Bone tumors

Bone CancerNCCN Review

AJCC Staging

Surgical staging

Overview

• Primary bone cancers make up less than 0.2% of all cancers.• In 2014, an estimated 3020 people were diagnosed in the US and

1460 will die from the disease.• Osteosarcoma (35%), chondrosarcoma (30%) and Ewing’s sarcoma

(16%) are the three most common forms of bone cancer.• Rare: High grade undifferentiated pleomorphic sarcoma (UPS) of

bone, fibrosarcoma, chordoma and giant cell tumor of bone (GCTB).

Diagnostic workup

Chondrosarcoma• These characteristically produce cartilage matrices from neoplastic tissue

devoid of osteoid• More common in older adults• Pelvis and proximal femur are most common sites• Conventional chondrosarcoma of the bone makes up 85% of all

chondrosarcomas• It is divided as follows:1. Primary or central lesions arising from normal appearing bone preformed

from cartilage2. Secondary or peripheral tumors arising from pre-existing benign cartilage

lesions such as enchondromas or cartilaginous portion of osteochondroma

Overview of clinical characteristics and therapeutic options in all subtypes of chondrosarcoma of bone.

Gelderblom H et al. The Oncologist 2008;13:320-329

©2008 by AlphaMed Press

Histology of grade I, II, and III chondrosarcoma.



Histology of rare chondrosarcoma subtypes.



Radiological presentation of a conventional low-grade chondrosarcoma.



Flowchart of the surgical management of central and peripheral chondrosarcoma.



Flowchart of the surgical management of local recurrence in central chondrosarcoma.



NCCN Recommendations

• Histologic grade and tumor locations are the most important variable determining choice of primary treatment.

• Wide excision or intralesional excision with or without adjuvant should be considered for resectable low grade and intracompartmental lesions.

• Wide excision is preferred treatment for pelvic low grade chondrosarcomas.• High grade (grade II, III), clear cell or extracompartmental lesions, if

resectable, should be treated with wide excision with negative margins.• Postop treatment with proton and/or photon beam RT may be used in

chondrosarcomas of skull base and axial skeleton.• RT may be considered in unresectable cases or for palliation.

Chondrosarcomas

Chordoma• They arise from embryonic remnants of notochord• More common in older adults• Predominantly arise in the axial skeletal with the sacrum (50-60%), skull base (25-

35%) and spine (15%)• Classified into 3 variants: conventional, chondroid and dedifferentiated• Conventional: most common, lack cartilaginous or mesenchymal components• Chondroid present with cartilage remnants and features of chordoma• Dedifferentiated chordomas have features of high grade pleomorphic spindle cell

soft tissue sarcoma, follow aggressive course• Chordomas of spine and sacrum present with localized deep pain or

radiculopathy, cervical ones cause airway obstruction or dysphagia or present with oropharyngeal mass

Chordomas

Ewing’s sarcoma

• Ewing’s sarcoma is the second most frequent primary malignant bone cancer, after osteosarcoma.

• It is a small round-cell tumor typically arising in the bones.• It is slightly more common in boys (55:45 male:female ratio). • Most common age of diagnosis is the second decade of life, although

20%–30% of cases are diagnosed in the first decade.

Localization

• Ewing’s sarcoma demonstrates a predilection for the trunk and long bones.

• In the truncal skeleton, the pelvis predominates, followed by the scapula, vertebral column, ribs and clavicle.

• Of the long bones, the most common site is the femur, followed by the humerus, tibia and bones of forearm in that order.

Localization

• As opposed to osteosarcoma, Ewing’s sarcoma of the long bones tends to arise from the diaphysis rather than the metaphysis.

• Ewing’s sarcoma has a strong potential to metastasize.• Metastases most commonly occur in the lungs and bone.• More than 10% of patients present with multiple bone

metastases at initial diagnosis. While metastases in the lungs, bone, bone marrow, or a combination thereof are detectable in approximately 25% of patients, metastases to lymph nodes are rare.

