- 1. BONES AND MUSCLES ROBERTO D. PADUA JR., MD, FPSP DEPARTMENT
OF PATHOLOGY FATIMA COLLEGE OF MEDICINE
2. SKELETAL DEVELOPMENTAL AND GENETIC DISORDERS
-
-
- Hereditary disorder involving defects in the synthesis or
structure of collagen type I
-
-
- Cardinal features : osteopenia associated with recurrent
fracture and skeletal deformity
-
-
- Biochemical findings : increased AP, increased level of
hydroxyproline, hypercalciuria
-
-
- Histology : abnormal skeletal matrix; cartilaginous bars formed
by vascular invasion of the metaphyses do not become envelop by
bones; cortical bone is almost non-existent
3. 4. 5. METABOLIC BONE DISEASES
-
-
- Loss of normally mineralized bone
-
-
- Diagnosed clinically with non-invasive radiographic techniques
that measures bone density
-
-
-
-
- Enlargement of the medullary space
-
-
-
-
- Reduction in cortical thickness
6. 7. 8. 9. METABOLIC BONE DISEASES
-
-
- Seen in patients with advanced renal failure
-
-
-
-
- Bone pain (most common), spontaneous fractures, aseptic
necrosis of hip, myopathy
-
-
-
-
- Low levels of 1;25(OH)2D3,hyperphosphatemia, hypocalcemia,
alterations in the secretion or activity of PTH
-
-
-
-
- Subperiosteal erosions, patchy osteosclerosis (rugger jersey
appearance of thoracic vertebral spine on lateral views, salt and
pepper appearance of skull, slipped epiphyses
10. METABOLIC BONE DISEASES
-
-
- Defective mineralization of the trabecular and cortical bone
matrix
-
-
- Associated with decreased serum calcium phosphate product
-
-
- A common complication of chronic renal failure in adults
-
-
-
-
- Secondary to Vitamin D deficiency
-
-
- Histologically characterized by excessive quantities of osteoid
because of the failed matrix calcification despite continued matrix
synthesis by the osteoblasts
11. METABOLIC BONE DISEASES
-
-
- Defective mineralization of the epiphyseal growth plate
cartilage
-
-
- Clinical features : craniotabes, frontal bossing, rachitic
rosary, pectus excavatum, Harrisons groove, thoracic kyphosis,
rachitic potbelly, genu varum/genu valgum
-
-
-
-
- Rachitic growth plate is wide and irregular
-
-
-
-
- Columnar rearrangement of the hypertrophic chondrocyts is
lost
-
-
-
-
- Zone of provisional calcification disappears
-
-
-
-
- Cartilage extends deep into the metaphyses
12. METABOLIC BONE DISEASES
-
-
- Skeletal manifestations are caused by unabated osteoclastic
bone resorption
-
-
- Entire skeleton is affected
-
-
- Anatomic changes of osteitis fibrosa cystica are now rarely
encountered
-
-
- Skeletal abnormalities in secondary hyperparathyroidism tends
to be milder
13. METABOLIC BONE DISEASES
-
-
-
-
- Increased osteoclast activity affects cortical bone more
severely than cancellous bone
-
-
-
-
- X-ray involves the radial aspect of the middle phalanges of the
index and middle fingers
-
-
-
-
- Dissecting osteitis osteoclasts tunnel into and dissect
centrally along the length of the trabeculae creating the railroad
appearance of railroad tracks
-
-
-
-
- Brown tumor bone loss predispose to microfractures and
secondary hemorrhages that elicit an influx of multinucleated
macrophages and an ingrowth of reparative fibrous tissue, creating
a mass of reactive tissue
14. METABOLIC BONE DISEASES
-
-
- Generalized osteitis fibrosa cystica (von Recklinghausen
disease of bone)
-
-
-
-
- Hallmark of severe hyperparathyroidism
-
-
-
-
- Composed of increased bone cell activity, peritrabecular
fibrosis, and cystic bone tumors
15. 16. 17. TRAUMA
-
-
- Blastemawound closurescar formation
-
-
- Initial repair tissue formed is called a CALLUS which is
composed of fibrous tissue, woven bone and cartilage
-
-
- 3 phases of fracture healing :
-
-
-
-
- B) reparative phase = orderly removal and replacement of
immature woven bone by cartilage differentiation
-
-
-
-
- C) modeling phase = realignment & mechanical shaping of the
bone and callus; restoration of the medullary cavity and bone
marrow
-
-
- Complications of fracture healing :
18. 19. INFLAMMATORY BONE DISORDERS
-
-
- Classified according to several factors
-
-
-
-
- 1. its duration = acute, subacute or chronic
-
-
-
-
- 2. nature of the exudate = hemorrhagic, purulent, or
nonsuppurative
-
-
-
-
- 3. its location = bone, periosteum, or epiphyses
-
-
-
-
- 4. etiologic agent = Staphylococcus, Tb, etc.
