Concise Soft Tissue Tumours Flashcards Preview

SMS Week 3 > Concise Soft Tissue Tumours > Flashcards

Flashcards in Concise Soft Tissue Tumours Deck (47):
1

Spinal Muscular Atrophy

autosomal recessive, defect in SMN1 gene. Atrophy of cell leads to denervation

2

Muscular Dystrophy

Duchenne MD = No Dystrophin, 2/3 of cases are familial, symptoms by age 5, duck-like Gait
Hands on knees to stand. Pseudohypertrophy of calves. Wheel chair by age 10. Survive
Until 35.
Becker MD = Abnormal Dystrophin. Later onset with mild sympotms. Muscle necrosis, then replaced by fat.
Female carriers have risk of higher creatine kinase. Survive past 40.

3

Myotonic Dystrophy

Autosomal Dominant, tri-nucleotide repeat. Most common adult MD. Sustained involuntary contraction of muscles, with sagging face.

4

Malignant Hyperpyrexia

muscle spasms, tachycardia, tachypnea, due to anesthetic inhalation and cytosolic
Ca control mutation.

5

Dermatomyositis

Skin and Muscle inflammation. Classic upper eyelid violaceous discoloration and edema.
Proximal muscles affected symmetrically. 25% of patients have malignancy. Perifascicular myocyte atrophy = myocytes dying around blood vessel

6

Polymyositis

Only Muscle effected, mainly in adults. T-Cell mediated injury necrotic tissue throughout
fascicle.

7

Inclusion Body Myositis

Asymmetric, starts distally. Vacuoles in biopsy.

8

Myasthenia Gravis

Autoimmune attack of ACh nicotinic receptor. Associated with Thymic hyperplasia or
thymoma. Treat with anti-cholinesterase.

9

Lambert-Eaton Myasthenia Syndrome

Autoimmune attack of Ca channels. Paraneoplastic syndrome
(probably have cancer).

10

Causes of elevated creatine kinase.

Creatine Kinase = signal for muscle damage
Acute Myocardial Infarct = Check for CK-MB and Troponin I
Skeletal Muscle Dystrophies = Check for total CK
Cerebrovascular accidents

11

Describe in one or two sentences the classification scheme for mesenchymal/soft tissue tumors.

Mesenchymal Proliferation of extra skeletal, non-epithelial tissue.
Classified as what they differentiate into.
Classified as Benign and Malignant (sarcoma). Benign outnumber Malignant 100:1.
Can occur anywhere.
Some Chromosomal abnormalities can be used as markers

12

State the most common type of genetic abnormality in soft tissue tumors.

Neurofibromatosis Type 1
Gardner Syndrome
Osler-Weber-Rendu Syndrome

13

Factors of prognostic importance for soft tissue tumors

Histological Classification = Look at cells and pattern of cells using immune stain
Histological Grade = scored based on differentiation, mitotic count, necrosis, histological grade
Staging of Tumor = TNM scale (Tumor, lymph Node, Metastasis)
Superficial vs. Deep Seated tumor = better prognosis for superficial
Size = Smaller better

14

State which imaging modality is most helpful in the assessment of soft tissue tumors of the extremities.

MRI and Biopsy

15

Lipoma

Most Common soft tissue tumor, Benign

16

Conventional Lipoma

Mature Fat, superficial

17

Angiolipoma

Fat + Blood Vessel, superficial

18

Spindle-Cell Lipoma

Spindle Cells + Fat, superficial

19

Intermuscular Lipoma

Overgrowth of fat in muscles

20

Lipoblastoma

Overgrowth of Fat in infants, due to steroids

21

Liposarcoma

Usually in deep soft tissue or retroperitoneum, Metastatic
Well-Differentiated = rarely metastasize, but likely to recur.
Myxoid/Round Cell = Intermediate Malignancy
Pleomorphic = Aggressive metastatic

22

Reactive Pseudosarcomatous Proliferations

Look like sarcomas, no sharp border, grows quickly

23

Nodular Fasciitis

Painful mass on upper extremeties of young adults after trauma. Has only been
present for a few weeks. Rarely recurs.

