Management of Cartilaginous Bone Tumors

Prof Harzem Ozger

IU Istanbul Tıp Fakultesi

Ortopedi ve Travmatoloji AD

Ulusal Ortopedi ve Travmatoloji Kongresi

Antalya, 2011

Epidemiology

• Arises from hyaline cartilage

• All age groups

• Most common benign tumor of the bone

• Most common site is the phalanges of the hands

• Most common tumors seen in the small bones of the hands and feet.

How do these tumors present?

1) Pain

2) Pathological fracture

3) Incidental radiological finding

Radiological evaluation

• All imaging modalities contribute in evaluation:• Xray• CT• Scintigraphy• MRI

• Minimum diagnostic procedure (time + cost + radiation) vs. maximum data:

xray + (contrast-enhanced) MRI + scintigraphy ?

Conventional management

• Symptomatic and progressive lesions: extended curettage (intralesional resection) + defect-filling procedure (graft, PMMA)

• Pathological fracture / Imminent pathological fracture: IL resection + defect-filling procedure (graft, PMMA) + osteosynthesis /augmentation

Conventional management

Multiple enchondromatosis (Ollier, Mafucci, metachondromatosis) !!

Multiple lesions : Deformity = corrective osteotomy Risk of malignant transformation = “ Meticulous

radiological follow-up + surgical intervention a.s.a.p. in case of radiological / clinical suspicion”

Impossible to resect all lesions prophylactically. Unacceptable approach because of unnecessary morbidity.

Conventional management

Xray and CT

Proximal location + endosteal erosion involving 2/3 of the cortex

Possibility of transformation to

chondrosarcoma

Urgent biopsy!!

Problems associated with conventional management

• Asymptomatic (incidental) and non-progressive lesions:

• Is the non-progressive lesion really non-progressive? Only way

to prove it is requesting films at regular intervals.

• How often? annual?, twice a year?, quarterly?

• For how long? Lifelong? Until totally calcified?

• Is plain radiography sufficient? Or should we request MRI or

scintigraphy for follow-up?

Pain

• How reliable a criterion is pain for determining surgical indication?

- Clin Orthop Relat Res. 2005 Feb;(431):181-6.

The causes of pain in benign solitary enchondromas of the proximal humerus.

Levy JC, Temple HT, Mollabashy A, Sanders J, Kransdorf M.

• Is the pain caused by the cartilaginous tumor itself, is it referred pain (eg. cervical radiculopathy) or is it due to a pathology of other neighboring structures (rotator cuff tear, patellofemoral, etc. )

• Can enchondroma cause pain or does it only get painful when it transforms into CS grade 1?

• Controversial criterion to decide surgery…

• Although various radiographic criteria have been cited in relation to cartilage tumors of the bone, it is very difficult to decide on surgery according to the x-ray alone.

• Long-term follow-up with frequent visits is recommended.

Plain x-ray

MRI and Scintigraphy

• MRI and scintigraphy cannot differentiate between “cellular enchondroma / GI chondrosarcoma”

• Textbook knowledge:• Diffuse contrast enhancement in early phase of C+

MRI and / or

• Increased radionuclide uptake in comparison to ASIS.

are indications for biopsy …

The lesion is regarded as “latent” in the absence of these findings.

• Well then! How are we going to follow-up this “latent” lesion?

Backwards approach: Pathological criteria???

Am J Surg Pathol. 2009 Jan;33(1):50-7.

Assessment of interobserver variability and histologic parameters to improve reliability in classification and grading of central cartilaginous tumors.

Eefting D, Schrage YM, Geirnaerdt MJ, Le Cessie S, Taminiau AH, Bovée JV,

Hogendoorn PC; EuroBoNeT consortium.

Conclusion:

Differentiation of enchondroma from G1 CS is difficult in central lesions even histopathologically.

Sarcoma. 2011; 2011: 405437.

XRAY + CT+ MRI + Pathology altogether

J Bone Joint Surg Am. 2007 Oct;89(10):2113-23.

Reliability of histopathologic and radiologic grading of cartilaginous neoplasms in long bones.

Skeletal Lesions Interobserver Correlation among Expert Diagnosticians (SLICED) Study Group.

Conclusion:

Even when examined by expert radiologists and expert pathologists, the grade of long bone cartilaginous lesions are assessed with low reliability.

Predicting malignant transformation

1% of solitary cartilage lesions are believed to undergo malignant transformation whereas this rate is predicted as 10-30% in multiple lesions.

It is not yet possible to predict which lesions will demonstrate malignant transformation (or grade progression).

