ORTHOPAEDIC PATHOLOGICAL SPECIMEN

AND HISTOLOGY

DESCRIBING GROSS SPECIMENA: Identify the part:

Knee / prox. femur/ prox. tibia/ pelvis /scapula.Epiphyseal / Metaphyseal / Diaphyseal.Physeal plate visible? – Immature pt.

B:Describe the abnormality:Nature: solid / soft & friable /cystic / varigatedMatrix: bony / cartilaginous / fibrousSecondary changes: central necrosis/ hemorrhage

/ cystic changeCortical destruction, periosteal elevationIntramedullary spreadTumor margin / capsule: well defined / illdefinedExtension: into soft tissue / joint; invasion /

infiltration to surrounding tissue

C: Give provisional diagnosis.

Ewing’s sarcomaMyelomaOsteoid osteoma

Ewing’s sarcomaMyelomaFibrous dysplasia

Majority

ChondroblastomaGiant cell tumor

Epiphyseal– Chondroblastoma

– Giant cell tumor Diaphyseal

– Ewing’s sarcoma

– Myeloma,

– Fibro sarcoma,

– Reticulum cell sarcoma

– Adamantinoma

– Fibrous dysplasia

– Osteoid Osteoma

Metaphyseal– Most others

EnchondromaUnicameral bone cystRound cell tumors

Majority

Central– Enchondroma

– Unicameral bone cyst

– Round cell tumors Cortical

– Osteoid osteoma Eccentric

– Most other tumors

Age: 20-40.Epiphyseal location (After epiphyseal closure)Common location: around knee (50%),

distal radiusXray: Geographic lytic lesion, thinned &ballooned out cortex.

Gross pathology:Large red - grey - brown tumorSoft & friableAreas of cystic degeneration / necrosis &blood filled cavities

GIANT CELL TUMOR

HISTOPATHOLOGY:•Uniform oval mononuclear stromalcells : mesenchymal origin

•Appear to grow in a syncytium

•Numerous osteoclast-type giantcells: reactive with centrally placed uniform size nuclei (40-100)•Necrosis, hemorrhage, hemosiderindeposition and reactive bone

formation•Relatively few mitotic figures inrelation to the dense cellularity of thetumor

GIANT CELL VARIANTSIncludes the tumors which show giant cells in histology-

A. ABCB. Brown tumorC. Chondromyxoid fibroma, chondroblastomaD. Desmoplastic fibromaE. Epulis – Giant cell reparative granulomaF. Fibrous dysplasia, non ossifying fibromaG. Giant cell rich osteosarcomaH. Benign fibrous histiocytoma

Giant cell……Physiological: Megakaryocyte

PathologicalLanghans : fused epitheliod cells. Peripherally arranged 7-21 nuclei. TB,

histoplasmosis, sarcoidosis, other mycobacteria

Foreign body: fused macrophages. Numerous nuclei scattered incytoplasm

Aschoff : Rheumatic heart disease

Reed Sternberg: Hodgkin’s lymphoma

Touton : xanthoma, xanthogranuloma, dematofibroma

Viral: Warthin Finkeldy (measles), resp syncitial virus, parainfluenza

Tumor

Features of malignant changes :

(1) Size and number of giant cells decrease

(2) Stroma shows : Heterogenicity as : Crowding, Plumping up, Increased chromatin, Increased mitotic figures.

(3) Vascularity increases

Grading of GCT (Jaffe):

  Stromal cells Giant cells

Grade-1 or conventional GCT

Stroma less Large number

Grade-II or Borderline GCT

Prominent cellular atypia

Decrease in number

Grade-III or fully malignant

Sarcomatous stroma Sparse

Stage I

(Latent)

10-15% No symptom, Pathological #s may occur

Stage II

(Active)

70% Benign, symptomatic, Pathological #s may occur

Stage IIIStage III

(Aggressive)(Aggressive)

10-15% Benign but rapidly growing, cortex perforated

Enneking’s Staging of Benign GCTEnneking’s Staging of Benign GCT

Campanacci’s Radiological GradingCampanacci’s Radiological Grading

Grade I – Cystic lesionGrade I – Cystic lesionGrade II – Cortex thin but not perforatedGrade II – Cortex thin but not perforatedGrade III – Tumour extended into surrounding soft tissueGrade III – Tumour extended into surrounding soft tissue

Treatment (Grade-I and Grade-II)

(1) Only curettage.

