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MSK > Soft Tissue Pathology > Flashcards

Soft Tissue Pathology Flashcards

1
Q

What is the pathogeneses of soft tissue tumors

A

The majority are sporadic - germline mutations, some environmental
Mostly pluripotent mesenchymal stem cell

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2
Q

Rate and characteristics of simple Karyotype tumors

A

15-20%
Single chromosomal change
common in younger

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3
Q

Rate and characteristics of complex karyotype

A

80-85%
Aneuploid or polyploid with multiple chromosome abnormalities
common in adults

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4
Q

Characteristics of benign tumors

A 
Superficial
Small
Slow growing
Avascular
(may have calcification)
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5
Q

Characteristics of malignant tumors

A 
Deep
Large
Rapidly growing
Hypervascular
(may have calcifications)
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6
Q

Characteristics of Lipomas

A 
Benign
Common
Soft, mobile, painless
Well circumscribed
Proximal extremities and trunk
Mature Adipocytes
Cured with excision
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7
Q

Characteristics of Liposarcomas

A 
Most common sarcoma
Occur in 50-60 yo
Deep tissue of proximal extremities and retroperitoneum 
Slow growing
From lipoblast
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8
Q

What are liposarcoma morphologies

A 
Well differentiated
Myxoid/round cell
Pleomorphic (large, bizarre tumor cells with variable amounts of lipoblasts)
Fibrous bands
Various size
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9
Q

Characteristics of Nodular Fasciitis

A 
Benign, self-limited
Fibroblastic and myofibroblastic
Young adults (10-40)
History of trauma (25%)
grows rapidly over weeks
Superficial
Cured with excision
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10
Q

Characteristics of Fibromatosis

A 
Locally aggressive proliferation
Slow growing
Occur anywhere
Male > Female
Incidence with diabetes, alcoholism, epilepsy
Wnt pathway - Beta Catenin
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11
Q

What are the 3 types of common superficial fibromatosis

A

Palmar - Thickenig in palmar facia (Dupuytrans), more common older
Plantar - Young people, unilateral
Penile - Peyronie disease, induration of mass on dorsolateral aspect, abnormal curvature

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12
Q

Deep fibromatosis

A 
Desmoid tumors
Large, infiltrative mass
Some painful
Facia and musculature
Do not metastisize
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13
Q

Rhabdomyoma

A

Rare, benign tumor of muscle

Cardiac rhabdomyoma with tuberous sclerosis complex

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14
Q

Rhabdomyosarcoma

A

Malignant mesenchymal tumor
Most common soft tissue sarcoma in children
Aggressive neoplasia
Tx: surgery, chemo +/- radiation

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15
Q

Leiomyoma

A 
Benign smooth muscle tumor
Often in uterus (Female> Male)
1-2 cm
Interesting fascicles of uniform spindled cells with cigar shaped nuclei
Markers of smooth muscle
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16
Q

Leiomyosarcoma

A 
Malignant tumors
10-20% of soft tissue sarcomas
Adult onset
Femal > Male
Deep soft tissue extremities and retroperitoneum
Metastasize late in life cycle
Smooth muscle markers: actin and Desmin
17
Q

Synovial Sarcoma

A 
Not from the synovium
Highly malignant
Most common in deep soft tissue near joints
occur in 20-40 yo
Painful mass
Chromosomal translocation of Chromosome X and 18
5 year survival 25-62%
metastasize to lung
18
Q

Myasthenia Gravis demographics

A

150-200 per million

2:1 F>M young; more common in Older Males

19
Q

Myasthenia Gravis presentation

A

Anti-Ach ab
Eye symptoms: Ptosis, diplopia
Association with thymic abnormalities
Muscle weakness: worse with activity, better with rest

20
Q

Lambert-Eaton Myasthenic Syndrome

A

Autoantibodies that block the presynaptic Ca2+ channels
Underlying malignancy such as Neuroendrocrine (small cell) carcinoma
Muscle weakness NOT IN THE EYES
Muscle weakness DOES NOT IMPROVE WITH REST

21
Q

Toxins that bock Ach receptors

A 
Botulinum
Ethanol
Steroids
Chloroquine
Statins
22
Q

Dermatomyositis

A

Proximal muscles first
Systemic autoimmune dz
Associated with carcinoma
Skin involvement: heliotrope rash, malar rash, red papules on elbow knuckles and knees

23
Q

Dermatomyositis Lab findings

A

Increased creatinine kinase
ANA+
Anti-Jo-1 ab
infiltration of mononuclear inflammatory cells (CD4 T ) around perimysium

24
Q

Polymyositis

A

Like dermatomyositis but lacks skin involvement

CD8 T Cells infiltrate endomysium

25
Q

X-Linked (Duchenne) Muscular Dystrophy

A

Progressive degeneration of the muscles (pelvic and shoulder)
1 in 3500 births
Present at 1 yo with muscle weakness
Deletion of the dystrophin (largest human gene)

26
Q

Duchenne Muscular dystrophy pathology

A

Chronic muscle damage that outpaces repair
segmented myofiber degeneration and regeneration with atrophic fibers
serum creatinine kinase is markedly elevated in first decade of life
pseudohypertrophy: muscle replaced with fat and collagen esp. in calf

27
Q

Becker Muscular Dystrophy

A

Mutation in dystrophin

Histologically similar to DMD

MSK (6 decks)

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