Pathology-Musculoskeletal System Ii

Front 1

Paget disease

Back 1

Characterized by:
1. localized osteoclastic activity and bone resorption
2. followed by exuberant bone formation

Front 2

Paget disease -morphology
three phases of development

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1. phase of osteoclastic activity (bone destruction)
2. phase of osteoblastic and osteoclastic activity (bone destruction and formation)
3. osteosclerotic phase (formation of dense sclerotic bone)

Front 3

Paget disease
Laboratory

Back 3

-estimating Bone specific alkaline phosphate leveles by immunoassay-it will be ELEVATED
-serum calcium increased (during the lytic phase)
-increased urine calcium-during lytic phase

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Paget Disease
Clinical Features

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Deformity, high output congestive cardiac failure, visual deformity, deafness, fractures, spinal nerver rot compression, NEOPLASTIC COMPLICATIONS

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Paget Disease
Clinical features-deformity

Back 5

-common feature of Pagets disease is skeletal deformity
-assymetric enlargment of the cranium
-transverse fracture of the femu "chalk stick fracture"

Front 6

Bone Tumors: "FACTS"

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-metastatic tumors are more common than primary tumors
-metastatic lessions are usually multiple

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Primary bone tumor Risk factors

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1. mostly arise de novo
2. paget disease of bone
3. chronic ostemyelitis
4. radiation
5. gardner syndrome (osteoma)
6. familial retinoblastoma (osteosarcoma)

Front 8

OSTEOMA
-(bone forming benign tumor)-

Back 8

-usually at neack and head
-multiple lessions are features of gardners syndrome
-histo: bland mixture of woven and lamellar bone

Front 9

OSTEOID OSTEOMA
-(bone forming benign tumor)-

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-painful mass
-responsive to aspirin
-invovlves cortex of the bone, produces a nidus
-site: femur and tibia

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OSTEOBLASTOMA
-(bone forming benign tumor)-

Back 10

-not significantly tender
-not responsive to aspirin
-no such lession
-site: vertebral column

Front 11

Osteogenic sarcoma (osteosarcoma)
-(malignant bone forming tumor)

Back 11

-malignant mesenchymal tumor of bone that produced osteoid
-usually shows up after multiple myeloma, osteogenic sarcoma is the most common tumor of the bones
-usually hits teens (below 20), metaphyseal regions of the long bones before epiphyses are closed

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Classification of osteosarcoma

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PRIMARY
1. arise de novo
2. common in teenagers
3. around knee joints
SECONDARY
Pagets disease, fibrous dysplasia, ostemyelitis, bone infract, bone fracture, previous irradiation

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Risk factors for osteosarcoma (malignant bone tumor)

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1. patients with familial retinoblastoma (Rb suppressor gene on chromosome 13)
2. associated with mutation of p53 suppressor gene on chromosome 17
3. over -expression (gene amplification) of MDM2 oncogene
REMEMBER-these tumors rarely destory the epiphyeal plate

Front 14

Osteosarcoma Clinical presentation

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-these tumors present with local swelling, tenderness or bone pain
-present with fracture

Front 15

Osteosarcoma
-spread/management-

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SPREAD
-these tumors metastasize widely to the lung, other organs and bones
-pulmonary metastasis is COMMON
TREATMENT
-it can include surgery, chemotherapy or radiation therapy

Front 16

Osteosarcoma /X-ray

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Codmans triangle:
these tumors may lead to destruction of the cortical bone and elevation of the periosteum (Codmans triangle)

Front 17

Osteosarcoma -what do you see on X ray?

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Sunburst Appearance
Codmans Triangle

Front 18

Enchondromas

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benign tumors made of mature hyaline cartilage-small bones of hands and feet

Front 19

Olliers Disease

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-cartilage benign forming tumor-
*multiple enchondrmas innvovling one side of body

Front 20

Maffucci syndrome

Back 20

-cartilage benign forming tumor-
*multiple enchondromas and benign vascular tumor
**multiple enchondromas-may develop chondrosarcoma

Front 21

Osteochondroma (exostoses)
-(cartilage benign tumor)-

Back 21

arise fromthe epiphysisi near the growth plate
-usually single
-familial disorder may have multiple exostosis (multiple hereditary exostosis)

Front 22

Osteochondroma -micro/gross

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-broad based bony excrescence and a cap of hyaling cartilage is present over the growth

Front 23

Chondrosarcoma
-(malignant cartilage tumor)-

Back 23

-tumor of cartilage (chondroid cells)
-malignant tumor producing a cartilagous matrix
-it occurs at older age (60)
-located at central portion of skeleton-shoulder, pelvis, femur and ribs
-pathogenesis: areise de novo or follow multiple enchondromas
-metastize to LUNGS

Front 24

Giant cell tumor (GCT)

Back 24

-also called osteoclastoma
-it contains large number of osteoclastic giant cells -"osteoclastomas"
-located at epiphysis of the logn bones
-gross: hemorrhagic, browth colored mass