-
-
- Histologically, inflammatory cells are seen
-
-
- Loss of normal marrow architecture
-
-
- Hematopoietic elements and fat are replaced by leukocytic
infiltrates
20. INFLAMMATORY BONE DISEASES
-
-
-
-
- Coagulase (+) Staph. Aureus (60-90%)
21. INFLAMMATORY BONE DISEASES
-
-
-
-
- Characteristic lesion : Chronic caseating granulomatous
inflammation which often involves the subchondral part of the
joint. Sequestrum forms in the subchondral bone and articular
cartilage resulting in a kissing sequestrum.
-
-
-
-
- Potts disease Tb of the spine
22. OSTEOMYELITIS X-RAY GROSS : UPPER FEMUR 23. Osteomyelitis
24. INFLAMMATORY BONE DISEASES
-
-
- Noncaseating granulomatous process
-
-
- Manifest as small lytic and sclerotic foci in the bones of the
hand
-
-
- Large areas of destruction are not typically found
25. INFLAMMATORY BONE DISEASES
- PAGETS DISEASE OF BONE (OSTEITIS DEFORMANS)
-
-
- A chronic osteolytic and osteosclerotic disease of uncertain
cause
-
-
- May involve one or more bones
-
-
- Presents with pain, skeletal deformities, and occasionally
sarcomatous transformation
-
-
- Usually affects 3% of white population over 40 y/o
-
-
- Incidence increases with age; men>women
-
-
- Most patients are asymptomatic (80-90%)
26. INFLAMMATORY BONE DISEASES
-
-
- Common skeletal sites of involvement are the sacrum, spine,
pelvis, skull, femur, clavicle, tibia, ribs, and humerus
-
-
-
-
- Normal marrow is replaced by a richly vascular, loose fibrous
connective tissue
-
-
-
-
- Isolated clusters of inflammatory cells may be seen
-
-
-
-
- Osteoclasts aggregate on the existing bone trabeculae and
within the cortex
-
-
-
-
- Innumerable small, irregularly shaped bone fragments (mosaic
pattern)
-
-
-
-
- Grossly resembles the gritty but brittle texture of pumice or
lava rock
27. INFLAMMATORY BONE DISEASES
-
-
- X-RAY : flocculant, radiopaque deposit likened to cotton wool.
Pelvis is the most common site of involvement.
-
-
- Elevated AP and osteocalcin level
-
-
- Elevated urinary excretion of hydroxyproline, pyridinoline and
deoxypyridinoline
-
-
- Malignant transformation are also observed
-
-
-
-
- Giant cell malignant fibrous histiocytoma
28. 29. PAGETS DISEASE OF BONE X-RAY OF TIBIA SHOWING BONE
DESTRUCTION AND BONE FORMATION 30. PAGETS DISEASE OF BONE EARLY
CHANGES SHOWING PROMINENTOSTEOCLASTIC ACTIVITY 31. DEGENERATIVE
DISEASES OF BONE
-
-
- Infarction of bone typically involving the femoral head
-
-
- 3 generic categories = post-fracture, idiopathic, and renal
transplant associated
-
-
- Also known as avascular necrosis of bone
-
-
- Earliest histologic changes are death of the bone and the
surrounding hematopoietic & fatty marrow
-
-
- X-ray : Crescent sign , a separation of fracture cleft forms
between the impacted fragments and the overlying sub-chondral
plate. Increased density within the necrotic bone.
32. 33. 34. BONE TUMORS
-
-
- Most malignant tumors arise de novo
-
-
- Benign bone lesions that predispose to the development of
skeletal malignancies
-
-
-
-
- Pagets disease, chondromatosis, osteochondromatosis, fibrous
dysplasia, and osteofibrous dysplasia
-
-
- Five basic parameters in the diagnosis of bone tumors
-
-
-
-
- Specific area within the bone
35. BONE FORMING TUMORS
-
-
- Seen almost exclusively in the flat bones of skull and
face
-
-
- Microscopically: composed of dense, mature, predominantly
lamellar bone
-
-
- Associated with Gardners syndrome
36. BONE-FORMING TUMORS
-
-
- Benign neoplasm seen in patients between 10 and 30 y/o
-
-
- Intense pain is the most prominent symptom
-
-
- Reported in practically every bone, most are centered in the
cortex (85%), spongiosa (13%), or subperiosteal region (2%)
-
-
- X-ray: typical finding is a radiolucent nidus that is seldom
larger than 1.5 cm and may or may not contain a dense center. This
nidus is surrounded by a peripheral sclerotic reaction.
-
-
- Microscopic: sharply delineated central nidus composed of more
or less calcified osteoid lined by plump osteoblast and growing
within vascularized connective tissue, without evidence of
inflammation.
