Mendy – Patho gross : sponge-like

BONES JOINTS
& SOFT TISSUE micro : thin trabeculae
Garry R. Gonzales, RMT, MD, DPSP
Anatomic & Clinical Pathologist clin : pain, lordosis, kyphoscoliosis

2 TYPES OF BONES
1. woven - weaved DISEASES CAUSED BY OSTEOCLAST
2. lamellar – layered DYSFUNCTION

*** The presence of woven bone in adult is OSTEOPETROSIS aka Marble bone disease,
indicative of a pathologic state, but is not Albers-Schonberg disease
pathognomonic or diagnostic of a particular – diffuse symmetrical skeletal sclerosis
disease. - “stone-like but brittle”  fracture

DEVELOPMENTAL ABNORMALITIES: Pathology: unknown

1. CRANIORASCHISIS – failure of
closure of the spinal Gross : lack medullary canal (filled with primary
column& skull (malformation) spongiosa) and the ends of the long bones
2. ACHONDROPLASIA – most are bulbous (Erlen-Meyer Flask Deformity)
common disease of the
growth plate Clinical features:
- major cause fractures
of dwarfism. anemia
- autosomal Hepatosplenomegaly
dominant
3. THANATOTROPIC DWARFISM
– most common lethal form of Treatment:
dwarfism affecting 1 in every - bone marrow transplant
20,000 live births.
PAGET’s DISEASE (Osteitis Deformans)
“collage of matrix madness”
DISEASES WITH ABNORMAL METABOLISM gain in bone mass
OF COLLAGEN stages:
1. osteolytic stage
1. TYPE 1 Collagen Disease/Deficiency 2. Mixed osteoclastic-blastic stage
(osteogenesis imperfecta) 3. burnt-out quiescent osteosclerotic
- “too little bone” stage
- characterized by skeletal fragility.
- Acquired, post natal fracture,
blue sclera & dental imperfection. incidence: mid adulthood, usually asymptomatic

2. TYPE 2 – uniformly fatal in utero Pathology: Viral (?)

- characterized by extraordinary
bone fragility with Microscopic : mosaic pattern
multiple fractures
occurring when the fetus is still within Clinical features: most common site : axial
the womb. skeleton (may be monostotic or polyostotic)

OSTEOPOROSIS – reduction in bone mass Lab : increased ALP

causes: Complication: Development of sarcoma

post-menopausal
senility
decreased physical activity DISEASES ASSOCIATED WITH ABNORMAL
genetic MINERAL HOMEOSTASIS
nutritional 1. Rickets
hormonal (estrogen deficiency) 2. Osteomalacia

1
 -1 & 2 Defect in matrix Epiphysis  chondroblastoma & GCT
mineralization
3.Hyperparathyroidism – entire skeleton Diaphysis  Ewing’s sarcoma,
is adamantinoma, osteofibrous
affected dysplasia
-
“brown tumor” BONE FORMING TUMORS

