Tumors of BoneTumors of Bone

Patrick Henderson, M-IVPatrick Henderson, M-IV

USCSOM USCSOM

August 28, 2007August 28, 2007

IntroIntro Bone tumors are very diverse in morphology Bone tumors are very diverse in morphology

and biological potential (can be no big deal or and biological potential (can be no big deal or rapidly fatal)rapidly fatal)

MOST bone tumors are benign lesionsMOST bone tumors are benign lesions Most benign lesions are seen <30 years of ageMost benign lesions are seen <30 years of age A new bone tumor in the elderly is more likely A new bone tumor in the elderly is more likely

to be malignantto be malignant No bone is safe (though most primaries are in No bone is safe (though most primaries are in

long bones)long bones) Locale in the bone gives important Dx infoLocale in the bone gives important Dx info More common benign lesions typically present More common benign lesions typically present

as incidental findings (non-painful, stable size)as incidental findings (non-painful, stable size) Be cautious with painful lesions and those that Be cautious with painful lesions and those that

grow relatively fast (over weeks or months)grow relatively fast (over weeks or months) Pathological fracture can be the first sign of Pathological fracture can be the first sign of

tumortumor

Bone neoplasms are very difficult to Bone neoplasms are very difficult to diagnose specifically on radiologic diagnose specifically on radiologic testing alonetesting alone

So why is radiology important?So why is radiology important? Exact location of lesionExact location of lesion Extent of growth/metastasisExtent of growth/metastasis AggressivenessAggressiveness

Best test for Dx= X-rayBest test for Dx= X-ray Best test for staging= CT or MRIBest test for staging= CT or MRI Quick shout out to the pathologists– Quick shout out to the pathologists–

histologic grade is the histologic grade is the most importantmost important prognostic feature of bone sarcomas prognostic feature of bone sarcomas and essential for staging most of the and essential for staging most of the bone tumor types.bone tumor types.

CasesCasesFind the lesionFind the lesion

Example:

CasesCasesFind the lesionFind the lesion

Example:

RIGHT THERE!

Case ICase I

16 yr old white male with pain in his 16 yr old white male with pain in his left upper arm.left upper arm. Mild swelling and tendernessMild swelling and tenderness Pain progressively getting worse for ~ 3 Pain progressively getting worse for ~ 3

monthsmonths Recent onset of mild feverRecent onset of mild fever

Imaging:Imaging:

Imaging:Imaging:

**

Biopsy material showed a highly cellular, infiltrative neoplasm consisting of sheets of tightly packed, round cells with very scant cytoplasm ("round blue cell tumor"). Occasional Homer-Wright rosettes were identified. Other fields showed extensive necrosis.

Dx: Ewing’s Sarcoma (or Dx: Ewing’s Sarcoma (or PNET)PNET)

#2 primary bone malignancy in kids #2 primary bone malignancy in kids (5-15 is most common age group(5-15 is most common age group

Much more common in CaucasiansMuch more common in Caucasians Typically in the diaphysis of long Typically in the diaphysis of long

tubular bones or in large flat bonetubular bones or in large flat bone Lytic tumor w/ permeative margins Lytic tumor w/ permeative margins

extending into the soft tissueextending into the soft tissue Periostial rxn creates sheets of Periostial rxn creates sheets of

reactive bone in an reactive bone in an onion-skinonion-skin fashionfashion

Another most excellent example of “onion-skinning”

Case IICase II

33 yr old black female with sudden 33 yr old black female with sudden severe hand pain after very minor severe hand pain after very minor trauma.trauma. Completely healthy otherwise.Completely healthy otherwise. All labs normalAll labs normal

Dx: EnchondromaDx: Enchondroma Benign cartilagenous tumors but hard to Benign cartilagenous tumors but hard to

distinguish from a low grade distinguish from a low grade chondrosarcomachondrosarcoma

Acral bones-- the most common primary Acral bones-- the most common primary hand tumorhand tumor

Usually solitary, usually incidental Usually solitary, usually incidental finding (non-painful unless associated finding (non-painful unless associated with fracture)with fracture)

Get hand films and look for dec. lucency Get hand films and look for dec. lucency but not so much as a cyst (more ground-but not so much as a cyst (more ground-glass) w/ or w/o areas of stippled glass) w/ or w/o areas of stippled calcifications or rings calcifications or rings

For boards and wards:For boards and wards:

Multiple enchondromas = Multiple enchondromas = ________________________

Multiple enchondromas + Multiple enchondromas + hemanigiomas of soft tissue = hemanigiomas of soft tissue = __________________________

For boards and wards:For boards and wards:

Multiple enchondromas = Multiple enchondromas = Ollier’s DzOllier’s Dz Multiple enchondromas + Multiple enchondromas +

hemangiomas of soft tissue = hemangiomas of soft tissue = Maffucci syndromeMaffucci syndrome

Case IIICase III

50 yr old white male with back pain 50 yr old white male with back pain Mainly lower spine/sacral pain, Mainly lower spine/sacral pain,

progressive ~ 8 monthsprogressive ~ 8 months New onset rectal pain and constipationNew onset rectal pain and constipation

CT guided FNA CT guided FNA confirmed…confirmed…

Dx: ChordomaDx: Chordoma

Arises from notochord remnants. Thus is Arises from notochord remnants. Thus is typically midline along the spine and typically midline along the spine and usually at the ends (Sacrococc or usually at the ends (Sacrococc or occ/cervical jxn)occ/cervical jxn)

Males>Females, middle ageMales>Females, middle age + staining w/ S-100 and epithelial + staining w/ S-100 and epithelial

markersmarkers Locally invasive until very late in disease Locally invasive until very late in disease

where mets can go to the lungs, LN, skin.where mets can go to the lungs, LN, skin.