37. OSTEOID OSTEOMA X-RAY GROSS MICROSCOPIC 38. BONE-FORMING
TUMORS
-
-
- Benign osteoblastoma, giant osteoid osteoma
-
-
- Closely related to osteoid osteoma both microscopically and
ultrastructurally
-
-
- It has a larger size of the nidus, absence of surrounding area
of reactive bone formation, and the lack of intense pain
-
-
- A cartilaginous matrix is present in some cases
-
-
- Most cases arise in the spongiosa of the bone involving the
spine or major bones of the lower extremity
-
-
- Osteomalacia can be seen as a complication
39. BONE-FORMING TUMORS
-
-
- The most frequent primary malignant tumor, exclusive of
hematopoietic malignancy
-
-
- Usually occurs in patients between 10 and 25 years of age and
is rare in pre-school children
-
-
- Another peak age incidence occurs after the age of 40, in
association with other disorders
-
-
- Most osteosarcomas arise de novo, but others arise within the
context of a preexisting condition
-
-
-
-
- Pagets disease, radiation exposure, chemotherapy, preexisting
benign bone lesions, foreign bodies, trauma
40. BONE-FORMING TUMORS
-
-
- Located in the metaphyseal area of long bones, particularly the
lower end of femur, upper end of the tibia, and the upper end of
the humerus
-
-
- Large majority arise within the medullary cavity from which
they extend into the cortex
-
-
- Gross appearance varies depending on the relative amounts of
bone, cartilage, cellular stroma and vesselsbony hard to cystic,
friable, and hemorrhagic
-
-
- From its usual origin in the metaphysis of a long bone, the
tumor may spread along the marrow cavity, invade the adjacent
cortex, or elevate or perforate the periosteum (Codmans
triangle)
41. BONE-FORMING TUMORS
-
-
- Extend into the soft tissues, extend into the epiphysis, extend
into the joint space, form satellite nodules independent from the
main tumor mass proximal to the primary lesion (skip metastases),
metastasize through the blood stream to distant sites particularly
the lung.
42. BONE-FORMING TUMORS
-
-
-
-
- May destroy preexisting bone trabeculae or grow around them in
an appositional fashion
-
-
-
-
- Key feature is the presence of osteoid and or bone produced
directly by tumor cells without interposition of cartilage
-
-
-
-
- Osteoblastic areas are often mixed with fibroblastic and
chondroblastic foci
-
-
-
-
- Tumor cells may grow in diffuse, nesting or pseudopapillary
arrangements
43. BONE-FORMING TUMORS
-
-
- OS cells usually exhibit strong AP activity, regardless of
their appearance
-
-
- Ultrastructurally, tumor cells resemble normal osteoblasts
-
-
- Consistently expresses Vimentin
-
-
- In some cases, they are positive for smooth muscle actin,
desmin, EMA, S-100 protein
-
-
- Osteonectin, osteocalcin, osteopontin bone morphogenetic
protein and bone GLA protein have been identified
immunohistochemically
44. BONE-FORMING TUMORS
-
-
-
-
- Well-differentiated intramedullary
-
-
-
-
- Others = parosteal (juxtacortical), periosteal
45. OSTEOSARCOMA GROSS SHOWING SKIP METASTASIS MICROSCOPIC
APPEARANCE 46. OSTEOSARCOMA TELANGIECTATIC VARIANT OF OSTEOSARCOMA
47. OSTEOSARCOMA JUXTACORTICAL OSTEOSARCOMA 48. BONE-FORMING
TUMORS
-
-
- Diagnosis: characteristic radiographic appearance, open biopsy,
needle biopsy, FNAB, frozen section.
-
-
- Therapy: amputation or disarticulation. At present,
limb-sparing procedures coupled with other therapeutic
modalities.
-
-
-
-
- Poor : presence of Pagets disease, multifocal OS,
chondroblastic type, Telangiectatic variant, elevated AP, low
postchemotherapy tumor necrosis, loss of heterozygosity of the RB
gene, HER2/neu expression, expression of P-glycoprotein
49. CARTILAGE-FORMING TUMORS
-
-
- A common benign cartilaginous tumor that occurs most frequently
in the small bones of the hands and feet, particularly the proximal
phalanges
-
-
- Microscopically, they are composed of mature hyaline cartilage.
Foci of myxoid degeneration, calcification, and endochondral
ossification are common
-
-
- Enchondromas begins in the spongiosa of the diaphysis from
which they expand and thin out the cortex
-
-
-
-
- Lesions with predominantly unilateral distribution are referred
to as Olliers disease
-
-
-
-
- Its association with soft tissue hemangiomas is known as
Maffuccis syndrome
50. Distribution according to the age and sex and the site of
the lesion. 51. CHONDROMA X-RAY GROSS MICROSCOPIC 52. Distribution
of multiple chondromas according to the age and sex of the patient.
