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PATHOANA > Mesenchymal tumours > Flashcards

Mesenchymal tumours Flashcards

1
Q

what are mesenchymal tumours

A

tumors of soft tissue and bone or

non -epi extra skeeltal tissues of the body, except the reticuloendothelial system, the glia and supproting tissues of specific organs and viscera

reticuloendo sys: cells able to remove and phsogocytose: kuppfer cells, macrophages…

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2
Q

what are mesenchumal tunours composed of

A

different CT of body: fibrous tissue. vartilage, bone, muscle , fatty tissue, vv

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3
Q

mesenchymal are …. and epithelial are

A

histoid

organoid

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4
Q

whre are lesions of these tissues embryonically derived form

A

mesoderm beside peripheral nerves, they come form ectoderm

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5
Q

etiology and pathogenesis

A
  1. history of previous trauma
  2. receal chromosomal abnormalities and mutations in genes
  3. most of soft tissue occur sporadicqlly: (occasionaly or irregualr)
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6
Q

exmaples of compnents of gentic syndromes (sporadically

A

neirofibromatous 1,
li-fraumeni syndrome
osler-weber-rendu syndrome

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7
Q

mesenchuamøa classification

A
  1. fibrous tumor tissue (fibrous reticulate)
  2. fibrohistiocytic tumors
  3. tumors of fat cells
  4. tumors of cartilage, synovium and bone
  5. vascular tumors
  6. tumors of muscle cells
  7. tumors of dorsal chorda
  8. tumors of schwann cells
  9. cells of lymphoid and hematopoietic bone marrow origin
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8
Q

interms of tissue it can be divided into

A

soft tissue
Bone tumors

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9
Q

lacation of mesenchymal tumors

A
  • lovated on extremetis becaoum those have whole mesenchymal origin, with minimal epi component like skin.
    everything inside is mesenchumal origin
  • in abdominal cavity-: retroperitoneum, mesentery, mesocoln
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10
Q

general feauture sof soft tissue

A
  1. superficially located tumors, tend to be bening(but deep-sided lesions are mostly malignant)
  2. large sized tumors generally more malognnat
  3. rapidly growing tumors-behave malignant
  4. maligant tumoras have frequently increased vascularity
  5. 40%-lower extremitites, 20% upper, turunk and peri-30, ehaf and

but marcinek says 70 IN LOWER

  1. more men than women
  2. 15% in choldren- rhabdomyosarcoma and synovial sarcoma
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11
Q

categories of mesenchymal tumors according to dignity

A
  1. bening:
    - dont recur and removed by excision-lipoma
  2. semimalignant without metasisatis
  3. semimalignant with low rate of metasasis
  4. malignant
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12
Q

bening mesenchymal

A

99%: superficial, above muscle fascia
- palpabel or visble mass

lipoma, fibrohystiocystic, vascular, nerve sheta tumors

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13
Q

malignant mesenchymal tumors=sarcomas, where do they come form

A
  • originate form primitive mesenchyamla cells(capicity to differentiate aling different cell pathways)
  • sjeldent kommer fra malignnat transfomation of preexistoing bening tumor
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14
Q

how do sarcomas and carcinomas move

A

Sarcoma:
- hematogenoys route
- in lungs, liver, bone and brian

carcinomas:
- lymohpgenous
-often late

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15
Q

localisation of sarcomas

A

75%-lower extremeties
- thihg-because largest muscle

10% chest and retroperitoneum

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16
Q

where ate sacromas usually placed in deep tissue

A

1/3 -superficial
2/3-deep sited

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17
Q

what are paraneoplatic feauters

A

-complications caused by some products synthesized by tumor cells
- sacroma domt uaslly make these features

  • some features: cachexia, anemia, pale, sleepiness, some bleeding condiitons
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18
Q

what is the most common features of malignancy

A

-weight loss–>cachexia:reduction of all organs(atrophy) becuase of lack of energy and or nutrition.
- comes from the products synthesized bu the cells in tumors, ike cytokines which influence metabolism