(2) Curettage + bone grafting.

(3) Curettage + bone grafting + liquid nitrogen (cryosurgery).

(4) Extended aggressive curettage + Phenol / argon beam coagulation / bone cementing.

Grade-III – Resection + Reconstructions

Resection : En Block (Joints lost)

* Role of Radiation in GCT- spine/pelvis (1500-5000 rads for 5-6 wks)

Giant Cell Giant Cell TumourTumour

Giant Cell Giant Cell TumourTumour

OSTEOSARCOMABimodal age distribution: Primary 10-20, secondary 50-70.75% around kneeBone forming tumor arising from boneSeveral subtypes –By location (Central or Juxtacortical)

Degree of differentiation: well to dedifferentiatedHistologic variance:

osteoblastic/chondroblastic/fibroblastic/telangictatic/small cellAssociated with Retinoblastoma (rb gene), Li Fraumeni

syndrome (p53 gene), Rothmund Thomsen syndrome.

GROSS PATHOLOGY:

MetaphysealTan-white solid tumor fills

most of the medullary cavityof the metaphysis andproximal diaphysis

Expanding & infiltratingthrough the cortex, lifts theperiosteum (Codman’striangle) and forms soft tissuemasses on the side of the bone

Areas of hemorrhage andcentral necrosis.

HISTOPATHOLOGY:Pleomorphic and anaplastic cell

population- large hyperchromaticnuclei, mitotic figures

Abundant fibrous/ chondroidmatrix

Formation of pink homogenousosteoid by neoplastic cell:characteristic.

The neoplastic bone has a coarse,lacelike architecture but is alsodeposited in broad sheets or asprimitive trabeculae.

Osteoblastic, chondroblastic orfibroblastic types

Osteoclast-like giant cells maybe present

CHONDROSARCOMA

Age: 30-60

Primary or Secondary ( Enchondromatosis [Ollier 50%,Maffucci 100%], diaphyseal aclasis 20%, osteochondroma 0.25%)

Pelvis (30%), Femur (20%)

Types: Conventional, mesenchymal, clear cell,juxtacotical, dedifferentiated

Most common malignant bone tumor of hand

GROSS PATHOLOGY:Large bulky tumorMade up of lobules of gray-white

/somewhat translucent glistening tissue.

Tumor permeating throughout themedullary cavity, growing through thecortex, and forming a relatively well-circumscribed soft tissue mass.At center – necrotic/liquefied/cystic.Gelatinous appearance secondary tomyxoid changes in matrix.May show calcification.The adjacent cortex is thickened oreroded, and the tumor grows with broadpushing fronts into the surrounding softtissue.

HISTOPATHOLOGY:

• Tumor cells producecartilaginous matrix; well,moderate or poorlydifferentiated.

• May have only minor orfocal atypia

• Intracytoplasmic hyalineglobules common in lowgrade tumors

EWING’S SARCOMAJames Ewing 1921GROSS PATHOLOGY:Diaphyseal in long bones;

also pelvis, rib, scapulaWhite tan grey mass like

brain; or red like red currantgelly if hemorrhagic

With necrosis & hemorrhageCortical destructionInvasion to soft tissue,

no capsule

HISTOPATHOLOGY:

•Homogenous & denslypacked undifferentiatedsmall round blue cells likelymphocytes; regular nuclei,infrequent mitoses, scantclear cytoplasm.

• Abundant glycogen: PAS +diastase digestible

• Rare Homer- Wrightpseudorosettes (7-8 tumorcells arranged in a circleabout a central fibrillaryspace)

D/D : Small Round Cell TumorEwing’s: mic2 overexpression - CD99/013 . t(11;22)(EWS-FLI-1). NSE +

PNET (Primitive neuroectodermal tumor): CD99/013. S100,chromogranin, synaptophysin; > 20% Homer Wright rosettes

Lymphoma (reticulum cell sarcoma, NHL): CD 45, LCA

Small cell carcinoma: keratin, synaptophysin, chromogranin

Mesenchymal chondrosarcoma

Neuroblastoma: neurofilament. S100, chromogranin, synaptophysin.