53. MULTIPLE CHONDROMAS IN OLLIERS DISEASE. The left hand of this
27-year-old man contains multiple deforming chondromas. 54.
MULTIPLE CHONDROMAS. The tumors involve the bones of the second and
third rays with sparing of the joint spaces. 55. MAFFUCCIS
SYNDROME. Left multiple benign cartilage tumors involve the
metacarpals and phalanges of the first to third digits. Soft tissue
swelling and masses with calcified phleboliths are compatible with
soft tissue hemangiomas. Right Coronal short tau inversion recovery
image shows multiple high signal intensity lesions in phalanges of
the 2 ndand 3 rddigits and 2 ndmetacarpal consitent with
enchondromas. The small soft tissue mass in the 2 nddigit and
serpigenous vessels in the 2 ndand 3 rddigits are compatible with
hemangiomas. 56. CARTILAGE-FORMING TUMORS
-
-
- Most frequent benign tumor
-
-
- Usually asymptomatic, but may lead to deformity or interfere
with the function of adjacent structures such as tendons and blood
vessels
-
-
- Most common locations are metaphyses of the lower femur, upper
tibia, upper humerus and pelvis
-
-
- Average age of onset is 10 y/o, majority appears before the age
of 20
-
-
- Average greatest diameter is 4 cm but may reach 10 cm or
more
-
-
- A cap of cartilage covered by fibrous membrane continous with
the periosteum of the adjacent bone
-
-
- Microscopically, the cells resemble those of normal hyaline
cartilage. Eosinophilic, PAS-(+) inclusions may be seen in the
cytoplasm. The bulk of the lesion is composed of mature bone
trabeculae located beneath the cartilaginous cap and containing
normal bone marrow.
57. Distribution according to the age and sex and the site of
the lesion. 58. OSTEOCHONDROMA GROSS, CUT SECTION MICROSCOPIC 59.
The cartilage cap is somewhat thick but smooth. The chalky white
area are calcification. 60. OSTEOCHONDROMA. A cartilage cap
overlies cancellous bone of the stalk 61. CARTILAGE-FORMING
TUMORS
-
-
- Occurs predominantly in males under 20 y/o
-
-
- Usually arises in the epiphyseal end of long bones before the
epiphyseal cartilage has disappeared, particularly in the distal
end of femur, proximal end of humerus, and proximal end of
tibia
-
-
- X-ray: tumor is fairly well delimited and contains areas of
rarefaction
-
-
-
-
- the basic tumor cell is an embryonic chondroblast with only a
limited capacity for the production of cartilaginous matrix.
-
-
-
-
- Presence of occasional scattered giant cells
62. CARTILAGE-FORMING TUMORS
-
-
-
-
- Cells are usually polyhedral with round to indented nuclei.
Reticulin fibers surround each individual cell.
-
-
-
-
- Presence of small zones of focal calcification (chicken
wire)
-
-
- Diagnosis can be made by fine needle aspiration which will show
neoplastic chondroblast, multinucleated osteoclast-like giant
cells, and chondroid myxoid fragments
-
-
- Treatment is by curettement with bone grafting
63. Distribution according to the age and sex and the site of
the lesion 64. CHONDROBLASTOMA X-RAY GROSS 65. This chondroblastoma
arising from the temporal bone contains areas of spindled
mononuclear cells, hemosiderin deposition , and multinucleated
giant cells. 66. CHONDROBLASTOMA MICROSCOPIC 67. CARTILAGE-FORMING
TUMORS
-
-
- Usually occurs in long bones of young adults
-
-
- Radiographically, it is sharply defined and may attain a large
size
-
-
- Grossly, it is solid and yellowish white or tan, replaces bone
and thins the cortex.
-
-
- Microscopically, shows hypocellular lobules with a
chondromyxoid appearance separated by intersecting bands of
fibroblast-like spindle cells and osteoclasts
-
-
- Strong positivity to S-100 protein
-
-
- Treatment is by curettage with a recurrence rate of 25%
68. Distribution according to age and sex and the site of the
lesion. 69. CHONDROMYXOID FIBROMA X-RAY GROSS MICROSCOPIC 70.