24

Myositis Ossificans

Deep lesion of proximal extremities following trauma. Outer margin develops
osteoblasts.

25

Superficial Fibromatosis

Benign, include fascia or aponeurosis.

Palmar Fibromatosis = Duptuytren’s Contracture
Plantar Fibromatosis = on foot
Penile Fibromatosis = on penis

26

Deep-Seated Fibromatosis

Benign, often recur. Usually in teens → 30 yr olds. Often mutated APC gene
Abdominal = Often during/after pregnancy
Extra-Abdominal = Chest, back, thigh
Intra-Abdominal = Pelvic side walls, associated with Familial Adenomatosis Polyposis

27

Fibrosarcoma

Malignant, common in deep soft tissue of extremities.
Fibromatosis Colli = Scar reaction of Sternocleidomastoid in newborns

28

Fibrohistiocytic Tumor

Fibroblast + Tissue Macrophage

29

Dermatofibroma

skin, benign

30

Dermatofibrosarcoma Protuberans

intermediate malignancy, can be confused for dermatofibroma

31

Malignant Fibrous Histiocytoma

High malignancy, high recurrence. Bizarre multinucleated cells.
Has been reclassified

32

Rhabdomyoma

Benign muscle (cardac or skeletal)

33

Rhabdomyosarcoma

Muscle, very aggressive, most common childhood sarcoma
Embryonal = In head or neck of younger than 10 yr old
Alveolar = Middle Adolescence
Pleomorphic = rarest, in deep soft tissue of adults

34

Leiomyoma

Most common in uterus, well circumscribed
smooth muscle

35

Leiomyosarcoma

Common in uterus or GI, can be anywhere smooth muscle.

36

Hemangioma

Benign, commonly in skin, can be anywhere
blood vessels and lymph

37

Pyogenic Granuloma

Benign, in skin
blood vessels and lymph

38

Lymphangioma

Benign, containing lymph, can be associated with Turner’s Syndrome
Glomus Tumor = Painful, arteriovenous structures in distal digits
Vascular Ectasias = Inflammation of preexisting vessels
Nevus Flammeus = Birth Mark
Spider Angiomas = Autosomal Dominant disease
Hereditary Hemorrahagic Telangiectasia = Autosomal Dominant
Bacillary Angiomatosis = Caused by infection from Bartonella bacteria

Angiosarcoma = Malignant, aggressive, often in skin. Can be secondary to radiation therapy.
Hemangioendotheliomas = Very Rare Malignant Vascular tumors.
Kaposi’s Sarcoma = Human Herpes Virus Type 8, purple patches on skin
Chronic
Lymphadenopathic = African Children
Transplant-Associated
HIV-Associated

blood vessels and lymph

39

Norton Neuroma

repeated minor trauma
peripheral nerve

40

Traumatic Neuroma

crushed nerve leading to tangled nerve fibers
peripheral nerve

41

Schwannoma

Benign, Associated with NF2
peripheral nerve sheath tumor

42

Neurofibroma

Benign, Associated with NF1
Cutaneous = solitary in peripheral nerve
Plexiform = Infiltrating peripheral nerve, causes significant deficit
peripheral nerve sheath tumor

43

Malignant Peripheral Nerve Sheath Tumor

Malignant, associated with NF1
peripheral nerve sheath tumor

44

Giant Cell Tumor or Tendon Sheath

Generally fingers and wrist
Diffuse form = Knee, ankle, and foot
synovial lesion

45

Synoval Sarcoma

Malignant, NOT synovial differentiation, occurs around joints, often calcifications present

46

Ganglion Cyst

MYXOMATOUS LESIONS
located in joints (wrist), feels like bubble, not neoplasm

47

Describe, in no more than a few sentences, the key differences between NF1 and NF2.

Neurofibromatosis = Dominant disease

Type 1 = More common, Café au lait spots, Plexiform neurofibromas, neurodevelopmental problems
Type 2 = Bilateral acoustic schwannomas in CN VIII