Alveolar rhabdomyosarcoma: actin, desmin, vimentin, MyoD1,myogenin

Leukemia

OSTEOCHONDROMAGROSS PATHOLOGY:Mushroom shapedCartilage-capped bony outgrowthCartilage cap usually regular and

thin

Sessile / PedunculatedPedunculated : attached to skeletonby bony stalk; medullary cavity ofthe osteochondroma and bone arein continuityDiaphyseal aclasis: multipleheriditary exostosisTrevor’s disease: Epiphyseal sideosteochondroma

Painful osteochondroma:Fracture

Bursitis

Malignant transformation: fluffy calcification in thecartilage cap, thickness of cartilage cap > 1 cm.

Radiology

Treatment

CHRONIC OSTEOMYELITISSequestrum is the necrotic

bone that is embedded in thepus/infected granulationtissue.

Involucrum is the new bonelaid down by the periosteumthat surrounds the sequestra.

Cloaca is the opening in theinvolucrum through whichpus & sequestra make theirway out.

Types of sequestrum…..

Tubular: long boneAnnular: amputation stumpRing: around pin tractsFlake, coke, rice grain: tubercularButton: histiocytosisFeathery: syphilisMatch stick: sickle cellColoured: fungalBlack: gun shotBombay: exposed bone after open fracture

TUBERCULAR SPONDYLITIS (POTT’S)SPINE

Paradiscal

Loss of heightof vertebra

Caseatingnecrotic tissue

Bone necrosis;sequestra

Sclerosis

Acute osteomylitis is most commonly caused by

a)staphylococcus aureus b)H. influenzae c)Streptococcus d)Salmonella e) Pseudomonas

Earliest site of bone involvement in hematogenous osteomyelitis

a)Metaphysis b)diaphysis c)Epiphysis d) Physis e)Point of entry of nutrient artery

Complications of acute osteomyelitis

a)malignancy b)fracture of the affected bone c)sepsis d)chronicity

The most common organism causing osteomyelitis in drug abusers is

a)E coli b)pseudomonas c)Klebsiella d)Staph aureus

Giant cell tumour is located in the?

a) Epiphysisb) Metaphysisc) diaphysisd) all of the above

Soap bubble appearance is seen in which of the following tumours?

a) Osteosarcomab) Chondrosarcomac) Osteoclastomad) Ewings sarcoma

A 15 year old boy is injured while playing cricket. X-rays of the leg rule out of a possible fracture. The radiologist reports the boy has an evidence of aggressive bone tumour with both bone destruction and soft tissue mass. The bone biopsy reveals a bone cancer with neural differentiation. Which of the following is the most likely diagnosis?

a) Chondroblastomab) Ewing‘s sarcomac) Neuroblastomad) Osteosarcoma

Both Ewing sarcoma and peripheral neuroepithelioma belong to the Ewing family of tumors and are considered neural tumors .

Unlike neuroblastoma, these neural tumors are not derived from the sympathetic system, and catecholamine metabolites are not excreted in the urine .

In vitro, these tumors show neural differentiation and have neural features. Results with neuron-specific enolase and S-100 protein testing are positive.

electron microscopy reveals neural structures such as neurites and dense-core granules. Glycogen granules are present, and alkaline phosphatase is absent.

Neuroblastoma (NB) is the most common extracranial solid cancer in childhood and the most common cancer in infancy

It is a neuroendocrine tumor, arising from any neural crest element of the sympathetic nervous system (SNS).

It most frequently originates in one of the adrenal glands, but can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.

Although it can affect people of all ages, it most often appears in males younger than 25 years old.

Biopsy and hematoxylin and eosin (H&E) staining under high light microscopy magnification show numerous chondroblastic cells embedded in a highly mesenchymal cartilaginous matrix.

Chicken-wire calcification in a latticelike pattern surrounding the tumor cells is present in 30% of chondroblastomas.