Curetted fragments of a glistening blue-gray tumor. There is no
obvious evidence of cartilaginous tissue. 71. Macroglobular growth
pattern Micronodular growth pattern 72. CARTILAGE-FORMING
TUMORS
-
-
- A malignant tumor of cartilage-forming tissues
-
-
- Divided into conventional and variants
-
-
- Conventional chondrosarcoma can be
-
-
-
-
- Central = located in the medullary cavity, usually of flat or
long bone. X-ray show osteolytic lesion with splotchy calcification
with ill-defined margins, fusiform thickening of the shaft, and
perforation of the cortex
-
-
-
-
- Peripheral = may arise de novo or from the cartilaginous cap of
a preexisting osteochondroma
-
-
-
-
- Juxtacortical (periosteal) = involves the shaft of a long
bonecharacterized by a cartilaginous lobular pattern with areas of
splotchy calcification and endochondral ossification
73. CARTILAGE-FORMING TUMORS
-
-
- Microscopically, there is production of cartilaginous matrix
and the lack of direct bone formation by the tumor cells
-
-
- Soft tissue implantation following biopsy is a well known
complication
-
-
-
-
- Clear cell chondrosarcoma
-
-
-
-
- Dedifferentiated chondrosarcoma
-
-
-
-
- Mesenchymal chondrosarcoma
74. Distribution according to the age and sex and the site of
the lesion. 75. CHONDROSARCOMA GROSS APPEARANCES OF CHONDROSARCOMA
76. The tumor arose in the sternum. It has a characteristic
gray-blue appearance, is lobulated and has a chalky-white calcific
deposits. Cartilage tumors at this site are almost always
malignant. 77. CHONDROSARCOMA X-RAY, FEMUR 78. CHONDROSARCOMA
WELL-DIFFERENTIATEDCLEAR CELL VARIANT 79. GIANT CELL TUMOR
- Patients are over 20 years of age
- More common in women then men
- More frequently in Oriental than Western countries
- Classic location is epiphysis of long bone
- Affects more commonly the lower end of femur, upper end of
tibia, and lower end of the radius. It also occurs in the humerus,
fibula, and skull particularly the sphenoid bone.
- Multicentricity has been reported particularly in young
patients and in the small bones of hands and feet
80. Distribution by age and sex and the site of the lesion 81.
GIANT CELL TUMOR
-
-
- The typical appearance is that of an entirely lytic, expansile
lesion in the epiphysis, usually without peripheral bone sclerosis,
or periosteal reaction
-
-
- The size of the tumor varies; when large, it may be associated
with a pathologic fracture
-
-
- The cut surface is solid and tan or light brown, traversed by
fibrous trabeculae, and often contains hemorrhagic areas
-
-
- The cortex is thinned, but periosteal new bone formation is
rare
82. GIANT CELL TUMOR
-
-
- Two main components are stromal cells and giant cells
-
-
- The giant cells are usually large and have over twenty or
thirty nuclei, most of then are arranged toward the center.
-
-
-
- They resemble osteoclasts at all levels: ultrastructurally,
enzyme histochemically and immunohistochemical
-
-
-
- Result of fusion of circulating monocytes that have been
recruited into the lesion
-
-
-
-
- Autocrine or paracrine loop mediated by transforming growth
factor beta
83. GIANT CELL TUMOR
-
-
-
-
- 2. Production of osteoprotegerin ligand (a factor essential for
osteoclastogenesis)
-
-
-
-
- 3. Expression of the ligand for RANK (receptor activator of
nuclear factor Kappa B)
-
-
- Mononuclear stromal cells is the only proliferating element in
the lesion and the one exhibiting atypia in the rare cytologically
malignant examples of this tumor
-
-
-
-
- These changes may be focal, hence a thorough sampling is
required
-
-
-
-
- Produces type I & III collagen and has receptors for
PTH
84. GIANT CELL TUMOR X-RAY GROSS APPEARANCE 85. The tumor arises
in the distal radius and forms a large destructive mass. The
treatment was amputation. 86. GIANT CELL TUMOR MICROSCOPIC
APPEARANCE 87. GIANT CELL TUMOR
- Frequent positivity for S-100 protein
- Many benign lesions with giant cells have been diagnosed as
giant cell tumor in the past
-
- A diagnosis of a lesion other than GCT should be favored
if:
-
-
-
-
- 2. lesion is located in the metaphysis or diaphysis of a long
bone
-
-
-
-
- 4. lesion is located in the vertebrae, jaw, or bones of the
hands or feet
88. MARROW TUMORS
- 1. EWINGS SARCOMA/PRIMITIVE NEUROECTODERMAL TUMOR (PNET)
-
-
- Undifferentiated type of bone sarcoma in children
-
-
- Related to the neoplasm originally described in the soft
tissues as primitive (peripheral) neuroectodermal tumor
-
-
- Usually seen in patients between the ages of 5 and 20
years
-
-
- Clinically, the tumor may simulate OM because of pain, fever,
and leukocytosis
89. EWINGS/PNET
-
-
- Occurs most often in long bones (femur, tibia, humerus, and
fibula) and in the bones of the pelvis, rib, vertebrae, mandible
and clavicle
-
-
- It generally arises in the medullary canal of the shaft, from
which it permeates the cortex and invade the tissues
-
-
- Can present clinically as a soft tissue neoplasm with a normal
appearance of the underlying bone on plain x-ray films
-
-
- X-ray : cortical thickening and widening of the medullary
canal. With progression of the lesion, reactive periosteal bone may
be deposited in layers parallel to the cortex (onion-skin
appearance) or at right angle to it (sun-ray appearance)