• Extra-skeletal Ewing’s sarcomas present with rapid growth and frequent distant metastases, similarly to Ewing’s sarcoma of bone.

Symptoms

Ewing’s sarcoma typically progresses quite rapidly. Skeletal lesions typically progress to large tumors that form in soft tissues within a few weeks.The earliest symptom is pain. At first, the pain can be intermittent and mild, but rapidly progresses to the point at which it becomes so intense as to require the use of analgesic drugs.When the tumor is vertebral or pelvic in origin, the pain may be accompanied by paresthesia and treated by irradiation.

Symptoms

• Pain often does not completely disappear during the night• Pain without defined trauma adequate to explain the symptoms,

lasting longer than a month, continuing at night, or with any other unusual features should therefore prompt early imaging studies.

Histologic and immunohistochemical features of Ewing’s sarcoma/pPNET.

Bernstein M et al. The Oncologist 2006;11:503-519


The reciprocal translocation between chromosomes 11 and 22 results in the formation of an ews-fli1 fusion gene on the abnormal chromosome 22 that codes for a chimeric transcription factor with the N-terminal transcriptional regulatory domain deriving from ews and the ets-

specific DNA-binding domain derived from fli1.



Imaging Evaluation of Ewing Sarcoma

Imaging in Ewing sarcoma can help to :(a) detect and accurately assess the extent of

disease prior to treatment.(b) evaluate for the presence of metastatic or

recurrent disease.• (c) monitor therapy response.

Imaging Evaluation of Ewing Sarcoma

• Conventional radiography and magnetic resonance (MR) imaging of the primary tumor.

• Tomography (CT) to evaluate for pulmonary metastases.

• Bone scintigraphy to identify osseous metastases.

DIAGNOSTIC IMAGING

• PLAIN RADIOGRAPH :

• The initial imaging investigation of a suspected bone tumor is a radiograph in two planes. Tumor-related osteolysis and periosteal reactions suggest a diagnosis of primary malignant tumor.

Periosteal reactions, the reactive osteogenesis of the periosteum, are caused by extra-osseous extension of the tumor.

DIAGNOSTIC IMAGING

Several types of periosteal reactions have been observed: an ‘onion skin’ or ‘onion-peel appearance’ is a prominent

multi-layered reaction, a ‘sunburst’ or ‘spiculae’ pattern is a perpendicular

reaction, while ‘Codman’s triangle’ is a triangular lifting of the periosteum from the bone at the site of detachment.

Typically, Ewing’s sarcoma appears as an ill-defined, permeative, or focally moth-eaten, destructive intramedullary lesion accompanied by a periosteal reaction (‘onion skin’) that affects the diaphyses of long bones.

The sunburst type of periosteal reactions can present, but is less common in comparison with its occurrence in osteosarcoma.

MRI

• The most precise definition of the local extent of bone tumors, including the degree of expansion into the intra medullary portion and the relationship of the lesion to adjacent blood vessels and nerves, is provided by MRI.

• When malignant bone tumors are suspected, MRI is routinely performed for staging and surgical planning.

• MRI is particularly important in the imaging of Ewing’s sarcoma.

MRI

MRI typically demonstrates lesions that involve large segments of the intramedullary cavity, which extend beyond the area indicated by plain radiographs.

MRI can also evaluate the extent of soft tissue masses, which can be quite large. MRI is widely used to assess responses to neoadjuvant chemotherapy or irradiation, because regression of the extra skeletal tumor

mass can be precisely defined. Currently, MRI is the standard imaging method for such evaluation. Recent

studies have demonstrated, however, that PET, thallium-201 scintiography and dynamic MRI provide more valuable information than MRI for assessment of therapeutic responses.

Staging investigations at diagnosis.



Prognostic Factors

• Favorable: • Distal site of primary disease• Tumor volume <100 ml• Normal LDH at presentation• Absence of metastatic disease at presentation

ESFT in spine and sacrum has worse prognosis.

