BONE PATHOLOGY TUMORS NEOPLASIA. LECTURE 3.pptx

Bone pathology

1. BONE PATHOLOGY

2. OUTLINE Osteopetrosis Paget’s Disease Hyperparathyroidism Osteogenic Imperfecta Bone Pathology

3. Also known as MARBLE BONE DISEASE Rare hereditary skeletal disorder characterized by a marked increase in bone density resulting from a defect in remodeling caused by failure of normal osteoclast function 2 clinical patterns: Infantile Adult OSTEOPETROSIS

4. Inherited as an autosomal recessive trait Facial deformity manifesting as broad face, hypertelorism , snub nose and frontal bossing Delayed tooth eruption Failure of normal bone resorption and bone remodeling leads to narrowing of the cranial foramin hence giving rise to optic nerve atrophy, blindness, deafness and facial pralysis Pathologic fractures and osteomyelitis are common INFANTILE OSTEOPETROSIS

5. INFANTILE OSTEOPETROSIS

6. RADIOGRAPHIC FEATURES: Skeletal density In dental radiographs, teeth are difficult to visualize due to bone density INFANTILE OSTEOPETROSIS

7. Discovered later in life Less severe Axial skeleton reveals significant seclerosis Cranial nerve compression Bone fractures are rare If mandible is involved, fracture may occur after a tooth extraction ADULT OSTEOPETROSIS

8. TREATMENT: Poor prognosis for infantile osteopetrosis Adult osteopetrosis has better prognosis Bone marrow transplant only hope for permanent cure OSTEOPETROSIS

9. Uncoordinated increase in the osteoclastic and osteoblastic activity in older bones Resultant bones are large and weak Patients develop extensive bone pains, high levels of serum alkaline phosphatase , and increased tendency to develop malignant bone neoplasms Bone resorption and bone deposition process are increased simultaneously This increased activity gives ‘cement’ or ‘reversal’ lines appearance on histopathology called MOSAIC PATTERN PAGET'S DISEASE

10. PAGET'S DISEASE

11. CLINICAL FEATURES: Older age group Male to female ratio is more May be monostotic or polystotic Most common sites are skull, maxilla, vertebrae, spinal chord and tibia maxilla > mandible When skull is involved, an increase in the size and shape of the skull is seen which leads to decreased foramina size Compression of spinal chords and cranial nerves occur leading to paralysis and loss of hearing and sight PAGET'S DISEASE

12. Because it is asymptomatic, the first sign of involvement is generalized spacing in the teeth In an edentulous patient, the dentures may become tight When bones are seriously effected, they become painful and subject to fractures Patient develop sarcomas in later stages PAGET'S DISEASE

13. PAGET'S DISEASE

14. RADIOGRAPHIC FEATURES: Early osteolytic stage, bone exhibits diffuse radiolucency Later stages show increased bone density Cotton ball appearance PAGET'S DISEASE

15. PAGET'S DISEASE

16. HISTOPATHOLOGY: Mosaic pattern of bone Increase in osteoclasts , osteoblasts and blood vessels Replacement of normal dense bone with less dense bone In osteolytic phase, osteoclasts are large and numerous In osteosclerotic phase, osteoblasts predominate PAGET'S DISEASE

17. PAGET'S DISEASE

18. TREATMENT: Treatment is towards slowing the process by giving calcitonin and disphosphonates PAGET'S DISEASE

19. Excess production of parathyroid hormone results in hyperparathyroidism PTH is produced by parathyroid gland in response to decreased serum calcium levels Triad signs, ‘ stones, bones and groans’ Renal calculi (stones) produced due to elevated serum levels Osseous changes occur (bones) Brown tumour a type of unilocular / multilocular radiolucency in mandible, clavicle, ribs etc HYPERPARATHYROIDISM

20. Name of brown tumour due to its redish brown colour which is due to hemosiderin deposition and hemorrhage with in the tumour Groans refer to numerous duodenal ulcers which are developed in relation to hyperparathyroidism Most prone patients are women above 60 yrs of age HYPERPARATHYROIDISM

21. HYPERPARATHYROIDISM

22. HYPERPARATHYROIDISM

23. HISTOPATHOLOGY: Similar to central giant cell granuloma Giant cells in a background of fibrocollagenous tissue background Numerous blood vessels of variable calibre HYPERPARATHYROIDISM

24. HYPERPARATHYROIDISM

25. Heritable group of connective tissue defect Fragile bones, blue sclera, ligament laxity, hearing loss, dentinogenesis imperfecta Four distinct types of osteogenesis imperfecta depending upon clinical and radiographic presentations Type I Type II Type III Type IV OSTEOGENESIS IMPERFECTA

26. Osteogenesis imperfecta type I: Osteoporosis Bone fragility Blue sclera Conductive hearing loss Fractures may be present in 10% of patients at birth Height and weight normal at birth but postnatal short stature relates to degree of involvement of bones and limbs Bowing of limbs OSTEOGENESIS IMPERFECTA

27. OSTEOGENESIS IMPERFECTA

28. Osteogenesis imperfecta type II: Lethal syndrome with half of the patients still born at birth Infants are of low birth weight, short stature, broad thighs extending at right angles to the trunk Limbs are short, curved and grossly deformed Skin is thin and frail and may tear during the delivery Defects in bone ossification lead to fragile bones and frequent fractures OSTEOGENESIS IMPERFECTA

29. OSTEOGENESIS IMPERFECTA

30. Osteogenesis imperfecta type III: Severe bone deformity Multiple fractures and bone fragility Blue sclerae at birth Shortest stature of all types of osteogenesis imperfecta Dentinogenesis imperfecta seen OSTEOGENESIS IMPERFECTA

31. OSTEOGENESIS IMPERFECTA

32. Osteogenesis imperfecta type IV: Bone fragility Blue sclarae at birth only Skeletal abnormalities vary from patient to patient Fractures can occur from childhood to adult hood Dentinogenesis imperfecta seen OSTEOGENESIS IMPERFECTA