90. EWINGS SARCOMA/PNET
-
-
- Consists of solid sheets of cells divided into irregular masses
by fibrous bands
-
-
- Individual cells are small and uniform
-
-
- The cells outline are indistinct, resulting in a syncitial
appearance
-
-
- The nuclei are round, with frequent indentations, small
nucleoli and variable but usually brisk mitotic activity
-
-
- There is well developed vascular network
-
-
- Pseudorosettes and rosettes arrangement of cells may be
seen
91. Distribution by age and sex and the site of the lesion 92.
EWINGS SARCOMA/PNET X-RAY GROSS APPEARANCE 93. EWINGS SARCOMA/PNET
MICROSCOPIC APPEARANCE 94. Prominent rosette formation occurs
throughout the tumor 95. The tumor cells show diffuse
immunoreactivity for CD99 96. EWINGS SARCOMA/PNET
-
-
- Cells contains large amounts of cytoplasmic glycogen --- (+)
PAS
-
-
- Ultrastructurally shows few dense core granules
-
-
- Immunohistochemically, positive for vimentin, LMW keratin, NSE,
protein gene product 9.5, Leu7, and neurofilaments
-
-
- Over 95% of cases show a reciprocal translocation 11;22
(q24;q12)
97. 98. 99. Fluorescence in situ hybridization (FISH) in Ewings
sarcoma 100. EWINGS SARCOMA/PNET
-
-
- Metastatic spread is to the lungs and pleura, other bones
(particularly the skull), CNS, and (rarely) regional LN
-
-
- About 25% of the patients have multiple bone and/or visceral
lesions at the time of presentation
-
-
-
-
- Combination of high-dose irradiation and multidrug chemotherapy
sometimes combined with limited surgery
101. MARROW TUMORS
-
-
- Can involve the skeletal system primarily or as a manifestation
of a systemic disease
-
-
-
-
- More common in adults than in children
-
-
-
-
- 60% of cases occurring in patients over 30 y/o
-
-
-
-
- Most cases involve the diaphysis or metaphysis og long bones or
vertebrae producing patchy cortical and medullary destruction
associated with minimal to moderate periosteal reaction
-
-
-
-
- The tumor is pinkish gray and granular, frequently extends into
the soft tissues and invades the muscle
102. MALIGNANT LYMPHOMA
-
-
- Radiographically, a combination of bone production and bone
destruction often involves a wide area of a long bone
-
-
- Microscopically, the appearance is similar to that of the large
cell lymphoma in nodal and other extranodal sites, some cases are
accompanied by prominent fibrosis
-
-
- The 5-year survival rate for localized B-cell lymphoma of bone
has ranged from 30-60%
-
-
- The stage of the disease is the single most important
prognostic determinator
103. MALIGNANT LYMPHOMA X-RAY MICROSCOPIC APPEARANCE 104.
MALIGNANT LYMPHOMA
-
-
- Produces radiographically detectable bone lesions in
approximately 15% of the patients
-
-
- Involvement is multifocal in about 60% of cases, most frequent
sites being vertebrae, pelvis, ribs, sternum, and femur
-
-
- Osseous lesions are often asymptomatic and in half of the cases
are not demonstrable radiographically
105. MALIGNANT LYMPHOMA
- Anaplastic large cell lymphoma
106. ACUTE LEUKEMIA
-
-
- Associated with radiographic abnormalities in the skeletal
system in 70-90% of cases
-
-
- Destructive bone lesions are extremely rare in the chronic
leukemias
107. VASCULAR TUMORS
-
-
- Often seen in the vertebrae as an incidental post-mortem
finding
-
-
- The most common locations are the skull, vertebrae, and
jaw
-
-
- Cut section has a currant jelly appearnce
-
-
- Microscopically, there is a thick-walled lattice-like pattern
of endothelial lined cavernous spaces filled with blood
-
-
- Multiple hemangiomas are mainly seen in children and are
associated in about half of the cases with cutaneous, soft tissue,
or visceral hemangiomas
108. VASCULAR TUMORS CAVRNOUS HEMANGIOMA OF BONE 109. VASCULAR
TUMORS
-
