tumours of bones, cartilage & joints mbbs lect
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Tumours of Bones, cartilage & joints
Dr Neha MahajanMD PathologyMGM Medical College, AbadTumours of Bones, cartilage & joints
Tumors and tumor-like conditions of bone- Diverse in their gross and morphologic features, and clinical behavior.- Benign tumors outnumber malignancies and are commoner in young- Specific tumor types have preferential age and sex distribution- Specific tumor types preferentially involve specific bones and specific regions within a bone
Metaphysis
CLASSIFICATIONBone formingBenign: Osteoid osteoma OsteoblastomaMalignant:OsteosarcomaCartilage formingBenign: Osteochondroma ChrondromaHaematopoetic: Multiple Myeloma Lymphoma
Notochordal tumorChordomaFibrous lesionsNonossifying fibroma Cortical desmoid Benign fibrous histiocytomaFibrous dysplasia Osteofibrous dysplasiaDesmoplastic fibromaTumors of unknown originBenign: Giant cell tumor Aneurysmal bone cystMalignant: Ewing`s sarcoma Adamantinoma
Bone tumorsHistologic typeBenignMalignantHematopoietic (40%)Myeloma, lymphomaChondrogenic (22%)Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibromaChondroscarcoma, dedifferentiated and mesenchymal chondrosarcomaOsteogenic (19%)Osteoid osteoma, osteoblastomaOsteosarcomaUnknown (10%)Giant cell tumorEwing sarcoma, giant cell tumor, adamantinomaHistiocyticFibrous histiocytomaMalignant fibrous histiocytomaFibrogenicFibromaDesomplastic fibroma, fibrosarcomaNotochordalChordomaVascularHemangiomaHemangioendothelioma, hemangiopericytoma, angiosarcomaLipogenicLipomaLiposarcomaNeurogenicNeurilemmoma (schwannoma)
Osteoma Osteoid osteoma Osteoblastoma. Osteosarcoma
Bone Forming Tumors: Production of bone by the neoplastic cells
Osteoid Osteoma & OsteoblastomaBenign bone tumors .Osteoid Osteoma are by definition >>FPELVIS, SCAPULAE, RIBS
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Osteochondroma29
CHONDROMAChondroma vs. EN-chondromaPURE Hyaline CartilageMULTIPLE enchondromas = Olliers dis.Maffucci synd. if hemangiomas present
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CHONDROBLASTOMARARE, in teenagersM>>FKNEES, usuallyEpiphysesMUCH LESS matrix than a chondroma
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CHONDROMYXOID FIBROMARAREST of allTEENS, MALESMYXOID conceptATYPIA
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CHONDROSARCOMAMalignant cartilage forming tumor that does not produce osteoidMay arise from osteochondromaThird most common bone malignancy after myeloma and osteosarcomaAge group: fourty and aboveM:F=2:1Sites: large bones - pelvis, ribs, femur, humerus, vertebrae; unusual in hands, feet, jaw, skull
ANATOMYINTRAMEDULLARYJUXTACORTICALHISTOLOGYCONVENTIONALHYALINEMYXOIDCLEARDE-DIFFERENTIATEDMESENCHYMAL
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Chondrosarcoma
GrossLarge bulky tumors made of nodules of glistening gray white,translucent cartilgaeMatrix is often gelatinous or myxoid oozes out on cutSpotty calcifications and central necrosis can produce cysticc spacesTumor spreads though cortex into surrounding muscle and fat
GROSS
MicroscopyCartilage infiltrates the marrow and surrounds preexisting bony trabeculaeTumor varies in cellularity, cytologic atypia and mitotic activity are assigned from grade 1 to 3Grade 1 low cellularity,chondrocyte have plump nuclei with small nucleoliGrade 3 high cellularity ,extreme pleomorphism with bizzare tumor giant cells and mitoses.
Conventional hyaline, mesenchymal, clear, conventional myxoid40
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Direct correlation between grade and biologic behaviour of tumorGrade 1 tumors rarely metastasize,5 yr survival rate of 80 to 90%( 43% for grade 3)Treatment is wide surgical excision with or without chemotherapy
GIANT CELL TUMOR/OSTEOCLASTOMAGCT so named histology dominated by multinucleated osteclast like giant cells(OSTEOCLASTOMA)Uncommon benign,but locally aggressive neoplasmUsually seen in 20 to 40 yrsArise in EPIPHYSISSite : around knee( distal femur and proximal tibia)Arthritis like symptoms,pathologic fractureMostly solitary, multicentric in extrmeties
OSTEOCLASTOMA showing soap bubble appearance
MORPHOLOGYGCT destroy overlying cortex, producing bulging soft tissue masss delineated by thin shell of reactive boneTumors are large, red brown masses, frequently undergo cystic degenerationM/E sheets of uniform oval mononuclear cells and numerous osteoclast type giant cellsNecrosis and mitotic activity prominent
Giant cell tumor are treated by curettage,but 40 to 60 % recur locally.4% metastasize to lung, sometimes spontaneously regress and seldom fatal.RANKL inhibitor, denosunab as adjuvant therapy
Ewing`s sarcoma/PNETSAME TUMORSMALL ROUNDNEUROENDOCRINEIDENTICAL CHROMOSOME TRANSLOCATIONSECOND most COMMON bone malignancy in CHILDRENARISE IN MEDULLARY CAVITY of BONELOOK LIKE LYMPHOMA
Ewing Sarcoma
Commonly affects DIAPHYSES of long tubular bones esp femur and flat bones of plevis
X ray destructive,lytic tumor with permeative marginMay be confused with osteomyelitis
Commonly affects diaphysis with onion skin appearance
Gross: soft tan white with areas of haemorrhage and necrosis
Small round blue cell tumor
Ewings sarcoma tumors are aggressive malignancies Neoadjuvant chemotherapy followed by surgical excision with or without radiation 5 yr survival 75 % and long term in 50%
METASTASES
MALE: PROSTATEFEMALE: BREASTRENAL, THYROID also seek bone early also
LYTIC?BLASTIC?
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Evaluation History
Physical examination
Investigations; labs, imaging
Biopsy
Analytic approach to evaluation of the bone neoplasm
What we learned today??Bone tumor classificationBone forming tumors: Osteoma,osteoid osteoma and osteosarcomaCartilage forming: Enchondroma and chondrosarcomaOsteoclastoma/Giant cell tumorEwing`s sarcoma
THANK YOU