Front 25

Giant Cell Tumor/X-ray

Back 25

soap bubble appearance
-usually benign only 4 % can becamse malignant
-you can manage it but it can come back

Front 26

Ewing's tumor

Back 26

-6-10% of all bone tumors
-occurs in children and second decade and is located at diaphysisis of long bones: femur, tibia and pelvis
-it can clinicallly mimmic acute inflamation
-ewings tumor fall in the grop of PNET (peripheral Neuroectodermal tumors)

Front 27

Ewing's Tumor /micro

Back 27

PAS positive-small blue round cells tumors
-minimmal cytoplasam with large hyperchromatin nuclei

Front 28

Ewing's tumor/X-ray

Back 28

Onion skin periosteal reaction

Front 29

Fibrous Dysplasia

Back 29

-tumor like condition
-trabecular bone is replaced by proliferating fibrous tissue and malformend bone
-located: ribs, tibia,jaw, calvarium
-has Woven bone and dense fibrous tissue-chinese letter pattern

Front 30

Fibrous Dysplasia
-monostotic/polyostotic-

Back 30

*monostotic-innvoving single bone
*polyostotic-innvovling multiple bones with endocrine abnormality

Front 31

McCune-Albright Syndrome

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Fibrous Dysplasia -polystotic with endocrine abnormality
1. unilateral lession
2. care-au-lait spots on same side
3. precocious puberty

Front 32

McCune-Albright Syndrome-clincial features and complications

Back 32

Clinical features
1. fracture
2. bone deformity
3. asymptomatic
Complications
1. formation of osteosarcoma

Front 33

Rheumatoid Arthritis
(joint disease)

Back 33

-autoimmune disease HLA-DR4 positive
-produce bony joint anylosis
-MOA-proliferation of the synovial membrane leads to pannus formation that erodes the cartilage and subchondrial bone

Front 34

what is Pannus?

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-Pannus is hyperplastic synovium that is infiltrated by numerous:
1. lymphocytes (produce lymphoid follicles)
2. Plasma Cells

Front 35

Rheumatoid arthritis
-clinical features-

Back 35

1. early PROXIMAL interphalangeal joints-plyarthritis and symmetrical innvolvement (painful and swolen)
2. late -bondy ankylosis-narrow joint space
3. serum is positive for Rheumatoid arthritis factor (IgM) (RA factors)

Front 36

Osteoarthritis
-(joint disease)-

Back 36

-it frequences with increase in age
-number of joints: usually ONE or more joitns can be affected
-joints affected: weight bearing joints (hips, knees, vertebra) and DISTAL interpharangeal joints

Front 37

Osteoarthritis-pathophysiology

Back 37

MOA-joints are subjected to stressful "wear and tear" and are more pronte to damage of hyaling cartilage
-this produces the joint damage
morphology-eroded articular cartilage; apposing bones rub together, reslting in eburnation of the bones

Front 38

Heberdane Nodes in Osteoarthritis

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-small osteophytes (bony outgrowths) in the distal interpharangeal joints seen in osteoarthritis, also decrease in joint space

Front 39

Osteoarthritis
-clinical features-

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-joint stiffness early in the morning with pain and decrease after some time
-pain aggravates at the end of the day
-joint crepitus present
D/D=rheumatoid arthritis

Front 40

Gout

Back 40

-disease caused by excessive accumulation of Uric Acid Crystals in the joints
-common sites: first metatarsophalangeal joint

Front 41

Gout
-primar/secondary-

Back 41

PRIMARY-uknown cause
SECONDARY
1. in leukemia
2. Lesch Nyhan syndrome-complete lack of HGPRT (hypoxanthine guanine phosphorybosyltransferase)

Front 42

Gout
-pathogenesis-

Back 42

1. over production of uric acid
2. release of IL1, TNF by monocytes and complete activation
LEADS TO
3. inflamation and tissue injury

Front 43

Gout
-morphology-

Back 43

ACUTE-deposition of monosodiumurate crystals in the joint (acute arthritis. Acute attack is self resolved
CHRONIC-presence of a tophus (aggregates of cyrstals) is the characteristic finding of chronic gout

Front 44

Tophi

Back 44

are most likely to be found in soft tissues, including tendons and ligaments around joints
-tophi are aggreagetes of urate crystals surrounded by infiltrates of lymphocytes, macrophages and forgein body giant cells

Front 45

Sites of gout attack

Back 45

1. great toe
2. instep
3. ankle
4. heel and wrist

Front 46

Gout
-complications-

Back 46

-pyelonephritis
-chronic renal failure (Gouty nephrophaty)
-attack of bout may occur due to destruction of tumor cells during chemoterapy

Front 47

Lyme disease
(infectious disease)

Back 47

-etiology: borrelia burgdorferi-spirochete
-produced CHRONIC ARTHRITIS
-effect large joints: knee, shoulder, elbow
-CSF: lymphocytes and plasma cells, antispirochete immunoglobin

