Neoplasms of the Musculoskeletal System - Clin Med

What does this refer to

• 68-year-old man presents to his primary care physician for left leg pain.

• His pain has been progressively worsening over the course of a month and is present at night.

• Physical examination demonstrates impaired left lower extremity weakness limited by pain.

• A radiograph of the left hip and femur demonstrates lytic lesions and intralesional calcifications.

Chondrosarcoma

What does this refer to

• Chondrosarcoma is defined as a cancer of the cartilage

• Central metaphyseal

Chondrosarcoma

What does this refer to

• MC 40-75yo

• M > F

• 3rd MC primary malignancy of bone

EpidemiologyChondrosarcoma

What does this refer to

• Malignant cartilage forming tumor that does not produce osteoid

• May arise from osteochondroma

• MC bone location

  • Proximal femur

  • Pelvic bones

  • Proximal humerus

  • Ribs

EtiologyChondrosarcoma

What does this refer to

• Pain that gets worse over time and with physical activity

• Swelling, stiffness, and tenderness at the affected area

• Back or thigh pain

• Sciatica-like sx

• Bladder sx

• Unilateral edema

Clinical historyChondrosarcoma

What does this refer to

• Tenderness to palpation at the site of involvement

• Neurologic dysfunction if there are pelvic lesions close to the neurovascular bundle

• Pearly white or light blue, often with focal calcification

• May have small cysts or myxoid change

Clinical presentation/physical examChondrosarcoma

What does this refer to

• Histopathology = Malignant chondrocytes in gelatinous cartilaginous matrix

WorkupChondrosarcoma

What does this refer to

• Radiograph shows popcorn leisions!!!

Chondrosarcoma

What does this refer to

• Consult/referral Orthopedic/Radiation Oncology

• Surgical

  • Intralesional curettage (low grade)

  • Wide en bloc local excision (intermediate-high grade)

Clinical interventionChondrosarcoma

What does this refer to

• Morbidity/Complications

  • Pathologic fractures

  • Metastasis (low probability)

  • Referral to orthopedic oncology for best prognosis

• Poor Prognostic Variables

  • More aggressive course

    • Axial and proximal extremity lesions

    • Advanced patient age

    • Inadequate surgical margins

• Patients may have local recurrence or metastases up to 20 years later

• 5-year survival rate for adult bone cancer is 66%

• Adults with chondrosarcoma have a 5-year survival rate of 80% compared to a 5-year survival rate of 54% for osteosarcoma.

• If metastatic disease 5-yr survival rate is 55%

PrognosisChondrosarcoma

What does this refer to

• Bone is 3rd MC site for metastatic disease

• Carcinomas that commonly spread to bone

  • Breast

  • Lung

  • Thyroid

  • Renal

  • Prostate

Metastatic Cancer to Bone

What does this refer to

• Metastatic bone lesions MC > 40 yo

• Most common sites of metastatic lesions

• Axial skeleton _ vertebrae, pelvis, ribs

• Proximal limb girdle

  • Proximal femur MC of fx secondary to metastatic bone lesions

EpidemiologyMetastatic Cancer to Bone

What does this refer to

• Metastatic bone destruction

  • Tumor induced activation of osteoclasts

EtiologyMetastatic Cancer to Bone

What does this refer to

• Pain

  • Bone due to destruction

  • Tumorigenic

    • Worse at night

• Pathologic fx

  • Up to 30% of patients

Clinical historyMetastatic Cancer to Bone

What does this refer to

• Muscle weakness if malignant hypercalcemia

Physical examMetastatic Cancer to Bone

What does this refer to

WorkupMetastatic Cancer to Bone

What does this refer to

• Radiation therapy

  • Breast

  • Prostate

  • MM

  • Lymphoma

• Operative

  • Arthroplasty with adjuvant radiation

  • Spinal neurologic decompression/stabilization

    • + post-op radiation

    • Indicated with mets to spine

Clinical interventionMetastatic Cancer to Bone

What does this refer to

• Bisphosphonate therapy

• Dexamethasone

  • Spine mets

Clinical managementMetastatic Cancer to Bone

What does this refer to

• Metastatic hypercalcemia

  • confusion

  • polyuria & polydipsia

  • nausea/vomiting

  • Dehydration

• Physical Exam

  • Muscle weakness

ComplicationMetastatic Cancer to Bone

What does this refer to

• Survival rate

• Thyroid – 48 months

• Prostate – 40 months

• Breast – 24 months

• Kidney – varies

  • As little as 6 months

• Lung – 6 months

PrognosisMetastatic Cancer to Bone

What does this refer to

• A 12-year-old boy presents to the pediatric emergency department with pain in his right lower extremity.