Enneking classification of surgical intervention.



Ewing’s sarcoma

Giant cell tumor of the bone

• 10 bone neoplasm• Generally benign• Potential for :

• Recurrence• Pulmonary metastasis• Frank malignancy

Epidemiology

• 5-10% 10 bone tumors• 20% benign bone tumors• F : M 1.5 : 1• 70-80% age 20-40• Epiphyseal

Incidence

• Ends of long bones• >50% about knee• High recurrence rate • 1-2% benign pulm. Mets• 10 malignant GCT <1%• Rare polyostotic form <1%

Location

Presentation

• Pain x wks. – mos.• Swelling• Mass• Pathologic # • Neuro deficit (spine / sacrum)• incidental

Radiology

• Lytic lesion• Epipyseal• Eccentric or central• Narrow zone transition• Cortical thinning• expansile• No sclerotic margin

Imaging

• Occ. Cortical breakthrough• +/- soft tissue mass

• Extend to subarticular cortex• Typically no host response• Often large @ presentation

Other modalities

• CT• Integrity cortical rim

• MRI• Assess subchondral breakthrough

• Bone Scan• Suspect multicentri loci• ie. HAND

Histology

• Fibrohistiocytic origin • Multinucleated giant cells• Mononuclear stroma

• Round / ovoid / spindle

• Indistinct cell membrane• Giant cells 20 fusion stromal cells

http://www.kumc.edu/instruction/medicine/pathology/ed/ch_26/c26_s41.jpg

Enneking Staging

Stage 1 Stage 2 Stage 3

Pt % 10-15% ~70% 10-15%

Symptoms asymp pain pain

Radiograph sclerotic rim

expanded cortex

corticalperforation

Histology benign benign benign

Biopsy

• Necessary for Dx• Tumor principles• Histologic grade not helpful• R/O 10 malignant GCT• Occ assoc.

• ABC• Pagets

Tx

• Traditionally:• Intralesional curettage / resection & bone graft• Recurrence 35-42%

• En Bloc resection• Recurrence ~10%• Multiple complications

• Adjuvant

Curettage

• Wide decortication (windowing)• Curettage / high speed burr• Aggressive• Choice of adjuvant

Adjuvant Tx

• Radiation - ~10% sarcomatous degeneration• PMMA, Liquid N2, Phenol, CO2 laser, Electrocautery

• Local extension of margin• Kill residual foci

PMMA

• Fill tumor cavity• Heat kill of tumor cells?• Effect size dependent• 8-26% recurrence• Easy recurrence detection• Degenerative changes

Subchondral bone grafting

Cryotherapy

• 3 freeze thaw cycles• Irrigate cartilage with cool saline• Circumferential necrosis• “difficult”• Complications

• Soft tissue injury• Late fractures

Phenol

• Wash cavity• Alcohol rinse• 10-20% recurrence

Enbloc Resection

• Expendable bones• Prox fibula / Distal ulna

• High recurrence with other Tx• Hand / Distal radius

• Recurrence• Pathologic #• Joint involvement• Osteochondral allograft reconstruction

Spine

• < 3% vertebrae above sacrum• All levels affected equally• Affects vertebral body c ext. pedicle• Resection with stabilization• Often incomplete• ?radiation as adjuvant (low dose 3000 Gyc)

• Incomplete excision• Local recurrence

Sacrum / Pelvis

• Intalesional excision• Adjuvant• +/- radiation

Pelvis

• GCT often vascular• Pre-op angiography• ? embolization

Angiography

Giant cell tumor of the bone

• Classic X-ray findings:1. Codman's triangle (periosteal elevation)2. Sunburst pattern3. Bone destruction

Osteosarcoma

Codman's triangle

Osteosarcoma

Osteosarcoma

Bone cancer chemotherapy

Bone tumors

Health & Medicine

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