Myopathies and Soft Tissue

Question 1

Provide tumor name for the fallowing..
- smooth muslce
- skeletal muslce
- lipocytes
- firbroblasts
-vascular
-

Answer 1

smooth muslce - leiomyoma
skeletal muslce - Rhabdomyoma
lipocytes - lipoma
firbroblasts - fibroma
vascular - hemangioma

Question 2

What are the non-neoplastic conditions?

Answer 2

nodular fascitis, myositis ossifacans, and fibromatosis

Question 3

benign lipoma

Answer 3

• Adult
• commonly encapsulated
• subcutaneous tissue (proximal extrem and trunk)
• treated by Excision

Question 4

malignant liposarcoma

Answer 4

• nonencapsulated

- deep/large in proximal extrem and retroperitoneum

Question 5

malignant fibro sarcoma

Answer 5

palisadig appearance

Question 6

fibrohistoiocytoma is found where?

Answer 6

dermis and subcutaneous in adults

Question 7

Where is leiomyoma usually found?

Answer 7

uterus

Question 8

what age for rhabdomyoma?

Answer 8

before 20

Question 9

malignant rhabdomyosarcoma is usually found where?

Answer 9

head and neck

Question 10

What are the fibrous tumor-like conditions?

Answer 10

• nodular fasciitis
• myositis ossificans
• fibromatosis

Question 11

reactive pseudosarcoma

Answer 11

composed of fibroblasts and mimic sarcomas histologically

Question 12

nodular fascitis

Answer 12

• most common
• plump randomly oriented spindle cells with myxoid stroma
• reatment by excision

Question 13

myositis ossificans (stiff man syndrome)

Answer 13

• CT and m. is replaced by bone
• seen in athletes, adolescents, and young adults
• in dermis, LE muscles

Question 14

What is the name for myositis ossificans found in the adductors?

Answer 14

• Prussian’s disease

Question 15

What are the common areas for fribromatosis?

Answer 15

• Palmar and plantar fascia

- more common in males

Question 16

What are the two most common types of muscular dystrophies?

Answer 16

• Duchenne

- Becker

Question 17

Duchenne type MD

Answer 17

• x-linked and mostly in males
• absence of Dystrophin
• pelvic girdle mm. –> shoulder girdle mm.
• compensatory hypertrophy

Question 18

What is high in Duchenne type MD?

Answer 18

CPK

Question 19

Beckers

Answer 19

• less common and less severe than Duchenne

- no mental retardation

Question 20

Fascioscapulohumeral MD

Answer 20

• AUTOSOMAL DOMINANT
• 10-30 years
• heart is not involved and CPK is normal

Question 21

Ocular-pharyngeal muscular dystrophy

Answer 21

• autosomal dominant
• extraocular and facial m. weakness
• ptosis and dysphagia

Question 22

limb-girdle MD

Answer 22

type 1 - autosomal dominant
type 2 - autosomal recessive
- slow progression
- CPK is less elevated

Question 23

myotonic dystrophy

Answer 23

• autosomal dominant
• myotonia - sustained involuntary contraction
• 2-4th decade

Question 24

what are some of the clinical findings in myotonic dystrophy?

Answer 24

mental retardation, apathy, hypersomnia, and mild sensory neuropathy

Question 25

congenital myopathies

Answer 25

• autosomal recessive

- hypotonia

Question 26

lipid myopathies

Answer 26

• lipid vacuoles in muslces

- progressive muslce weakness

Question 27

mitochondrial myopathies

Answer 27

• chronic progressive ophthalmoplegia

- kearns-Sayre syndrome

Question 28

hypothyroidism

Answer 28

• glycogen and mucopolysaccharide accumulation
• atrophy, cramping, and muslce aching
• slow movements and reflexes

Question 29

drug induced myopathy

Answer 29

• steroids

- statins

Question 30

What is Myasthenia Gravis?

Answer 30

• autoimmune disorder: type ll hypersensitivity that is antibody mediated
• 3 : 100, 000

Question 31

myasthenia gravis initially affects?

Answer 31

extraocular muslces with ptosis and diplopia

- eventually respiratory failure

Question 32

Lambert-Eaton Myasthenic syndrome

Answer 32

• proximal muscle weakness

- anti-AchR antibodies