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19
Q

most common site of distant metastasis

A

lunsg

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20
Q

mwhich diagnostic methons should be used

A

first ultrasound, then MRI or spiral CT

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21
Q

if sarcoma can be problemati to resect, what can we use

A

prefeered radiotherapy and the chemothrapy(most sarcomas are resistant to chemo)

22
Q

what cells does tumors arise form

A

stem cells fordi de er litt immature
fortsatt i fasen hvor de fifferentiate og spnt. tidlig og noe kan skje til dem

23
Q

diagnosus of soft tissue tumors

A
  1. mophology of tumor cells
    - if it reseble ant tissue
    - should be proved by immunohistolchemistry
  2. immunohistochemistry
    - labeling of molecules by visbile markes: antibody against antigen by some color
    - for soft tissue tumors -VIMETIN
    - for epi=Cytokeratin or epit memrbane antigens (EMA)
    - muscle differentitation: DESMIN
    - smmot m: SMOOTH MUSCLE ACTIN+H-CALDESMON-LEIOMYOSACROMA
    -striated m: MYOGENEIN+MYOD 1-rhabdomyosarcoma
    - vascualr diff: CD34, CD31, FL1
    - neurla fiff: S100–>then NEURAL SEPCIFIC ENCOLASE (NSE), melanocytic: HMP 45, MELAN A, hystocytic: CD68
  3. Genetic abnormalities
    - 1/3 of sarcomas
24
Q

typing of sarcomas

A

determiantion of tumor type

25
Q

grading of sarcomas

A

-aggressivity
- grade of malignant
- 2grade, 3 grade

assesment:
1. differentiation
- resembles of normal
- good=low grade tumor and ooppsye

  1. mitotic actvity
    -nr of mitosses
    - ^mitoses=more aggressive
  2. necrosis
26
Q

staging of sacromas

A

TNM:
T:
pT1: <5cm (superficial-pT1A, deep-pt1B
pT2: >4cm, (superfical samme opplegg)

N:
N0: absent
N1: present

M:

M0: absent
M1: distal

27
Q

what is firbroma

A
  • tumor of fibrous tissue
  • ex: hyperplastic fibrous tissye
  • gibrous growth with mesenchymal tissue elements-neurofibroma, fibromyoma
28
Q

Fibrous growth with mesenchymal tissue elements: ex

A
  1. fibroma durum
  2. fibroma mole or fibrolipoma
  3. elastofibroma
29
Q

fibroma durum

A

-bening, pedunculated and weel circumsied
- skin
-body surfaces and mucosal membranes
- rich collagenous fibrous connective tissue

30
Q

fibroma mole or fibrolipoma

A
  • soft fibroma
  • bening, mixed with mature fibrous CT and adult type fat
31
Q

elastrofibroma

A

-rare bening
- subscapular region
- collagen bundles and branching elastic fibers

32
Q

what is fibromatosis

A

tumor like lesions of fibrous tissue which continue to proliferate activly and can be difficulat to differentiate from sarcomas

  • semimalignant
33
Q

how do fibromatosis look

A
  • invade locally and recur after surgical excision
  • like myofibroblasts
34
Q

what do we divide fibromatosis into

A
  1. infatile and juvenule
  2. adult
35
Q

adult type of fibromatosis

A
  • palmar and plantar, nodular fasciits, kleoid

-keloid: progressive fibrous growth bc cutaneous injury like burns, inscision, vaccination
- in blacks
- smooth, firm, elevated, claw like projections, colagen+fibroblasts

nodular fadcitis:
- bening and reactive fibroblastic growth form superficial fascia into subcutanous fat and sometime nearby muscles
- upper ext, trunk, neck, youg adults, local excision is curative, little recurrens, as noduel, often after traum

palmar anf plantar
- most common superfically
- Dupuytren liek contracture
PALMAR:
- old man–>flexion of fingers
- painless, nodular or irregualr, infilatarting, bening
- bilateral
- fibrovascualr tissue with dividng fibrolblast like myofibroblasts
PLANTAR
- medial aspect of plantar arch
- no contractures
-adult
- similar to penile fibromatosis or peyronie disase(lesions in shaft of penis) or soft tissue of knuckles
- recurrence:50-60%

