Malignant Bone Tumors Flashcards
Periosteum
Thick, fibrous membrane covering outer surface of the bone; serves as attachment for muscles and tendons
Endosteum
Layer of cells lining the inner surface of bone in the central medullary cavity
Marrow cavity
Contains the bone marrow in the diaphysis
Nutrient foramen
External opening for entrance of blood vessels in a bone
Red bone marrow
Marrow containing the developmental stages of erythrocytes, leukocytes, and is megakaryocytic. Gradually replaced by yellow marrow
Yellow bone marrow
Storage layer, reticular network filled with fat. Contains abundant capillaries and specialized lipid storage tissue. Replaces red marrow early in life but can convert to haemotopoetic red marrow in severe blood loss
Compact (cortical) bone
Dense, rigid outer shell of bones formed from osteons (formed from osteoclasts and osteoblasts)
Spongy (cancellous) bone
Porous, highly vascularized bone containing red bone marrow
Primary bone tumor
Originates in the bone (benign/malignant)
Secondary bone tumor
Originates elsewhere and metastasizes to bone
Chondro
Cartilage
Osteo
Bone
Curettage
Special instruments used to scrape the tumor out of the bone
Bone graft
After curettage, filling the cavity with a bone graft to help stabilize the bone. A bone graft is either taken from a donor (allograft) or from another bone in your body (autograft)—most often the pelvis
Primary Bone Cancer
Tumors of mesenchymal (adult stem cell) origin that reflect skeletal tissues (bone, cartilage, connective tissue) and tumors developing in bones that are hematopoietic, nerve, vascular, fat cell, and notochordal origin.
Can be benign or malignant.
Can grow rapidly and metastasize widely, but more are benign and rarely metastasize
Is Primary Bone Cancer common ?
Primary bone cancer is relatively uncommon in comparison with secondary or metastatic neoplasms
Where do most metastatic bone cancers arise from?
Prostate, breast, kidney, thyroid, and lung.
PbKTL= “lead kettle”
What are risk factors for primary bone cancer?
—Retinoblastoma —Li Fraumeni syndrome —Rothmund Thomas syndrome —Werner syndrome —Pagets disease —Radiation exposure —Fibrous dysplasia
What are the s/sx of primary bone cancer?
Many can be asymptomatic
Pain, usually in the area of the tumor —Persistent —Dull, achy —Worse at night **Hallmark of primary bone tumors** —Increased with activity
Others: —Fever —Night sweats —Injury in the area, possible fracture —swelling —tenderness —skin changes —+/- limited ROM —Fatigue, anorexia, weight loss
What’s the best initial imaging diagnostic study for primary bone cancer?
X rays:
—radiographs are the best initial modality for evaluation of primary bone lesions, viewed in at least 2 planes
—looking for evidence of erosion, bone formation, or fracture
—Primary malignant cancer shows cortical destruction, radiolucency, extraosseous extension, permitting destruction with poorly defined borders
What’s the imaging modality of choice if a malignant bone tumor is suspected in primary bone cancer?
MRI
—Modality of choice when malignant bone tumor suspected
—Provides best contrast resolution for demonstrating soft tissue masses and invasion of adjacent structures
What are the different imaging modalities you can use for suspected primary bone cancer?
X rays
MRI
CT
What does CT do for diagnosing primary bone cancer?
—Better defines location (periosteal, cortex, medullary)
—More accurately evaluate changes (focal destruction or endosteal scalloping)
—Better delineate matrix mineralization
—Better guide therapy
What other diagnostic studies/labs do you get for suspected primary bone cancer?
—Bone scan
—CBC, Urinalysis to R/O other pathology
—Biopsy: either needle biopsy or an open biopsy in operating room
Characteristics of Malignant (or aggressive non malignant) primary bone cancer
Border= Poorly defined
Periosteal reaction= often present e.g spiculated, interrupted, wedge shaped.
—The absence of periosteal rxn does not exclude malignant lesion
Cortical destruction= typical moth eaten or permeative pattern
Extension- Extends into the soft tissues
Characteristics of Non malignant and non aggressive primary bone cancers
Border= well defined or sclerotic border
—sharp zone of transition from normal to abnormal bone
Periosteal rxn= absent
Cortical destruction= usually absent
Extension= Confined by natural barriers like the growth plate or cortex, and does not extend into the soft tissues
What are the 3 primary Malignant bone tumor types?
Osteosarcoma
Chondrosarcoma
Ewings Sarcoma
Primary malignant bone tumors represent how many cancer cases in the US?