There are numerous studies going on. However, no direct genetic link has been established such as the link between EXT-1/ EXT-2 genes and osteochondromas.. (1,2,3,4)

1. Constitutive Hedgehog Signaling in Chondrosarcoma Up-Regulates Tumor Cell Proliferation. Am J Pathol 2006, 168:321–3302. Enchondromatosis: insights on the different subtypes. Int J Clin Exp Pathol 2010;3(6):557-5693. C-Propeptides of Procollagens Iα1 and II that Differentially Accumulate in Enchondromas versus Chondrosarcomas Regulate

Tumor Cell Survival and Migration. Cancer Res 2010;70:4739-4748.4. PTHR1 mutations associated with Ollier disease result in receptor loss of function. Human Molecular Genetics, 2008, Vol. 17,

No. 182766–2775

Conservative management

Is it superior in terms of cost effectivity?

Best guess: yearly orthopedics and traumatology outpatient visits

Getting an appointment from radiology and/or nuclear medicine each year

Getting the examination done

A follow-up visit with the result of radiological examination.

more convenient?

Surgical treatment

Proper technique!

Surgical approach must be in accordance with the rules of biopsy – eg. no use of elevators.

Rounding off of the corners of the cortical window in order to decrease the risk of iatrogenic fracture

Meticulous resection with curette first and then with high-speed burr.

Surgical treatment

Extended curettage: phenolisation / cautrisation / crysurgery

Grafting/ cementation / neglecting the defect

Implants must be avoided unless there is absolute indication for fixation! Imaging problems Tumor contamination

Results of surgical treatment

Clin Orthop Relat Res. 2010 Oct;468(10):2765-73. Epub 2010 Jun 24.Mohler DG, Chiu R, McCall DA, Avedian RS.

Curettage and cryosurgery for low-grade cartilage tumors is associated with low recurrence and high function.

• Low complication rate ✔

• Local recurrence is low ✔

• Good radiological results ✔

• Functional results are very good ✔

• Surgical treatment is a rational treatment option.…

Why should we intervene with incidental cartilage tumors?

1. Medicolegal issues

Once the lesion is documented, regardless of whether it is symptomatic or incidental, the orthopedist, who requested the examination, has the full responsibility of managing this lesion.

Would you risk missing a malignant lesion in order to avoid a relatively safe and simple procedure?

Would you rather perform a highly morbid procedure with all the risks and complications, in the setting of a lesion, which was being followed up for some time and rapidly progressed all of a sudden?

2. Psychological issues

• Would you rather live with a time-bomb inside if you were the patient?

Is it necessary to screen for asymptomatic lesions?

It is not rational because imaging modalities commonly used for screening of solid lesions are not suitable for this purpose…*

* Update: Whole body MRI is a newer technology developed for check-up. The fact that it does not use radiation is the topmost advantage. It allows a closer look at the detected lesions in the same session. But it is currently very expensive. Its role in screening has not been established.

Istanbul Medical Faculty Enchondroma Experience

1990-2010

119 pts. (low-grade / GI chondrosarcoma pts excluded)

Anatomical site : Femur 49 Metacarpals & phalanges of the hand 24 Humerus 16 Tibia 13 Phalanges of the foot 5

Multiple enchondromatosis in 5 pts.

Treatment

10 pts: lost to follow-up w/out surgery

5 pts: resection: 1 pt. – resection of fibular head 1 pt. – finger amputation (multiple enchondr.) 1 pt. – pathological fx. due to prox femur enchondroma 1 pt. – eccentric superficial lesion in medial femoral 1 pt. – subtotal iliac wing resection (multiple enchondr.)

104 pts: intralesional resection + burr + phenol grafting 53 cement 31 curettage only 20

Internal fixation in only 4 pts. (plate & screw 2 / screw only 1 / IM nail 1)

Curettage + Cementation = Relatively easy surgery +

morbidity

Curettage + Cementation = Relatively easy surgery +

morbidity

Curettage + Cementation = Relatively easy surgery +

morbidity

late diagnosis / delayed intervention = resection + morbidity

WLR + Mutars prosthesis

late diagnosis / delayed intervention = resection + morbidity

late diagnosis / delayed intervention = resection + morbidity

WLR + Finn prosthesis

WLR of local recurrence + Kotz prosthesis

Skip met. + Pathological fx. Ext. hemipelvectomy

Conclusion

“Critical window” for intervening in premalignant lesions should not be missed.

Failure to perform a timely, low morbidity, intralesional intervention for an enchondroma or a low-grade cartilage tumor, which has been documented radiologically, leaves us deprived of the safety of histopathological verification. The fact that some of these lesions will progress to high-grade lesions with highly morbid and fatal consequences is extremely troublesome for the orthopedic oncologist in medicolegal terms.

Thank you for your attention…