Osteoma

FRACTURES Osteoid osteoma

Osteosarcoma

OSTEONECROSIS (vascular necrosis) OSTEOMA

– thrombi, fracture, embolus - bosselated, sessile tumors
- skull & facial bone
INFECTIONS - usually solitary (Gardner’s syndrome
1. PYOGENIC if multiple)
Mode of spread: - slow growing
- hematogenous - do not transform into osteosarcoma
- contiguous
- direct implantation OSTEOID OSTEOMA
- less than 2cm, painful
Organisms - M > F w/ predilection for appendicular
S. aureus – most common skeleton
H. influenzae & group B streptococcus – - involves the spine more frequently
neonates unlike osteoma
Salmonella – patients with sickle cell - non responsive to salicylates
disease.
- Gross : round, oval, well-circumscribed
2. TB OSTEOMYELITIS - Micro : haphazardly interconnecting
- occurs in 1-3% of patients with pulmonary trabeculae of woven
or extrapulmonaty TB bone that are rimmed by
- solitary osteoblast. The
- spine, knee, hips intertrabecular space are filled by
vascular loose
3. SKELETAL SYPHILIS connective tissue.
- congenital syphilis
- saber-shin deformity Nidus –is the actual tumor that manifests
- silver stain radiographically as small round and
luscent.
BONE TUMORS (in descending order of
frequency)
Osteosarcoma
Chondrosarcoma OSTEOSARCOMA
Ewing’s sarcoma - most common primary malignant
bone tumor
*** Benign >>> Malignant - bone producing tumor
- origin : metaphysic of long bone (50%
Age: of cases)
benign more common in the first 3 decades - knee – 60%
of life - pelvis – 15%
Malignant – adult - 10% - shoulder
- 08% - jaw
ORIGIN OF TUMORS:
- Gross : large, bulky, gritty, grey-white with
Metaphysis  osteosarcoma areas of hemorrhage &
necrosis

2
- Mirco - Olliers disease = multiple
1. pleomorphic, large hyperchromatic enchondromatosis
nuclei with bizaare tumor giant cells &
frequent metastasis - Maffucis Syndrome = enchondroma +
2. formation of bone by tumor cells hemangioma
3. have coarse, lacelike architecture - have increased risk of
developing other type of
*** when malignant cartilage is abundant, the carcinoma like
tumor is called CHONDROBLASTIC 1. ovarian carcinoma
SARCOMA. 2. brain glioma

Clinical Features: painful, progressively - Gross : small (<3cm), well circumscribed,

enlarging mass. grey-blue, translucent
Fracture - Micro : nodule of hyaline cartilage encased
90% have by a thin layer of reactive bone
pulmonary - Clinical Feature: most are asymptomatic
metastasis (incidental finding)
- Radiologic Finding: “ring sign” – circumscribed
Radiologic Findings: Codman’s triangle – oval luscency surrounded by a
characteristic but not diagnostic. thin rim of radiodense bone.

Chondroblastoma
- represents < 1% of bone tumor
- knee with predilection in epiphysis

CARTILAGE FORMING TUMORS - Micro : cellular, compact, polyhedral

Benign >>> Malignant
Osteochondroma
Chondroma chondroblasts with well defined cell
Chondroblastoma borders & pink
Chondromyxoid fibroma moderate amount of
Chondrosarcoma cytoplasm.
- hyperlobated nuclei with
Osteochondroma longitudinal groove
- “exostosis” - frequent MF & necrosis
- benign “cartilage-capped” overgrowth - produces a “chicken wire
that is attached to the skeleton pattern” when the matrix
- arise from metaphysic calcifies.

- Gross : mushroom shaped - Clinical Features : painful, may cause

- Micro : benign cartilage delineated by effusion
perichondrium
- Clinical feature: Chondromyxoid fibroma
- slow growing, painful, - rarest of all cartilagenous tumors
- stops growing at the time of growth - mistaken for sarcoma
plate closure - M>>> F; 20’s
- metaphysis
- Gross : 3-8 cms, well circumscribed, solid &
glistening tan grey.
- Micro : poorly formed nodules of hyaline
CHONDROMA cartilage & myxoid tissue
- “enchondromas” delineated by fibrous septae.
- most common intraosseous cartilage - cellularity varies – greatest in
tumor the periphery
- benign tumor of the hyaline cartilage - stellate cells within myxoid
- arise in medullary cavity stroma
- affects short tubular bones of the - nuclei show varying amount
hands and feet. of atypia with
hyperchromatic nuclei.