• He was recently playing in soccer, but he denies any trauma to the leg.

• Physical examination is notable for mild swelling in the right mid-tibia and tenderness to palpation.

• A radiograph of the leg demonstrate periosteal elevation and "onion skinning" concerning for malignancy.

Ewing sarcoma

What does this refer to

• Cancerous tumor that grows in the bones or in the tissue around bones (soft tissue)

  • legs, pelvis, ribs, arms or spine

• Includes

  • Ewing sarcoma

  • Askin tumor

  • Peripheral neuroectodermal tumors

Ewing sarcoma

What does this refer to

• MC birth to age 20

• M > F

• 9x > Caucasian v AA

EpidemiologyEwing sarcoma

What does this refer to

• Genetic

• t(11:22) translocation

• Specific cause for the transformation hasn’t been identified

EtiologyEwing Sarcoma

What does this refer to

• Patients usually present with pain

• Patients often have a palpable mass

• Back pain may indicate a paraspinal, retroperitoneal, or deep pelvic tumor

• Systemic symptoms of fever and weight loss can also occur and often indicate metastatic disease

Clinical HistoryEwing Sarcoma

What does this refer to

• Fever

• Weight loss

• Diaphysis of Long bones

  • Upper arm

• Flat bones

  • Pelvis

  • Skull

  • Ribs

• Palpable mass

• Local tenderness

• Joint swelling

Physical examEwing Sarcoma

What does this refer to

Physical examEwing Sarcoma

What does this refer to

WorkupEwing Sarcoma

What does this refer to

• Ewing sarcoma or another tumor is probable

  • Consultation with a pediatric oncologist BEFORE biopsy

  • Biopsy is required for definitive diagnosis

• Histology

  • Small, round blue cell tumors

    • Differentiated or undifferentiated

• Eval for Mets includes bilateral bone marrow biopsies

BiopsyEwing Sarcoma

What does this refer to

Clinical managementEwing Sarcoma

What does this refer to

• Alopecia, nausea, vomiting, occasionally diarrhea

• Increased risk of infection from immune suppression

• Change in mood/appetite

  • Monitor Nutritional and psychological status

• Side Effects of chemotherapy

  • Organ damage

  • Infertility

  • Risk of secondary malignancy

Chemotherapy ComplicationsEwing Sarcoma

What does this refer to

• Most significant factor to determine prognosis is presence or absence of metastatic disease

• Primary tumor site also a factor

  • Distal extremities more favorable

• Age younger than 15yr more favorable prognosis

PrognosisEwing sarcoma

What does this refer to

• Osteogenic bone tumor

• MC bone Locations

• Metaphysis of long bones

  • Distal femur

  • Proximal tibia

• Distant Mets

  • MC lungs

  • Can also mets to same bone or different bone

Osteosarcoma

What does this refer to

• More Common in Adolescents

• Osteosarcoma is the 5th most common malignancy among adolescents 15-19

• Bimodal distribution with 2nd peak in 50-60 yo

• M > F

• In children – MC site is LE

• In adults – MC site is axial

• In adults – greatest risk factor is Hx of Paget’s disease (benign bone lesion)

EpidemiologyOsteosarcoma

What does this refer to

• Bone pain is MC complaint (Long Bone)

