and new bone formation

-soft tissue extension demonstrated on SPECT

-MRI clearly defines marrow involvement on T1 and soft tissue component on T2

Secondary Osteosarcoma

-paget's disease

-ollier's disease

-ionizing radiation

-polyostotic fibrous dysplasia

-hereditary multiple exostosis

Chondrosarcoma

primary and secondary forms; secondary is due to complication of preexisting skeletal abnormality such as an osteohchondroma or enchondroma; may be central or peripheral

Chondrosarcoma clinical features:

-40-60 years old (typically >50)

-male to female ratio = 2:1

-development of a large soft tissue mass

-late development of pain

-duration of symptoms

Common locations of a chondrosarcoma:

-proximal humerus

-neck of femur

-ribs, sternum

-pelvis

Radiographic features of a chondrosarcoma:

-round or oval area of demineralization with ill defined margins

-endosteal scalloping from pressure erosion

-popcorn matrix calicification in 2/3 with 1/3 being purely lytic

-large soft tissue mass

-calcifications can be popcorn, fluffy, stippled, cotton wool, rings, broken rings

Ewing's Sarcoma

-primitive primary. malignant tumor composed of tumor cells derived from the connective tissue framework of bone marrow

-4th MC primary malignant bone tumor overall

-2nd MC primary malignant bone tumor in children

-round cell tumor

-caused by chromosomal translocation (11 and 22)

Clinical features/age and sex of Ewing's Sarcoma:

-MC in 10-25 year olds with a peak at 15 years old

-male to female ratio = 2:1

Clinical features/signs and symptoms of Ewing's Sarcoma

-severe localized pain

-palpable soft tissue mass

-fever, secondary anemia, leukocytosis, and increased ESR simulating infection

True or False? Ewing's Sarcoma is the only primary malignant tumor that simulates an infection

true

Ewing's sarcoma skeletal locations:

-long tubular bone (60%) (femur, tibia, humerus, fibula)

-DIAPHYSEAL is classic location

-flat bone involvement (40%)

Radiographic features of Ewing's sarcoma:

-8 to 10cm long lytic lesions in the shaft of long bone

-moth eaten to permeative lesion

-long zone of transition

-ONION SKIN/LAMINATED periosteal reaction

-cortical saucerizatoin

-soft tissue formation

-skeletal metastasis

-lung metastasis

Cortical Saucerization

erosion of outer cortex due to soft tissue extension of tumor; can be present in ewing's sarcoma

Ewing's sarcoma is the MC primary malignancy to metastasize to ____

bone

Treatment for Ewing's sarcoma:

amputation, radiation and chemotherapy with 60-75% 5 year survival