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- Clinical feature: localized, dull, achy pain
Site: central portion of the skeleton EXCEPT
Chondrosarcoma clear cell variant (originates in
- half as frequent as osteosarcoma epiphysis of long bones.
- 2nd most common malignant matrix : rarely involves the distal extremities.
producing tumor of bone
- M >>> F ; 40’s Clinical features : painful, progressively
- Subclassification of Chondrosarcoma enlarging mass.
according to site:
1. intramedullary - X-ray : The more radioluscent the tumor the
2. juxtacortical greater the likelihood it is high grade (destroy
the cortex)
Histologic classification of chondrosarcoma:
1. conventional (hyaline or myxoid) - Metastasis : Lung & skeleton
2. clear cell
3. dedifferentiated Prognosis: (5 year survival rate)
4. mesenchymal variants Grade 1 – 90% (non- metastasizing)
Grade 2 – 81%
Gross : large, bulky, grey-white, translucent, Grade 3 – 43% (70% metastasize)
glistening mass
Treatment: wide excision
Micro : infiltration of the marrow by malignant chemotherapy
chondrocytes
- spotty calcification are FIBROUS & FIBRO-OSSEOUS TUMORS
typically present & Fibrous Cortical Defect & Non ossifying
central necrosis may create Fibroma
cystic spaces.
Fibrous Dysplasia
Grading:
LOW GRADE (Grade 1) Fibrosarcoma & MFH
- mild hypercellularity
- mild atypia with small nucleoli OSSIFYING FIBROMA
- rare to no MF adolescent
Gross : grey to brown tissue & cellular
HIGH GRADE (Grade 3) lesions composed of fibroblast &
- marked cellularity histiocyte
- extremely pleomorphic with Micro : storiform pattern + histiocyte or
bizarre giant cells fibroblast

*** Pure grade 3 chrondrosarcoma are Clinical : Usually asymptomatic

uncommon.
Such malignant cartilage is frequently a FIBROUS DYSPLASIA
component benign tumor-like lesion
of chondroblastic osteosarcoma characterized as a localized developmental
arrest; all of the components of normal
bone are present, but they do not
differentiate into mature structures.

Clinical Patterns:
- CLEAR CELL CHONDROSARCOMA a. MONOSTOTIC FIBROUS
- (hallmark) sheets of large malignant DYSPLASIA
chondrocytes with abundant clear cytoplasm, b. POLYOSTOTIC FIBROUS
numerous osteoclast-type giant cells and DYSPLASIA
intralesional reactive bone formation. WITHOUT ENDOCRINE
DYSFUNCTION
- MESENCHYMAL CHONDROSARCOMA c. FIBROUS DYSPLASIA
- composed of islands of well ASSOCIATED WITH CAFÉ-
differentiated hyaline cartilage surrounded by AU-LAIT SKIN PIGMENTATION &
sheets of small round cells.

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 ENDOCRINOPATHIES (McCune-
Albright Syndrome) - Gross : well circumscribed,
intramedullary large lesion. May
MONOSTOTIC FIBROUS DYSPLASIA expand and distort the bone
- most common clinical pattern (70% of
all cases) - Microscopic ; fibroblastic
- M = F, usually in early adolescent and proliferation with
often stops growing at the trabeculae resembling Chinese letters &
time of growth plate closure. LACKS OSTEOBLASTIC
RIMMING.
- Sites (in descending order)
1. ribs Other common findings:
2. femur 1. cystic degeneration
3. tibia 2. hemorrhage
4. jaw 3. foamy macrophage
5. calvaria
6. humerus X-ray : ground glass appearance

