Bone and Cartilage Tumors Benign and Malignant

Bone and Cartilage tumors
Benign and Malignant
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Benign tumors of bone.
 OSTEOMA: involves the skull and facial
bones, w/extremely slow growth rate
(HYPEROSTOSIS FRONTALIS)it may extends
into the orbit or sinuses(Gardner´s
syndrome)Peak incidence: 40-50 years of age
 OSTEOID OSTEOMA:benign, painful growth
of the diaphysis of a long bone (often the tibia
or femur)
- Age : 5-25 years, mostly males
Benign tumors of bone.
-Symptoms: Pain is worse at night and is
relieved with aspirin
-X rays: central radiolucency surrounded by a
sclerotic rim.
-Micro: small (< 2 cms) lesion of the cortex
with central nidus of osteoid surrounded by
dense sclerotic rim of reactive cortical bone.
Benign tumors.
 OSTEOBLASTOMA: Similar to an osteoid
osteoma but larger than 2 cms in size and
often involving vertebrae.
Benign tumors.
 OSTEOCHONDROMA (exostosis)
-Benign bone metaphyseal growths capped with
cartilage that originates from epiphyseal growth
plate.
-It may affects adolescent males as a firm, solitary
growth at the ends of long bones.
-It may be asymptomatic or cause pain, producing
deformity, and can undergo with malignant
transformation ( rarely)
Osteochondroma
Benign tumors, Cartilage(cont.)
OSTEOCHONDROMATOSIS ( Multiple
hereditary exostosis)
-Characterized with multiple, often symmetric,
osteochondromas.
ENCHONDROMA: benign cartilaginous growth
within the medullary cavity of bone, usually
involving the hands and feet.
-Is a typical solitary lesion often asymptomatic and
require no treatment.
Enchondroma
Benign tumors. Cartilage
 MULTIPLE ENCHONDROMAS
(Enchondromatosis)
 OLLIER DISEASE: a non hereditary
syndrome,with multiple enchondromas in hands
and feet.
 It may presents with pain and spontaneous Fxs
 It may undergo malignant transformation to
chondrosarcoma.
Benign tumors. Cartilage(cont.)
 MAFUCCI SYNDROME
 Multiple enchondromas
 Soft tissue hemangiomas
 Increased risk of malignant transformation,
ovarian Ca. and brain gliomas.
Maffucci Syndrome
Maffucci Syndrome
Malignant Tumors of Bone.
 OSTEOSARCOMA ( Osteogenic sarcoma)
- Most common primary malignant tumor of