-
- Has microscopic similarities with skeletal angiomatosis
-
-
- It has a destructive character
-
-
- It results in reabsorption of a whole bone or several bones and
the filling of the residual spaces by a heavy vascularized fibrous
tissue
110. VASCULAR TUMORS
-
-
- Most cases are multiple and associated with soft tissue tumors
of similar appearance
-
-
- May erode the underlying bone
-
-
- Can present as a primary bone lesion, most common location is
the pelvis
111. VASCULAR TUMORS
- 6. EPITHELIOID HEMANGIOENDOTHELIOMA
-
-
- A borderline type of vascular neoplasm characterized
microscopically by the presence epithelial- or histiocyte-like
endothelial cells with abundant acidophilic and often vacuolated
cytoplasm, large vesicular nucleus, modest atypia, scanty mitotic
activity, inconspicous or absent anastomosing channels, recent and
old hemorrhage and an inconstant but sometimes prominent
inflammatory component rich in eosinophils
112. VASCULAR TUMORS EPITHELIOID HEMANGIOENDOTHELIOMA 113.
VASCULAR TUMORS
-
-
- Malignant hemangioendothelioma, hemangioendothelial
sarcoma
-
-
- Exhibits obvious atypia of the tumor cells, formation of solid
areas alternating with others with anastomosingvascular channels,
and foci of necrosis and hemorrhage
-
-
- Multicentricity is common
-
-
- Distant metastasis are common, particularly lungs
114. METASTATIC TUMORS
-
-
- In most cases the lesions are multiple
-
-
- More than 80% arises from the breast, lung, prostate, thyroid,
or kidney
-
-
- These metastases can be accompanied by visceral deposits or
represent the only apparent site of dissemination
-
-
- Soft tissue sarcomas rarely metastasize to the bones except
embryonal rhabdomyosarcoma in children
-
-
- They are usually osteolytic but maybe osteoblastic or
mixed
115. METASTATIC TUMORS
-
-
- The mechanism is thought to be the production of bone growth
factors by tumor cells, such as TGF-beta, fibroblast growth factor,
and bone morphogenetic proteins
-
-
- Treatment is relief of pain and to prevent fracture of
weight-bearing bones
116. TUMORLIKE LESIONS
-
-
- Usually occur in long bones, most often in the upper portion of
the shaft of the humerus and femur
-
-
- Also seen in the short bones, calcaneus
-
-
- Mostly affects males and are seen in patients under 20
years
-
-
- Usually are advanced when first seen, most are centered in the
metaphysis and they migrate away from the epiphyseal line
117. TUMORLIKE LESIONS
-
-
- The cysts contains a clear or yellow fluid that is lined by a
smooth fibrous membrane
-
-
- Maybe hemorrhagic if previous fracture occurred
-
-
- Microscopic: well-vascularized connective tissue, hemosiderin
and cholesterol clefts are frequent
-
-
- Treatment of choice is curettement and replacement of the cyst
with bone chips
118. SOLITARY BONE CYST X-RAY GROSS APPEARANCE 119. TUMORLIKE
LESIONS
-
-
- Usually seen in patients between 10 and 20 years of age
-
-
- Occurs mainly in the vertebrae and flat bones but can also
arise in the shaft of long bones
-
-
- Multiple involvement is common in the vertebral lesions
-
-
- X-ray: shows eccentric expansion of the bone with erosion and
destruction of the cortex and a small area of periosteal new bone
formation
120. TUMORLIKE LESIONS
-
-
- GROSS: it forms a spongy hemorrhagic mass covered by a thin
shell of reactive bone, which may extend into the soft tissue
-
-
-
-
- show large spaces filled with blood
-
-
-
-
- They do not contain endothelial lining but are rather delimited
by cells with similar features to fibroblast, myofibroblasts, and
histiocytes
-
-
-
-
- A row of osteoclasts is often seen immediately beneath the
surface
-
-
-
-
- There is significant deposition of generated calcifying
fibromyxoid tissue.