POLYOSTOTIC FIBROUS DYSPLASIA Clinical : sexual precocity, hyperthyroidism,

WITHOUT ENDOCRINE DYSFUNCTION pituitary adenoma secreting GH,
- affects many but not all bones Primary adrenal hyperplasia.
- Age : younger age group than MFD &
may continue to grow until Complication : transformation into sarcoma
adulthood. ( i.e. osteosarcoma & MFH)
- Sites (in descending order)
1. femur FIBROSARCOMA & MALIGNANT FIBROUS
2. skull HISTIOCYTOMA
3. tibia - are fibroblastic collagen producing
4. humerus sarcoma of bone.
5. ribs - have overlapping clinical, radiologic &
6. fibula, histologic features.
7. radius - affects any age group (but are more
8. ulna common in adulthood)
9. mandible - Fibrosarcoma M = F while MFH M > F
10. vertebra
- Gross : large, hemorrhagic, tan-white
mass that destroys the underlying
-Craniofacial involvement: bone with extension to adjacent soft
- 50% of patients with moderate bone tissue.
involvement
- 100% with extensive skeletal disease. Microscopic :
FIBROSARCOMA - in herring-bone
- Shepherds-Crook Deformity patterns
- crippling deformity due to the - bizarre multinucleated giant cells – not
involvement of the proximal femur. common
- most are low to intermediate grade
MFH – composed of spindled fibroblasts
FIBROUS DYSPLASIA ASSOCIATED WITH admixed with large, ovoid, bizarre
CAFÉ-AU-LAIT SKIN PIGMENTATION & MNGC.
ENDOCRINOPATHIES (McCune-Albright - generally high grade pleomorphic
Syndrome) tumors

- 3% of cases Clin : enlarging, painful masses arising in

- F >>> M the metaphysic of long bones and
pelvic flat bones.
- Pathology: excessive production of Cx : pathologic fracture
cAMP Prognosis : grade dependent
 Endocrine gland
hyperfunction. MISCELLANEOUS TUMORS

5
 - osteoclast type giant cell with 100 or
Ewing’s Sarcoma & PNET more nuclei that are
identical to mononuclear cells.
Giant Cell Tumor - secondary fractures
a. necrosis
Metastatic Disease b. hemorrhage
c. reactive new bone formation
EWING’s SARCOMA & PNET
- is a common primary malignant Recurrence Rate: 40-60% after conservative
bone tumor primary small rund cell tumor of surgery.
bone
- these are closely related tumors that
differs in the degree of differentiation.
METASTATIC DISEASE
PNET – have neural differentiation (Homer- - most common form of skeletal
Wright rosettes) malignancy.
- typically multifocal
E.S. - undifferentiated - site : axial skeleton
- youngest average age of distribution - pattern of spread:
(10-20 y.o.) 1. direct extension
- M >> F ; whites >> blacks 2. lymphatics
3. vascular or hematogenous
Micro : invasive tumor composed of small 4. intraspinal seeding
round blue cells that are larger than - (Batsons plexus of
lymphocytes with scant cytoplasm vein)
which may appear clear because it is
rich in glycogen content. Common primary tumor that metastasize to the
bones:
X-ray : lytic skin lesion (Onion-skin lesion)
ADULTS
Treatment : Surgery + chemotherapy with or Prostate
without radiation Breast
Kidneys
- 5year SR : 5,15, 75 % with at least 50% long Lungs
term cure.
CHILDREN
Neuroblastoma
Wilm’s tumor
GIANT CELL TUMOR (Osteoclastoma) Osteosarcoma
- fused multinucleated osteoclast type Ewing’s Sarcoma
giant cells - most are solitary, locally Rhabdomyosarcoma
aggressive,
rarely metastasize
- age : 20-40 y.o.
- origin : epiphysis & metaphysic of long
bone DISEASES OF THE JOINTS
- site : knee ARTHRITIS

- Gross : large, red brown with cystic Osteoarthritis

degeneration
Rheumatoid Arthritis

Microscopic : Juvenile Rheumatoid Arthritis

- uniform, oval mononuclear cells with
indistinct cell
membranes and
appears to grow in syncythium.
- Increased mitotic figures
OSTEOARTHRITIS (degenerative bone dse)
- most common type of joint disease.
- characteristic feature : progressive
erosion of articular cartilage 

6
 polished ivory (bone - skin that are subjected to pressure.
eburnation) – due to friction. - most common skin manifestation
- sites : knee, hands in female patients - show areas of fibrinoid necrosis
& hips in male patients. surrounded by palisade of
macrophage & scattered chronic
- Gross : polished ivory inflammatory cells
joint mice – due to small fractured
bone - Blood Vessels – especially if with increased RF
titer
Clinical : insidious, usually asymptomatic - obstruction, peripheral
- morning stiffness, deep, dull achy pain. neuropathy, ulcer,
- crepitus and limitation of movement. gangrene.