bone
-Males> females. Most occur in teenagers ( ages
10-25)
-Patients with familial retinoblastoma have a high
risk
-Clinical features: localized pain and swelling
Malignant tumors of bone
OSTEOSARCOMA(cont.)
 Classic X ray findings:
-Codman’s triangle ( periosteal elevation)
-Sunburst pattern
-Bone destruction
-Grossly: often involves the metaphyses of long
bones, usually around the knee (distal femur/pro
ximal tibia.) and it may be seen as a large, firm,
white mass with necrosis and hemorrhage.
Malignant Tumors of Bone
.Osteosarcoma
 Micro: Anaplastic cells producing osteoid and
bone.
 Tx: surgery/ chemotherapy
 Prognosis: poor (hematogenous metastastasis to
the lungs is a common complication)
 “SECONDARY” OSTEOSARCOMAS. Occur in
elderly persons, associated with Paget’s disease,
irradiation and chronic osteomyelitis
 Highly agressive.
Osteosarcoma
Osteosarcoma
CHONDROSARCOMA
 Malignant tumor of cartilage
-Males> females age 30-60
-Tumor may arise primarily or secondary to a preexisting
enchondroma,exostosis or Paget’s disease.
-Clinical presentation: progressively enlarging mass with
pain and swelling, that typically involves the pelvic bones,
spine, and shoulder girdle.
-Micro: composed of atypical chondrocytes and
chondroblasts, often with multiple nuclei in a lacunar
structure
Chondrosarcoma
GIANT CELL BONE TUMOR
(Osteoclastoma)
 Uncommon malignant neoplasm containing multinucleated
giant cells admixed with stromal cells. Females>males,
with ages between 20-50 years
 Clinical features: bulky mass with pain and Fx.
 X rays: expanding lytic lession surrounded by a thin rim of
bone.
 It may have also a soap bubble appearance
Gross: often involves the epiphyses of long bones, usually
around the knee ( distal femur and proximal tibia) seen a
red brown mass with cystic degeneration.
GIANT CELL TUMOR(cont.)
 Micro: multiple osteoclast-like giant cells
that are distributed within a background of
mononuclear stromal cells.
 Tx: surgery/ curetage or block resection
 Prognosis: locally aggressive with a high
rate of recurrence.
Giant cell tumor
EWING SARCOMA
 Malignant neoplasm of undifferentiated cells
arising within the marrow cavity
Males are affected slightly more often than
females, most occur in teenagers ( 5-20)
 Clinical features: pain , swelling and tenderness
 Classic translocation t11;22 which produces the
EWS- FL11 fusion protein
 X-ray: concentric onion skin layering of new
periosteal bone.
Ewing sarcoma
EWING SARCOMA(cont.)
 Gross: often affects the diaphyses of long bones with most
common sites like femur, pelvis and tibia seen a white tan
mass with necrosis and hemorrhage.
 Micro: sheets of undifferentiated small round blue cells
resembling lymphocytes.
 Characteristic Homer- Wright pseudorosettes
 Frequently the tumoral cells erode cortex and periosteum
and invade surrounding tissues.
 Tx.: chemotherapy, surgery and/ or radiation
 Prognosis: 5 year survival rate of 75%
NEUROMUSCULAR DISORD.
 The “motor unit”consists of a lower motor
neuron(anterior horn cell or neuron in cranial
nerve nuclei), its axon and the muscle fibers
innervated by it. The number of muscle fibers
innervated varies from a few fibers(oculo-motor
muscles) to several hundreds(extremity muscles).
Muscles fibers of one motor unit are scattered in a
wide area in a random fashion(“checkerdboard”).
 Diseases can be classified as involving:
A. Motor neuron B. Peripheral nerves
C.Neuro muscular junction
D. Muscles
NEUROMUSCULAR DIS.(cont)
 A. DISEASES OF MOTOR NEURON.
-Etiology of most of the motor neuron dis.
(AML, progressive muscular atrophy) is not
known, but may be caused by several agents:
adriamycin, vincristine,
aluminiumperikaryon(neuronal cell
body) primarily affected w/loss of
microtubules and nuclear displacement +
increased cytoplasmic neurofilaments or tangled
masses of neurofilaments. Viral
infections(Polyomyelitis, Herpes encephalitis,
Varicella-zoster) may also affect the motor neuron
or sensory ganglia.www.freelivedoctor.com
 B. DISEASES OF PERIPHERAL NERVES
I. Axonal degeneration(axonal
neuropathy) a. Wallerian
degeneration(crush/cut along a myelinated fiber)
b.
Axonal degeneration caused by other diseases
