BONE & SOFT TISSUE PATHOLOGY

COL Y ILIYASU FMCPath; FICS Chief Consultant Histopathologist

Classification of Diseases
Congenital Acquired Traumatic Inflammatory Neoplastic Metabolic Haematologic Vascular Degenerative Autoimmune Idiopathic
Iatrogenic

OSTEOMYELITIS
Inflammation of bone and marrow Primary or Secondary to systemic infection Bacteria, viruses, fungi, parasites Pyogenic and Mycobacterial infections are commonest

Pyogenic Osteomyelitis
Route Haematogenous Extension from a contigous site Direct implantationsurgery,open fractures

Organisms
Staphy aureus E coli Pseudomonas Klebsiella H influenza & B Strept Salmonella--80-90% GU Infections

Drug users Infants Sicklers

Sites
Determined by vascular circulation hence age Neonates---metaphysis, epiphysis Children-----metaphysis Adults--------epiphysis, subchondral area

Morphology
Depends on stage and location Acute osteomyelitis Necrosis and subperiosteal abcess Sequestrum Draining sinus Joint involvement in children-septic athritis Involucrumnew bone

Complications
Chronic osteomyelitis Pathological fracture Amyloidosis Endocarditis Septicaemia SCC in sinus tract Bone sarcoma--rare

BONE TUMOURS
Haematopoeticmyeloma, lymphoma Chondrogenic Osteochondroma Chondroma Chondrosarcoma Chondroblastoma Chondromyxoid fibroma

 Osteogenic Osteiod osteoma Osteoblastoma Giant Cell Tumour

Osteosarcoma Erwings sarcoma Giant Cell Tumour Adamantinoma

MFH Desmoplastic fibroma Fibrosarcoma

Histiocytic FH

 Notochordal Chordoma Vascular haemangioma,- sarcoma

haemangiopericytoma haemangioendothelioma Lipogenic Lipoma Liposarcoma

Neurogenic Neurolimemmoma MPNST

Osteomas
Slow growing bosselated tumours Solitary Multiple---Gardners Syndrome Mechanical effectsbrain,eye,oral cavity

Osteoid Osteoma/ Osteoblastoma

Same tumour depending on location Osteoid Osteoma 2% in diameter 50% involves the femur & tibia Pain-PGE2@ relieved by aspirin Osteoblastomainvolves the spine

Osteogenic Sarcoma
Malignant tumour characterised by osteoid production 3rd commonest primary tumour of bone after myeloma and lymphoma Commonest in younger people 75% <20yrs M:F=2:1 Usually arises from metaphyseal regions of extremeties esp knee ( 50-60% ) Due to abnormalities of P53 gene

 Several subtypes Anatomic portion of long bone-surface, intracortical, intramedullary Degree of differentiation Multicentricity-synchronous,metachronous Primary / Secondary eg Pagets disease Histological variantsosteoblastic,chondroblastic,fibroblastic,tele ngiectatic,small cell,giant cell

 Big bulky tumours, gritty, gray-white, haemorrhage, necrosis Can lift periosteumCodmans Triangle Osteoid production Chondrosarcomatous osteogenic sarcoma-when malignant cartilage is present Vascular invasion

Clinicals
Bone swelling Pathological fracture Metastases-lungs, brain, other bones Long-term survival60% MDR 1 gene product p-glycoprotein mediates multidrug resistance

Osteochondroma
Exostosis Solitary Multiple-multiple hereditery exostosis

Chondromas
Benign tumours of hyaline cartilage Medullary cavity-enchondroma Bone surfacejuxtacortical/subperiosteal Usually solitary-tubular bones of hand/foot Multiple enchondromas ( enchondromatosis) / Olliers Disease Enchondromas + haemangiomasMaffuchis Syndrome

CHONDROSARCOMA
Malignant tumour charactesied by production of neoplastic cartilage 2nd most common malignant matrix producing tumour Seen 40 years and above M:F=2:1 Arise in association with pre-existing disease eg enchondroma,osteochondroma Occurs in bones of axial skeleton-pelvis, ribs, shoulder

Rarely involves the extremities Painful progressively enlarging masses Indolent slow growing tumours Metastases to lungs and other bones

Erwings Sarcoma
Primary small round cell tumour of bone DD of small blue cell tumours of infancy and childhood Second commonest malignant bone tumour in children after osteosarcoma C-myc translocation 11:22 in 85% of cases

Secondary Bone Tumours

Direct; lymphatic / vascular; intraspinal seeding ( Batson plexus of veins ) Adults-75% from prostate, kidney, lung and breast Children-from neuroblastoma, Wilms Tumour, rhabdomyosarcoma

METABOLIC DISORDERS
A. Ricketts and Osteomalacia Defect in matrix mineralisation Due to the lack of Vit D or disturbances of its metabolism B.Hyperparathyroidism C.Renal Osteodystrophy

MUSCULAR DYSTROPHIES
Heterogenous group of inherited disoders often beginning in childhood and characterised by progressive muscle weakness and wasting Duchenne-most common, X-linked Limb Girdleautosomal dominant Myotonic dystrophy-autosomal dominant Myochondrial myopathies-oxidative phosphorylation disease