HEBERDEN NODE (characteristically seen - Clinical : - typically involves small joints

women but not in men) followed by large joints
- seen in fingers representing prominent - initial limitation of movement
osteophytes at the distal that in time becomes more severe.
interphalangeal
joints Laboratory- No specific laboratory test is
- spared sites : diagnostic.
- wrist, elbow & shoulder.

RHEUMATOID ARTHRITIS
- is a chronic systemic inflammatory - Diagnostic Criteria (4 of the following)
disorder that may affect 1. Morning stiffness
many tissues & organs (skin, 2. Arthritis in 3 or more joints
blood vessels, heart, lung & muscle) 3. Arthritis of hand joints
but principally attacks the joints, 4. Symmetric Arthritis
producing a non-suppurative 5. Rheumatoid nodule
proliferative synovitis that often 6. Serum RF
progresses to destruction of the 7. Typical Radiologic Changes (joint
articular cartilage & effusion; narrow joint space with
ankylosis of the joints. loss of articular cartilage).

- Pathogenesis: .JUVENILE RHEUMATOID ARTHRITIS

1. Genetic - major cause of functional disability in
2. Primary exogenous arthrogen children, F >> M ; 2 : 1
3. Autoimmune
4. Presence of mediators of - Differs in RA in:
bone damage 1. Oligoarthritis is more common
2. Systemic onset is frequent
- Gross : joints are edematous, thickened and 3. Large joints are affected than
hyperplastic small joints
- Micro : perivascular inflammatory infiltrate, 4. Rheumatoid Nodule & RF are
increased vascularity, absent
organization of fibrin, neutrophil 5. ANA seropositivity is common
accumulation
SERONEGATIVE SPONDYLOARTHOPATHIES
- may produce juxta-articular erosion,
subchondral cyst & osteoporosis 1. Ankylosing Spondylitis - 90% are HLA-
B27 (+)
- PANNUS – is a fibrocellular mass of synovium 2. Reiter Syndrome
& synovial stroma consisting of 3. Enteropathic Arthritis
inflammatory cells, granulation tissue 4. Psoriatic Arthritis
& fibroblast which causes erosion of the
underlying cartilage. INFECTIOUS ARTHRITIS

1. Suppurative – GC, Staph, Strep,

- Skin : Rheumatoid Nodules Gram (-) bacilli.

7
 - Salmonella affects any age. RISK FACTORS FOR GOUTY ARTHRITIS:
- H. influenza – affects children < 2 y.o.
age (old age)
- 90% of non-gonococcal arthritis genetic (HGPRT)
affects a single joint alcohol
(usually the knee joint) obesity (increased risk of asymptomatic
gouty arthritis)
2. TB – is a chronic progressive mono articular drugs (thiazides-predispose to gout)
disease that occurs in any age Lead toxicity
group.
- hematogenous 4 DISTINCT MORPHOLOGIC CHANGES IN
GOUT
3. Lyme Arthritis - caused by Borella bugdorferi 1. Acute Arthritis
- symptom develop 2 weeks post- 2. Chronic Tophaceous Arthritis
exposure 3. Tophi at various sites
- silver stain 4. Gouty Nephropathy