Case IVCase IV

21 yr old male with new onset chest 21 yr old male with new onset chest pain today, worse on inhalation. pain today, worse on inhalation. ROS significant for an ongoing aching ROS significant for an ongoing aching

leg pain for the past 6 months which he leg pain for the past 6 months which he has put off seeing a doctor for.has put off seeing a doctor for.

Dx: The dreaded Dx: The dreaded OsteosarcomaOsteosarcoma

#1 primary bone malignancy#1 primary bone malignancy Associated with RB1 and p53 gene Associated with RB1 and p53 gene

mutationsmutations 1000x greater risk w/ Hx of hereditary 1000x greater risk w/ Hx of hereditary

retinoblastomaretinoblastoma Member of the Li-Fraumeni Syndrome familyMember of the Li-Fraumeni Syndrome family

Bimodal age spike: young and elderlyBimodal age spike: young and elderly 75% <age 2075% <age 20 Osteosarcoma in elderly usually from Osteosarcoma in elderly usually from

predisposing mechanism (secondary)predisposing mechanism (secondary) Paget Dz, bone infarcts, history of radiation, etcPaget Dz, bone infarcts, history of radiation, etc

Most patients die from pulmonary complications after metastatic seeding of the lungs (ex: pneumothorax)

Metaphysial tumorMetaphysial tumor 60% at the knee (distal femur or prox 60% at the knee (distal femur or prox

tibia)tibia) Radiographic terms to know:Radiographic terms to know:

Codman’s Triangle: Codman’s Triangle:

““Sunburst” periostial formation:Sunburst” periostial formation: AKA “Hair on end”AKA “Hair on end”

For the future Surgeons:For the future Surgeons: Rotationplasty is a new solution to Rotationplasty is a new solution to

disfiguring surgical resections of disfiguring surgical resections of lower limb sarcomas:lower limb sarcomas:

Quick Hits:Quick Hits:

Gout

IncidentaIncidental finding l finding on knee on knee xrayxray

Fabella = posterior sesmoids or little confused knee caps

13 yr old boy with superior tibial pain, r/o neoplasm w/ xray shows:

Osgood Schlatter

Metastatic DiseaseMetastatic Disease

Most common malignant lesion of boneMost common malignant lesion of bone Bone is # 3 on the list of favorite places for Bone is # 3 on the list of favorite places for

mobile cancers to gomobile cancers to go Malignant lesions are more likely to be in axial Malignant lesions are more likely to be in axial

bonesbones Typically multifocal BUT renal and thyroid Typically multifocal BUT renal and thyroid

carcinomas are notorious for producing only a carcinomas are notorious for producing only a solitary lesionsolitary lesion

Can be lytic, blastic, or both:Can be lytic, blastic, or both: LLung is ung is LLytic, ytic, PProstate rostate PProduces, roduces, BBreast does reast does BBothoth

Mets (cont)Mets (cont)

AdultsAdults LungLung ProstateProstate BreastBreast KidneyKidney

KidsKids NBNB Wilm’sWilm’s OSOS Ewing’sEwing’s RhabdomyosarcomRhabdomyosarcom

aa

The EndThe End

Thanks for your attention and good luck on applications!

BibliographyBibliography Robbin’s and Cotran, Robbin’s and Cotran, Pathological Basis of Disease, 7, 7thth

EditionEdition MD Murphey, MR Robbin, GA McRae, DJ Flemming, HT MD Murphey, MR Robbin, GA McRae, DJ Flemming, HT

Temple, and MJ KransdorfTemple, and MJ KransdorfThe Many Faces of Osteosarcoma The Many Faces of Osteosarcoma RadioGraphics 1997; 17: 1205 RadioGraphics 1997; 17: 1205

William R. Reinus, Louis A. Gilula IESS CommitteeWilliam R. Reinus, Louis A. Gilula IESS CommitteeRadiology of Ewing's sarcoma: Intergroup Ewing's Radiology of Ewing's sarcoma: Intergroup Ewing's Sarcoma Study (IESS)Sarcoma Study (IESS)RadioGraphics 1984; 4: 929-944. RadioGraphics 1984; 4: 929-944.

Washington Univ. in St. Louis (website)Washington Univ. in St. Louis (website) Harvard Medical School (website)Harvard Medical School (website) Learning Radiology.com (website… duh)Learning Radiology.com (website… duh) Bonetumor.org (You’re not even reading this are you?)Bonetumor.org (You’re not even reading this are you?)