• Pathologic fx usually absent

• Pain may result in a limp, loss of function

• +/- swelling

Osteosarcoma

What does this refer to

• Palpable mass

• ↓ ROM in affected bone/joint

• Motion/activity increases pain

Physical examOsteosarcoma

What does this refer to

WorkupOsteosarcoma

What does this refer to

Clinical managementOsteosarcoma

What does this refer to

PrognosisOsteosarcoma

What does this refer to

ComparisonOsteosarcoma – Ewing Sarcoma

What does this refer to

• ________ is a type of cancer that occurs in (lipocytes)fat cells

  • Muscles of the limbs or the abdomen

  • Rare cancer

  • Type of soft tissue sarcoma

• Slow growing

Liposarcoma

What does this refer to

• Most common soft tissue worldwide

• Only 20% of soft tissue cancers in the US are a liposarcoma

• Don’t confuse this with a lipoma

• Average age of dx is 50 yo

• No race of gender predilection

EpidemiologyLiposarcoma

What does this refer to

• Risk factors

  • Radiation therapy

  • Family cancer syndromes

  • Damage/trauma to the lymphatic system

  • Toxic chemical exposure

EtiologyLiposarcoma

What does this refer to

• Types of liposarcoma

  • Well differentiated & dedifferentiated liposarcoma

  • Myxoid and round cell liposarcoma

  • Pleomorphic liposarcoma

PathologyLiposarcoma

What does this refer to

• History and physical exam findings depend on location of the tumor

• Paresthesias

• Varicose veins

• Fatigue

• Weight loss

• N/V

• When in the abdomen – dysphagia & regurgitation of food

Clinical historyLiposarcoma

What does this refer to

• Most are asx

• Pain/tenderness

• Edema

• Functional loss

• Deep mass in the lower extremity

  • Often non-tender

Physical examLiposarcoma

What does this refer to

WorkupLiposarcoma

What does this refer to

• Surgical excision

  • Wide and deep

• Adjuvant radiation (possibly with chemo) for high-grade lesions

Clinical interventionLiposarcoma

What does this refer to

• Strong correlation with histology subtypes, tumor grade, location and status of surgical margins

PrognosisLiposarcoma

What does this refer to

• Subtypes

  • Embryonal

    • MC infants/young children

  • Alveolar

    • MC Adolescents/young adults

  • Botryoid

    • MC infants/young children

  • Pleomorphic

    • MC patients 40-70 yo

• Nodal metastasis often occurs with rhabdomyosarcoma

  • Bx of sentinel node should be part of the treatment

  • Bone marrow bx required for staging

EpidemiologyRhabdomyosarcoma

What does this refer to

• Orbit: Proptosis or dysconjugate gaze

• Paratesticular: Painless scrotal mass

• Prostate: Bladder or bowel difficulties

• Uterus, cervix, bladder: Menorrhagia or metrorrhagia

• Vagina: Protruding polypoid mass (botryoid, meaning a grapelike cluster)

• Extremity: Painless mass

• Parameningeal (ear, mastoid, nasal cavity, paranasal sinuses, infratemporal fossa, pterygopalatine fossa): Upper respiratory symptoms or pain

Clinical historyRhabdomyosarcoma

What does this refer to

RhabdomyosarcomaHead and Neck

What does this refer to

• Cranial Nerve Palsies

• Hearing Loss

• Chronic aural or sinus drainage

• Image shows PARAMENINGEAL

Physical examRhabdomyosarcoma – Head and Neck

What does this refer to

WorkupRhabdomyosarcoma

What does this refer to

• Localized disease, overall 5-year survival rates have improved to more than 80% with the combined use of surgery, radiation therapy, and chemotherapy

PrognosisRhabdomyosarcoma

What does this refer to

• ___________ are tumors that effect the cartilage inside the bones

• MC benign bone neoplasm of the hand, but can effect other areas

Enchondroma – Benign Bone Tumor

What does this refer to

• Typically asymptomatic unless accompanied with injury such as a fracture

• Symptoms

  • Pain

  • Changes in growth

  • Abnormal exam

    • Affected area may enlarge

Clinical history/physical examEnchondroma

What does this refer to

• Bone infarct

• Chondrosarcoma

Differential diagnosisEnchondroma

What does this refer to

• Core needle bx

  • From areas of bone scalloping or lysis

• Histology

  • Blue-gray lobulated hyaline cartilage with scattered calcifications

WorkupEnchondroma

What does this refer to

WorkupEnchondroma

What does this refer to

Clinical managementEnchondroma