4. Viral – rubella, Hepatitis C virus, Parvovirus 1. Acute Arthritis

B19 - monosodium urate crystal in the
cytoplasm of neutrophil
- negative birefringent (needle-shaped)
GOUT & GOUTY ARTHRITIS
2. Chronic Tophaceous Arthritis
GOUT- is a common endpoint of a group of - due to repetitive precipitation of urate
disorder that produce crystals
hyperurecemia & deposits of monosodium - synovium becomes hyperplastic,
urate crystals in joints. thickened by inflammatory
- acute arthritis initiated by cells & forms pannus that
crystallization of urates within & about destroys the underlying cartilage leading
the joints leading to chronic gouty arthritis to juxta-articular bone erosion.
& deposition of masses of urates in joints &
other sites creating tophi. 3. Tophi at various sites
- pathognomonic hallmark of gout
TOPHI- represent large aggregates of urate - urate crystals are surrounded by
crystals surrounded by inflammatory reaction. macrophages, lymphocytes & FB type
GC.
Urate Nephropathy
4. Gouty Nephropathy
NV of Urate in Plasma : < 7 mg/dl - deposition of monosodium urate
crystals in the renal medullary
Pathology: Uric acid is the end product of purine interstitium.
metabolism
Complications: Pyelonephritis & urinary
Purine synthesis: obstruction.
1. de novo
2. Salvage pathway involves HGPRT CLINICAL COURSE (4 stages)
(decreased HGPRT favors Asymptomatic hyperuricemia
increased Uric Acid production) Acute gouty arthritis
Asymptomatic intracritical gout
LESCH-NYHAN Syndrome (lack or absence of Chronic tophaceous gout
HGPRT)
- Seen only in males: *** Hypertension is common inpatients
1. hyperuricemia with gout.
2. severe neurologic deficit
3. mental retardation
4. Self mutilation

*** gouty arthritis in some cases.

PSEUDOGOUT

8
 (Calcium Pyrophosphate Crystal
Deposition Disease)
- aka : chondrocalcinosis
- > 50 y/o
- crystal first develop in articular matrix,
menisci & intervertebral
disc
 may enlarge and rupture.
Gross : chalky white, friable deposits
Micro : oval blue people aggregates
Weakly birefringent
FIBROUS TUMORS
SUPERFICIAL FIBROMATOSES
characterized by nodules or poorly defined
fascicle of mature appearing
fibroblast surrounded by abundant
dense collagen.
(a) Depuytren’s contracture
(b) Peyronier Disease – dorsolateral
aspect of
penis

Clin : usually asymptomatic DEEP SEATED FIBROMATOSIS (Desmoid

Tumor)
is the interface between exuberant
TUMOR & TUMOR-LIKE LESIONS fibrous proliferation & low grade
fibrosarcoma.
GANGLION & SYNOVIAL CYST Recur after excision
near joint capsule & tendon sheath Do not metastasize
firm, fluctuant, pea sized, translucent.
Cyst wall lacks true cell lining 3 divisions:
“Bakers Cyst” 1. Extra-abdominal – shoulder, chest
wall, back
GIANT CELL TUMOR OF THE TENDON 2. Abdominal – arise from
SHEATH musculoaponeurotic
structure of the anterior abdominal wall
M = F ; 20’s in women during or after
VNS – develop in synovial lining of joints, pregnancy. 3. Inra-
tendon sheath & bursa. abdominal – mesentery, pelvic wall
PVNS – or diffuse giant cell tumor of the - Gardner’s
tendon sheath syndrome
involves 1 or more joints
GCT – localized nodule tenosynovitis Gross : unicentric, firm/rubbery, grey-white,
occur as a discrete nodule on tendon sheath poorly demarcated, 1-15 cm,

SOFT TISSUE TUMOR & TUMOR-LIKE Micro : Central region – oldest part of
LESIONS growth (collagenous stroma)