Front 48

Lyme Disease
-acute illness-

Back 48

weeks-STAGE 1
-tick bite-erythematous papule, erythema chrnicum migrans
-lymphadentitis
-Clinical picture-coin like skin rash, fever, lumphadenopathy. Coin like erythematous rash with central pallor

Front 49

Lyme disease
-diseemination-

Back 49

weeks to months -STAGE 2
CNS:
-meningoencephalitis, cranial neuritis
CARDIAC:
-heart block, pericarditis, myocarditis

Front 50

Lyme Disease
-late chronic form-

Back 50

destructive chronic arthritis, acrodermatitis atrophicans, neuropahty

Front 51

Duchenne muscular dystrophy
-(muscle disorder)-

Back 51

-an X-linked recessive disorder
-cause : complete absence of membrane skeletal protein dystrophin

Front 52

Duchenne muscular dystrophy
-micro view-

Back 52

-degeneration of muscle fibers, scattered chronic inflamatory cells, fibrosis and hypetrophy of remaining muscle fibers
-adipose tissue in the muscle produces Pseudohypetrophy of the calf muscle

Front 53

Duchenne muscular dystrophy
-clinical features-

Back 53

initially develops as more proximal muscle weakness early in childhood
-die of respiratory failure by the second or third decade

Front 54

Becker Muscular Dystrophy
-(muscle disorder)-

Back 54

-milder form of the disease
-mild dystrphyn deficiency

Front 55

Type II muscle atrophy

Back 55

-common abnormality in skeletal muscles
-disuse atrophy-bedriden patients, immobilised patients
-patients on glucocorticoids
-patients with Cushing syndrome

Front 56

Polymyositis

Back 56

-autoimmune disease that can be associated with SLE or Dermatomyositis
-presence of neutrophils in the muscle
-use Jo-1 to diagnose it
-patients have difficulty climbing stairs or geting up from chair
-common in women associated with visceral cancer, symmetric weaknes of large muscles

Front 57

Dermatomyositis

Back 57

-has skin rash
-typically the violaceous"helitrope" rash of eyelids

Front 58

Myastenia Gravis

Back 58

-presence of immunoglobin directed against the acetylcholine receptors of the motor end plate
-D/D=lambert eaton myasthenic syndrome

Front 59

Lambert Eaton Myastenic Syndrome

Back 59

in small cell carcinoma of lung.
-antibody directed against the calcium channel

Front 60

Thymic Hyperplasia
-(myastenia gravis)-

Back 60

-thymic hyperplasia in myastenia gravis-hyperplasia of thymus, (thymus is located substernally) it becames hyperplastis.
-intense folicular hyperplasia is seen in myastenia gravis

Front 61

Myastenia Gravis
-(clinical Features)-

Back 61

muscle weaknes prominent after repeated stimulation of the muscle: worsens in the later part of the day

Front 62

Myastenia Gravis
-(common sites)-

Back 62

-muscle controlling eyelids and eye movement
-respiratory muscles

Front 63

Lipoma
-(soft tissue tumor/adipose tissue)

Back 63

-subcutanout tissu: trunk, neck, abdomen but it can happend anywhere
-well demarked tumor, yelow, soft
-under microscope-many adipose tissue with litlle stroma
-do frozen section biopsy/used a lot in breast surgery patients

Front 64

Sarcoma
-(malignant neoplasm of Mesenchymal tissue)-

Back 64

Molecular marker of sarcoma
1. tumors of the muscle -DESMIN
2. tumors of other conncetive tissue-VIMENTIN

Front 65

Desmoid Tumor

Back 65

-also called "aggressive fibroblastic proliferations"
-site: shoulder, chest and abdominal wall, nec and thight in both men and women
*In women during pregnancy or just following pregnancy they may appear in abdominal wall

Front 66

Rhabdomyosarcoma
-(muscle -skeletal tumor)

Back 66

-common tumor in children
-located: bladder wall, head and neck, vagina
-surgically can repair but it can come back
-"strap cells" can see cross section that indicated that skeletal muscles, but you can see elongated cells,pleomorphysm of nuclei

Front 67

Synovial Sarcoma

Back 67

-mesenchymal cells about joint cavities
-clinical-mass near joints
-small poorly circumscribed cystic mass
-biphasic pattern-spindle and epithelial component
-monophasic-spindle cells only

Front 68

Metastatic Carcinoma

Back 68

Prostate: osteoblastic lession
-metastatic carcinoma can develop at prostate (commonly located there) and also it can metastaze and became metastatic tumor

Front 69

Schwanomma
-(neurieloma)-

Back 69

"Nerve Tissue Tumor"
-marker S-100 protein
-histo: Verocacy Body

Front 70

Angiosarcoma/Hemangioendothelioma

Back 70

"Blood vessel Tumor"
-red plaque lession on skin
-Kaposi sarcoma (HIV induced) bloody tip of nose