121. TUMORLIKE LESIONS
-
-
- Pathogenesis is still unknown
-
-
- In a few cases, the lesion is preceded by trauma with fracture
or subperiosteal hematoma
-
-
- It may also arise in some preexisting bone lesion as a result
of changed hemodynamics
-
-
- Insulin-like growth factor-I may play in its pathogenesis
-
-
- Ddx: chondroblastoma, GCT, fibrous dysplasia, nonossifying
fibroma, osteoblastoma, chondrosarcoma
-
-
- Treatment : en bloc resection or curettage with bone
grafting
122. Distribution by age and sex and the site of the lesion 123.
ANEURYSMAL BONE CYST X-RAY GROSS APPEARANCE 124. ANEURYSMAL BONE
CYST MICROSCOPIC APPEARANCE 125. TUMORS OF SKELETAL MUSCLES
-
-
- Most common soft tissue sarcoma of childhood and
adolescence
-
-
- Usually appears before the age of 20
-
-
- Commonly occurs in the head and neck or genitourinary tract,
extremities
126. TUMORS OF SKELETAL MUSCLES
-
-
- In the more common translocation, t(2;13), the PAX3 gene on
chromosome 2 fuses with the FKHR gene on chromosome 13
-
-
- PAX3 gene functions upstream of genes that control muscle
differentiation
-
-
- Pathogenesis of tumor involves dysregulation of muscle
differentiation by the chimeric PAX3-FKHR protein
127. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- *** Diagnostic cell is the RHABDOMYOBLAST
-
-
- = contains eccentric eosinophilic granularcytoplasm rich in
thick and thin filaments
-
-
- = may be round or elongated (tadpole or strap cells
-
-
- = Ultrastructurally, contain sarcomeres
-
-
- = Immunohistochemically, they stain withantibodies to the
myogenic markers desmin,MYOD1 and myogenin
128. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- EMBRYONAL RHABDOMYOSARCOMA
-
-
-
-
- Most common type, accounting to 60%
-
-
-
-
- Includes Sarcoma Botryoides
-
-
-
-
- Occurs in children under 10 years of age
-
-
-
-
- Typicallyarises in the nasal cavity, orbit, middle ear,
prostate and paratesticular region
-
-
-
-
- Allelic loss of chromosome 11p15.5 as its major genomic
abnormality
129. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- EMBRYONAL RHABDOMYOSARCOMA
-
-
-
-
- Grossly, they present as a soft gray infiltrative mass
-
-
-
-
- Microscopically, the tumor cells mimic skeletal muscle cells at
various stages of embryogenesis and consist of sheets of both
malignant round and spindled cells in a variably myxoid stroma
-
-
-
-
- Sarcoma botryoides grows in a polypoid fashion, producing the
appearance of a cluster of grapes protruding into a hollow
structure such as the bladder or vagina
130. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- ALVEOLAR RHABDOMYOSARCOMA
-
-
-
-
- Most common in the early and mid-adolescence and usually arises
in the deep musculature of the extremities
-
-
-
-
- Histologically, the tumor is traversed by a network of fibrous
septae that divide the cells into clusters or aggregates; as the
central cells degenerate and drop out, resembles pulmonary
alveolae
-
-
-
-
- Tumor cells are moderate in size and have little cytoplasm
131. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- ALVEOLAR RHABDOMYOSARCOMA
-
-
-
-
- Cells with cross-striations are identified in about 25% of
cases
-
-
-
-
- Cytogenetic studies show a t(2;13) or t(1;13) chromosomal
translocations
132. TUMORS OF SKELETAL MUSCLES
- RHABDOMYOSARCOMA MORPHOLOGY
-
-
- PLEOMORPHIC RHABDOMYOSARCOMA
-
-
-
-
- Characterized by numerous large, sometimes multinucleated,
bizarre eosinophilic tumor cells
-
-
-
-
- Arises in the deep soft tissue of adults
-
-
-
-
- Resemble malignant fibrous histiocytoma histologically
133. TUMORS OF SKELETAL MUSCLES
-
-
- Usually treated with a combination of surgey and chemotherapy
with or without radiation
-
-
- Histologic variant and location of the tumor influence
survival
-
-
- Sarcoma botryoides have the best prognosis, followed by
embryonal, pleomorphic, and alveolar variants
-
-
- Overall prognosis for children is good = 65%;less for
adults
134. TUMORS OF SMOOTH MUSCLE
-
-
- Benign smooth muscle tumor, commonly arises in the uterus
-
-
- May also arise in the erector pili muscles found in the skin,
nipples, scrotum and labia and less in the deep soft tissues
-
-
- Multiple lesions is thought to be hereditary and transmitted as
an autosomal dominant trait
-
-
- Occur in adolescence and early adult life
135. TUMORS OF SMOOTH MUSCLE
-
-
- Tumors are usually not larger than 1 to 2 cm in greatest
dimension
-
-
- Composed of fascicles of spindle cells that tend to intersect
each other at right angles
-
-
- Tumor cells have blunt-ended, elongated nuclei and show minimal
atypia and few mitotic figures
136. TUMORS OF SMOOTH MUSCLE
-
-
- Account for 10 to 20% of soft tissue sarcomas
-
-
- Occurs in adults, women>men
-
-
- Most develop in the skin and deep soft tissues of the
extremities and retroperitoneum
-
-
- Present as painful, firm masses
-
-
- Retroperitoneal tumors may be large and bulky and cause
abdominal symptoms
137. TUMORS OF SMOOTH MUSCLE
-
-
- Histologically, characterized by malignant spindle cells that
have cigar-shaped nuclei arranged in interweaving fascicles
-
-
- Immunologically, they stain with antibodies to vimentin, actin,
smooth muscle actin and desmin
-
-
- Treatment depends on the size, location and grade of the
tumor
138. Thank You