 Inflammatory myopathies- polymyositis Toxic myopathies- thyrotoxic myopathy Alcoholic myopathies rhabdomyolysis myoglobinuria renal failure Drug-induced- steroids, chloroquine

RHABDOMYOSARCOMA
Malignant tumours of skeletal muscles Common in head, neck and genitourinary tract Characterised by rhabdomyoblast A.Embryonal / Sarcoma botryoides. Commonest. nasal cavity, orbit, prostate B.Alveolar-deep musculature of extremities C.Pleomorphic-bizarre multinucleate giant cells

LEIOMYOMA
Benign smooth muscle tumours Commonly seen in the uterusfibroids May arise from skin and subcutis from arrector pili muscleskin, nipples,scrotum Heridetery leiomyomatosisautosomal dominant

LEIOMYOSARCOMA
Seen in adults. More common in women 10-20% of all soft tissue tumours Occurs in skin and deep soft tissues of the extremities and retroperitoneum Prognosis determined by site and location

LIPOMA
Benign tumours of adipocytes Most common soft tissue adult tumours Subcutis of proximal extremities and trunk Can be large and intramuscular Soft mobile and painless Subtypesfibrolipoma, angiolipoma, spindle cell lipoma, etc

LIPOSARCOMA
Common sarcomas of adulthood Age 40-60 years Deep soft tissues of proximal extremities and retroperitoneum Behaviour determined by histological type

SYNOVIAL SARCOMA
4th most common sarcoma Thought to arise from the synovium but only few are intra-articular Majority develop in the vicinity of large joints of the extremities 60-70% seen around the knee or thigh Biphasic in appearance Spreads to lymph nodes,lungs and bones

BENIGN FIBROUS HISTIOCYTOMA

Seen in dermis and subcutis Painless slow growing, firm and mobile nodule Proliferating spindle cells arranged in storiform pattern---dermatofibroma May contain numerous blood vessels sclerosing haemangioma

MALIGNANT FIBROUS HISTIOCYTOMA

Heterogeneous group of aggressive soft tissue tumours Characterised by considerable cytological pleomorphism, bizarre multinucleate giant cells, storiform pattern and foamy macrophages Recur unless widely excised May spread to distant sites

FIBROSARCOMA
Rare Occur in retroperitoneum, thigh, knee and distal extremeties Infiltrative, soft, fish-flesh masses with areas of haemorrhage and necrosis Herringbone pattern Aggressive and may metastasize to distant sites

SEPTIC ARTHRITIS
Occurs in septicaemia or contiguous spread from epiphyseal osteomyelitis in neonates Staph; Strept; H influenzae and Gram Negative Bacilli eg E coli Salmonella organism in sicklers Usually monoarticular except in gonorrhoe Can be due to TB, Lyme Disease,Viruses

RHEUMATOID ARTHRITIS
Chronic systemic inflammatory disorder Nonsuppurative proliferative synovitis Destruction of articular cartilage and ankylosis Skin---rheumatoid nodules Vasculitisperipheral neuropathies, ulcers and gangrene

JUVENILE RHEUMATOID ARTHRITIS

Stills Disease Oligoarthritis More frequent systemic symptoms Absent rheumatoid nodules and factor

OSTEOARTHRITIS
Degenerative joint disease Progressive erosion of articular cartilage Primary / idiopathicnormal aging SecondaryDM; ochronosis; haemochromatosis Knees and hands in women, hips in men Heberden nodes in womenosteophytes Cervical / lumbar nerve compression-radiculopathy

PERIPHERAL NEUROPATHIES
A.Inflammatory neuropathies Inflammatory infiltrate into peripheral nerve root,sensory and autonomic ganglia by infectious agents or autoimmune mechanism Guilliam-Barre-acute inflammatory demyelinating polyradiculoneuropathy.Seen following viral infections

B.Infectious polyneuropathies Leprosy Diphtheria Varicella-zoster C.Heridetery neuropathies Motor and sensory Sensory and autonomic Familial Amyloidotic Polyneuropathies

D.Acquired metabolic and toxic Diabetes Mellitus Uraemia E. Malignancies Direct infiltration or compression of nerves Paraneoplastic Syndromes F.Toxicheavy metals eg lead,arsenic

G. Trauma Lacerations Avulsions Compression Carpal Tunnel Syndrome

MYASTHENIA GRAVIS
Autoimmune disorder Antiacetylcholine receptor antibodies Increased degradation of receptors leading to postsynaptic membrane damage Associated with thymic hyperplasia and thymoma Ptosis and diplopia Treated with anticholinestrases

LAMBERT-EATON MYASTHENIC SYNDROME

A paraneoplastic syndrome with SCLC60% Can occur in the absence of malignancy Proximal myopathy and autonomic dysfunction

SCHWANNOMA
Benign tumour arising from neural crestderived Schwann cell Associated with neurofibromatosis Encapsulated masses attached to nerves Acoustic neuroma at cerebellopontine angle attached to vestibular branch of VIII Tinnitus and hearing loss Trigerminal nerve and dorsal roots

NEUROFIBROMA
Occurs sporadically or in association with neurofibromatosis type 1 Cutaneousdermis and subcutaneous fat Delineated but unencapsulated masses Plexiform large nerve trunk. Very difficult to remove surgically