0.2% of cases in the US
2,900 cases annually
What is Osteosarcoma?
—The most common primary bone cancer (including children)
—Prevalence is 2-5 people per 1 million each year
—Age: mostly children and teenagers
—Location= most common in the femur and tibia (around the knee) at the growth plates
—Usually appears after a sports related injury
What do Osteosarcoma labs look like?
Elevated serum alkaline phosphatase (broken/remodeled bone)
Can be used to assess response to chemotherapy
How do you confirm a diagnosis of Osteosarcoma?
Biopsy needed to confirm. Shows a “spindle shaped” appearance
What is the treatment for Osteosarcoma?
Chemotherapy and surgical resection
What Are osteosarcoma characteristics on x ray?
SUN RAY or SUN BURST appearance: the formation of new bone
—Presence of Codman triangle: elevation of periosteum from the bone surface
—Cumulus cloud appearance/Cloud like lesions
What is Chrondrosarcoma?
Primary Malignant tumor composed of cartilage producing cells.
Located around the pelvis, proximal femur, hip, or shoulders.
Generally located in the metaphases of long bones
What are the sx of Chondrosarcoma?
Progressive pain, constant, not relieved with rest, and slow growing
How do you diagnose Chrondrosarcoma?
X ray, CT, MRI, and biopsy
Is there a significant difference in 10 year survival rates between low grade and high grade lesions in chondrosarcoma?
Yes
Less than 1% of solitary _______ and ______ dedifferentiate into chondrosarcomas
— enchondromas
—Osteochondromas
What patients have a definable risk for developing secondary chondrosarcomas?
Only patients with HMO (Hereditary Multiple Osteochondromas) have a definable risk for developing secondary chondrosarcoma
What is the treatment for Chondrosarcoma?
Surgery is the only definitive treatment
What is an aggressive variant of chondrosarcoma that is seen in younger patients?
Mesenchymal chondrosarcoma
What does the prognosis of chondrosarcoma depend on?
Prognosis depends on grade and location
Patients with pelvic chondrosarcoma have a poorer prognosis than those in the extremity
What is Ewing’s sarcoma?
The second most common bone tumor in children and young adults> between ages 5 and 20 most commonly.
Unknown cause or type of cell. Known to occur in a mismatching of chromosomes 11 and 22, occurring after birth
What labs are done for Ewings Sarcoma?
Serum lactate dehydrogenase will be elevated
What does a biopsy of Ewing’s sarcoma show?
Round cell tumors
Does Ewing’s sarcoma have a risk of metastasizing?
Yes
What is treatment for Ewing’s sarcoma?
Combination= chemo, radiation, surgery (multiple specialists)
What does an X ray of Ewing’s sarcoma show?
ONION PEEL EFFECT or “onion skinning”
Also bone destruction
What is Multiple Myeloma?
a malignant tumor of the BONE MARROW, where cells come from changed plasma cells. Tends to involve the entire skeleton (infiltrates all bone marrow)
**Typically occurs spontaneously
Named for the clock face appearance of cells under the microscope
What is the prevalence of Multiple Myeloma?
7 people per 100,000 each year
Mostly ages 50s-70s
M>F
2:1 African American: Caucasian
What are risk factors for Multiple Myeloma?
Typically occurs spontaneously but risk factors are —Radiation —Dioxin —HIV —Herpesvirus 8
What are the sx of Multiple Myeloma?
Diffuse bone tenderness in sternum and hips.
Pathologic fractures are common — Spine most common (spinal cord compression)
What is treatment for Multiple Myeloma?
Multiple myeloma is not curable. Chemotherapy may prolong life expectancy and decrease sx
—Melphalan=chemo drug
+ Prednisone
What do multiple myeloma x rays look like?
PUNCHED OUT LESIONS of the bone “Plasma cell myeloma”
What is Fibrosarcoma?
Malignant tumor derived from nonosteoblastic connective tissue.
Majority are high grade
What populations do you see fibrosarcoma in?
Usually older pts >50yo
Infants: Most cases are congenital, almost never after 2 yo
What are sx of fibrosarcoma?
Pain and swelling
What do radiographs of fibrosarcoma show?
Lytic lesion with bone destruction, ill defined features
Hot bone scan
What is the treatment for fibrosarcoma?
Wide surgical resection and chemotherapy if high grade
What is the prognosis for fibrosarcoma?
Poor
High grade 30% 5 year survival
Low grade 80% survival at 10 years