(less axoplasm leakage+ more
inflammation + chronic evolution):
1.Proximal
axonal degeneration: intoxic. w/
IDPN(BB-Iminodipropionitrite)shrinkage of
distal axons due to focal proximal blockage.
www.freelivedoctor.com 2.Distal
 DISEASES OF PERIPHERAL...(cont.)
Many diseases w/earlier changes in the
most distal portion of axonsslow spread to
proximal structuresperikaryon cannot support
the terminal axon, vgr.:
--Hereditary
neuropathies.
-HSMN I (Charcot-Marie-Tooth)disease, the
MOST common of these, inherited as autosomal-
dominant is usually present
in childhood/early
adulthood(PMP 22 gene/17p11.2-p12 locus),
characterized by progressive atrophy of leg mus-
--Hereditary neuropathies...
-HSMN II(CMT2A) also AD, with similar
manifestations than CMT I but without nerve
enlargement and presentation at a later age.
Linked to chromosome 1p35-p36.
-HSMN III(Dejerine-Sottas disease) is an AR
condition that begins slowly in early childhood w/
delayed acquisition of motor skills and
involvement of muscles of limbs and trunkenlar
gement of nerves easy to
detect. Genetic heteroge nicity(PMP
22, MPZ, PRX and EGR2).
--Acquired metabolic/toxic neuropathies.
-Diabetic peripheral neuropathy:
w/symme tric neuropathy
involving distal sensory/mo tor
nerves + dysfunction of autonomic nervous
system(20-40% of cases).It can also present as a
single peripheral or cranial
neuroneuropathy(oculomotor nerve).
-Metabolic/nutritional: chronic liver
disease,resp. insuff., renal failure, thiamine
def.,Vit.B12,B6,E. Chronic
alcoholism, etc. www.freelivedoctor.com
STRIATED MUSCLE DISORDERS
(Dystrophies, Myositis and Tumors)
 INFLAMMATORY DISORDERS.
 POLYMYOSITIS:
 It may affect adults, females with bilateral
progressive, proximal muscle weakness
 Micro: endomysial lymphocytic
inflammation (mostly cytotoxic T8)
 Skeletal muscle fiber degeneration and
regeneration.
DERMATOMYOSITIS.
 It may affect children or adults, females,with bilateral and
proximal muscle weakness
 Also: skin rash of the upper eyelids , periorbital edema
 Micro: perimysial and vascular lymphocytic inflammation
with perifascicular fiber atrophy
 Skeletal muscle fiber degeneration and regeneration.
 Increased risk of lung, stomach, ovarian and breast
cancers.
Dermatomyositis
MUSCULAR DYSTROPHIES
 DUCHENNE MUSCULAR DYSTROPHY
 MOST COMMON and severe form of muscular dystrophy.
 X linked inheritance
 Dystrophin gene in Xp 21
(Mutation results in a virtual absence of dystrophin protein)
 Normal at birth with onset of symptoms by age 5 , with progressive muscular weakness
of proximal ,shoulder and pelvic girdles.
 CALF PSEUDOHYPERTROPHY
 Heart failure and arrhytmias may occur
 Progressive respiratory failure and pulmonary infections
 Increased serum creatine kinase
 Muscle fibers of various sizes , necrosis, degeneration and regeneration fibers
 Fibrosis
 Fatty infiltration.
Duchenne muscular dystrophy
 Dx: muscle biopsy with immunostains
shows decreased dystrophin protein
 DNA analysis by PCR.
Duchenne muscular dystrophy
BECKER MUSCULAR
DISTROPHY
 It is a less common condition

 The observed mutation produces an altered
dystrophin protein
 Later onset with variable progression
 Cardiac involvement is rarely seen
 Patients have a relatively normal life span
MUSCULARY
DISTROPHY(cont.)
-Inclusion body myositis.
-Myasthenic Syndromes
-Inflammatory Neuropathies.
SOFT TISSUE TUMORS.
 Adipose tissue.: Lipomas –Liposarcomas
 Fibrosarcoma
 Rhabdomyoma , rhabdomyosarcoma
 Smooth muscle
 Vascular tumors
 Peripheral nerve tumors.
SOFT TISSUE TUMORS
 RHABDOMYOSARCOMA (cont.)
 Dx:
-Excisional biopsy
-Immunochemistry
+vimentin
+desmin
+actin
+myoglobin
SOFT TISSUE TUMORS
 SMOOTH MUSCLE TUMORS.
1. Leiomyoma
2. Leiomyosarcoma
 VASCULAR TUMORS
1. Hemangiomas
2. Angiosarcomas
 SYNOVIALSARCOMA

Bone and Cartilage Tumors Benign and Malignant

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Bone and Cartilage Tumors Benign and Malignant

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Bone and Cartilage tumors

Benign and Malignant

- Most common primary malignant tumor of

 It is a less common condition

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