CHONDROBLASTOMA

Malignant tumour of mesenchymal cells characterized by formation of osteoid is description fit for ?

a) Osteosarcomab) Chondrosarcomac) Osteoclastomad) Ewings sarcoma

Secondary osteosarcoma can be caused after? a) Pagets diseaseb) fibrous dysplasiac) multiple enchondromatosisd) All the above

Causes of secondary osteosarcoma:

1- Radiation

2- Bone Diseases: a) Pagets disease b)Hereditary multiple osteochondroma c)Enchondromatosis d) Fibrous dysplasia

3- Inherited Cancer Syndromes: a) Li–Fraumeni syndrome b) Rothmund-Thomson syndrome c) Hereditary retinoblastoma d) Bloom syndrome e) Werner syndrome f) Diamond-Blackfan anemia

Most common site of metastasis in Osteosarcoma is ?

a) Lungsb) Heartc) Braind) Liver

True statement about involucrum and sequestrum:

a) Involucrum is a piece of dead boneb) Sequestrum is a sheath of new bone formationc) Sequestrum is surrounded by involucrumd) Involucrum is surrounded by sequestrum

Pulsatile tumor is seen in

1. Osteosarcoma2. Osteoclastoma3. Ewings4. Chondrosarcoma

Pulsating Tumors of the Bone

*Primary tumors that may present as pulsating lesions :

1- Telengiectatic Osteogenic sarcoma2- Angioendothelioma/Angiosarcoma of bone3- Aneurysmal bone cyst4- Giant cell tumor (rarely)

*Secondaries/Metastases that may present as pulsating lesions :

1- Metastasis from Renal cell carcinoma2- Metastasis from Thyroid carcinoma

Ewings sarcoma is believed to arise from :

a) Aberrant cartilage restsb) Endothelial cells of bone marrowc) Mesothelial cellsd) Periosteocytes

Which of the following malignant tumours is radio resistant?

A. Ewing’s sarcomaB. RetinoblastomaC. OsteosarcomaD. Neuroblastoma

Most radio resistant tumors are:

1.Malignant melanoma 2.Osteosarcoma 3.Pancreatic carcinoma

All the statements are true about exostosis, except

A. It occurs at the growing end of boneB. Growth continues after skeletal maturityC. It is covered by cartilaginous capD. Malignant transformation may occur

Which of the following conditions is least likely to present as an eccentric osteolytic lesion:

A. Aneurysmal bone cystB. Giant cell tumourC. Fibrous cortical defectD. Simple bone cyst

7 year old child presents with a lesion in upper tibia. X-ray shows radiolucent area with Codman’s triangle and Sunray appearance. Diagnosis is :

a) Ewing’s sarcomab) Osteosarcomac) Osteoid osteomad) Chondrosarcoma

Sunray appearance” on x-rays is suggestive of :

1. an osteogenic sarcoma.2. a chondrosarcoma.3. a metastatic tumor in the bone.4. an Ewing’s sarcoma.

The Codman triangle may be seen with aggressive lesions:

1- osteosarcoma 2- Ewing sarcoma 3- osteomyelitis 4- active aneurysmal bone cyst 5- giant cell tumour 6- metastasis 7- chondrosarcoma (especially juxtacortical chondrosarcoma) 8- malignant fibrous histiocytoma

Chondroblastomas most commonly occurs in ?

a- epiphysisb- diaphysisc- metaphysisd- medullary cavity

Most common benign tumour of the bone ?

a- giant cell tumourb- simple bone cystc- osteochondromad- enchondroma

Onion peel appearance on x ray is seen in?

a- osteosarcomab- ewings sarcomac- osteoclastomad- osteochondroma

Which of the following is not a benign tumour ?

a- chondromab- chordomac- osteochondromad- enchondroma

Most common site of osteogenic sarcoma ?

a- femur upper endb- femur lower endc- tibia upper endd- tibia lower end

Turn - o - plasty is used in the treatment of which of the following tumours ?

a- osteosarcomab- osteoclastomac- osteochondromad- osteoblastomae- chondrosarcoma

Which of the following is the least commonly affected site for chondrosarcoma?

a. Pelvisb. Proximal Femurc. Proximal Humerusd. Hands and Feet

A 30-year-old man has pain about his left knee that he says has been worsening "for weeks". On physical examination the left knee appears larger than the right, and there is tenderness to palpaption. A radiograph reveals a 7 cm lytic lesion involving the epiphyseal region of the distal femur with a "soap bubble" appearance. The lesion is curretted, and histologically there are numerous multinucleated cells in a stroma with plump to spindle shaped mononuclear cells. Which of the following is the most likely diagnosis?

A OsteosarcomaB ChondrosarcomaC Malignant fibrous histiocytomaD Giant cell tumorE Tuberculosis

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