Bone and Cartilage tumors Benign and Malignant

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Bone and Cartilage tumors Benign and Malignant

• Department of Pathology

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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(Gardners
• syndrome)Peak incidence 40-50 years of age
• OSTEOID OSTEOMAbenign, painful growth of the
  diaphysis of a long bone (often the tibia or
  femur)
• - Age 5-25 years, mostly males

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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 (lt 2 cms) lesion of the cortex
  with central nidus of osteoid surrounded by dense
  sclerotic rim of reactive cortical bone.

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Benign tumors.

• OSTEOBLASTOMA Similar to an osteoid osteoma but
  larger than 2 cms in size and often involving
  vertebrae.

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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)

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Osteochondroma
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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.

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Enchondroma
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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.

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Benign tumors. Cartilage(cont.)

• MAFUCCI SYNDROME
• Multiple enchondromas
• Soft tissue hemangiomas
• Increased risk of malignant transformation,
  ovarian Ca. and brain gliomas.

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Maffucci Syndrome
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Maffucci Syndrome
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Malignant Tumors of Bone.

• OSTEOSARCOMA ( Osteogenic sarcoma)
  
• - Most common primary malignant tumor of
  bone
• -Malesgt females. Most occur in teenagers (
  ages 10-25)
• -Patients with familial retinoblastoma have a
  high risk
• -Clinical features localized pain and swelling

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Malignant tumors of bone OSTEOSARCOMA(cont.)

• Classic X ray findings-Codmans 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.

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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 Pagets disease,
  irradiation and chronic osteomyelitis
• Highly agressive.

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Osteosarcoma
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Osteosarcoma
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CHONDROSARCOMA

• Malignant tumor of cartilage
• -Malesgt females age 30-60
• -Tumor may arise primarily or secondary to a
  preexisting enchondroma,exostosis or Pagets
  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

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Chondrosarcoma
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GIANT CELL BONE TUMOR (Osteoclastoma)

• Uncommon malignant neoplasm containing
  multinucleated giant cells admixed with stromal
  cells. Femalesgtmales, 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.

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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.

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Giant cell tumor
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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 t1122 which produces the
  EWS- FL11 fusion protein
• X-ray concentric onion skin layering of new
  periosteal bone.

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Ewing sarcoma
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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

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NEUROMUSCULAR DISORD.

• The motor unitconsists 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

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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(neur
  onal cell body)
  primarily affected w/loss of microtubules and
  nuclear displacement increased cytoplasmic
  neurofilaments or tangled masses of neurofila-
  ments. Viral infections(Polyomyelitis, Herpes
  encephalitis, Varicella-zoster) may also affect
  the motor neuron or sensory ganglia.

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NEUROMUSCULAR DISORD.

• 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-Iminodipropioni
  trite)?shrinkage of distal axons due to focal
  proximal blockage.
  2.Distal
  axonal degeneration earliest changes
  occur in the most distal portion of axons

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NEUROMUSCULAR DISORD.

• 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-
  cles,foot
  drop/deformed feet w/less sensory defect
  
  

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NEUROMUSCULAR DISORD.

• DISEASES OF PERIPHERAL...(cont.)
  --Hereditary neuropathies...
  -HSMN II(CMT2A) also AD,
  with similar manifestations than CMT I but
  without nerve enlargement and presentation at a
  la- ter 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).

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NEUROMUSCULAR DISORD.

• DISEASES OF PERIPHERAL...(cont.)
  --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.

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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.

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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.

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Dermatomyositis
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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.

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Duchenne muscular dystrophy

• Dx muscle biopsy with immunostains shows
  decreased dystrophin protein
• DNA analysis by PCR.

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Duchenne muscular dystrophy
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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

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MUSCULARY DISTROPHY(cont.)

• -Inclusion body myositis.
• -Myasthenic Syndromes
• -Inflammatory Neuropathies.

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SOFT TISSUE TUMORS.

• Adipose tissue. Lipomas Liposarcomas
• Fibrosarcoma
• Rhabdomyoma , rhabdomyosarcoma
• Smooth muscle
• Vascular tumors
• Peripheral nerve tumors.

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SOFT TISSUE TUMORS

• RHABDOMYOSARCOMA (cont.)
• Dx
  -Excisional
  biopsy
  -Immunochemistry
  vimentin
  desmin
  
  actin
  myoglobin

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SOFT TISSUE TUMORS

• SMOOTH MUSCLE TUMORS.
  1. Leiomyoma
  2. Leiomyosarcoma
• VASCULAR TUMORS
  1. Hemangiomas
  2. Angiosarcomas
• SYNOVIALSARCOMA

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