FATTY TUMORS Peripheral region – made up of

1. LIPOMA - most common soft fibroblast having
tissue tumor of adulthood. minimal
- proximal extremities & variation in cell & nuclear size
trunk - soft, mobile,
painless (except ; MF is infrequent
angiolipoma)
FIBROSARCOMA
- Treatment; Excision rare, but are most commonly seen in the
retroperitoneum, thigh, knee & distal
2. LIPOSARCOMA extremities.
- 40’s – 60’s y.o.
- lipoblast with scalloped nuclei Gross : encapsulated, fish-flesh
- variants:
1. Well differentiated – Micro : highly cellular, architectural disarray,
indolent pleomorphic, frequent MF &
2. Myxoid – necrosis.
intermediate course - “herring-bone pattern”.
3. Pleomorphic –
aggressive FIBROHISTIOCYTIC TUMOR

9
 aka Dermatofibroma, Sclerosing Prognosis (5YSR) : 25-62%, ONLY 11-30% live
hemangioma for 10 years or
Stroriform pattern longer.

MALIGNANT FIBROUS HISTIOCYTOMA PNS

pleomorphic, MNGC, stroriform, inflamed, &
foamy histiocytes. SKELETAL MUSCLE
Grey-white, unencapsulated
Variants IMMUNE MEDIATED NEUROPATHIY
1. storiform Guillain-Barre Syndrome (acute
2. pleomorphic inflammatory demyelinating
3. myxoid polyradiculoneuropathy)
4. inflammatory
INFECTIOUS POLYNEUROPATHY
TUMORS OF THE SKELETAL MUSCLE Leprosy
almost all are malignant Diphtheria (toxin)
VZV – thoracic & trigeminal nerve
1. RHABDOMYOMA
2. RHABDOMYOSARCOMA HEREDITARY MOTOR & SENSORY
most common soft tissue sarcoma of NEUROPATHY 1 (HMSNI 1)
childhood & adolescent. - Charcot Marie-Toot Disease
Head & neck, GUT most common
Histologic Classification childhood & adulthood
Rhabdomyoblast – diagnostic cell in all
types ACQUIRED METABOLIC & TOXIC
- tad pole or strap cells NEUROPATHY
1. Embryonal –most common (sarcoma DM
botryoides) Nutritional (Thiamine)
2. Alveolar – most common in early to Malignancies
mid adolescent Toxic (lead & heavy metal)
- deep muscle of
extremities. MUSCULAR DYSTROPHY
3. Pleomorphic – uncommon - (Duchene Muscular Dystrophy & Becker
MD) -
TUMORS OF SMOOTH MUSCLE
- DMD – most severe & most common form
LEIOMYOMA – skin, nipple, scrotum, labia onset before age 5
walking is delayed
LEIOMYOSARCOMA – 10-20% of all soft weakness begins in pelvic girdle
tissue sarcoma extends to shoulder girdle.
enlargement of calf muscle associated
SYNOVIAL SARCOMA with weakness 
10-20% of all soft tissue sarcoma, 20’s-40’s PSEUDOHYPERTROPHY
deep seated mass
Causes of death: (DMD)
Histologic Hallmark (biphasic) Respiratory Insufficiency
1. Epithelial Pulmonary infection
2. Spindle cells – cuboidal to columnar Cardiac complications
cells densely arranged in
fascicular pattern
BMD – less common and occurs in older age
Metastasis group.
1. Regional lymph node
2. Lung DISEASES OF THE NEUROMUSCULAR
3. Skeleton SYSTEM
Treatment : treated aggressively with limb Myesthenia Gravis
sparing Tx Lambert-Eaton Myesthenic Syndrome.

Myesthenia Gravis

10
 autoimmune disease
loss of acetylcholine receptors
F>M
Thymic hypoplasia

Clinical features:
EXTRAOCCULAR MUSCLE
WEAKNESS;
- ptosis
- double vision

Treatment
1. Anticholinestarase agents
2. Prednisolone
3. Plasmapheresis
4. Thymectomy

Lambert-Eaton Myesthenic Syndrome.

disease of the neuromuscular function
usually develops as a paraneoplastic
process (most common Small cell
carcinoma of the lungs)

Clinical features:
proximal body weakness along with
autonomic dysfunction
anticholinesterase in normal
autoimmunity (?)

11