36
Q

desmoid fibromatosis

A

aggresive behavior recurr often and multiple
- role of antecedent trauma, genetic and estorgen lvls

types:
1. abdominal: locally aggressive, in musculoaponeurotic structures of rectus muscle in ant abd wall in women during/after pregnancy

  1. extra-abdominal: men, upper and lower extr, chest wall, back, buttocks, head and neck
  2. intarabdomainal: root of small bowel mesentery sassocated with Gardner syndrome,

solitary, large, firm , infiltating muscle

37
Q

fibromatoses

A

infiltartion
non metastasing
active proliferation
recur after removal
painful and deformaing

38
Q

fibrosarcoma

A
  • from fibroblast
  • 25% come again-lungs
    slow groth, deep seated, young-mid age adult, retroperitoneum, thich knee
  • uncapsulated, infiltarteive
  • firm, necrosis, hemoragees, uniform spindle shped fibroblasts
  • oncogene ETV6 egene
    . via blood
    -45% 5 yr suvival
39
Q

Fibrohistocystic tumors

A
  • presence of cells wirh fibroblastic and histiocytisc feature in varying proportions
  • bening, low grade malingnad or malignnat
40
Q

bening fibrohistiocytsi tumors

A

dermatofibroma, slceronsing ehrmngionma, fibroxanthoma, giant cell tumor of tendon sheath

41
Q

low grade malingna tof fibrocystic tumors

A

dermatofibrosarcoma protuberanse
-trunk

42
Q

malingnat firbohystiocytsic tumors

A
  • malignant fibrous histiocytoma
  • 20-30% of all soft tissue
  • msot common and most freqeunt sarcoma associated with radiotherapy
    -males , 50-70y
    -lowe and upper ext, retroperitoenum
  • meatsaiss–>lungs and region LN
43
Q

tumors of adipose tissue

A

lipomas-bening
liposarcoma-malignant

44
Q

lipomas-AT

A
  • bening
  • most common soft tissue tumor!!
  • females, 40-50
  • subcutaneous tissue in neck, back and shoulder
    -smal oval, encapsulated
  • can be seen with fibrolipoma, angiolipoma, myelipoma
45
Q

liposarcoma-AT

A
  • 2nd most common
  • form primitive mesenchymal cells, the lipoblasts
  • dep tissues: intermuscular region of thigh, buttocks, retroperitonemum
    -hallmark: variable number of lipoblasts, either univacuolated or multivacoulated
  • types: well diff, myxoid, round cell and pleomorphic
46
Q

skeletal mucle tumors

A

rhabdomyoma
rhadomyosarcoma

47
Q

rhabdomyoma

A
  • bening,r are
    ehad and neck, upper neck, tomng, larynd and pahrunc
    -
48
Q

rhabdomyosarcoma

A

-malignant
-most common soft tissue tumor in children and young adults
- arising form rhabdomyoblsts
-types: embryonal, botryoid, alveolar, pleomorphic

49
Q

tumros of uncertain histogeneiss

A
  1. synovial sarcoma(malignant synovioma)¨
    - pigmented villonodular and giant cell tumors of tnedon sheats, bortg of which are tumor like lesions of synovial tissue
    - form multipotent stem cells
    - multilobular and encapsulated, foci of calcificaiton and cyst spaces, area of hemorrhages and necrosis
  2. alveolar soft part sarcoma
  3. granular cell myobalstoma
  4. epitheloid sarcoma
  5. clear cell sarcoma
50
Q

tumor like lesions

A
  1. nodular fascitis
  2. myositis ossificans
    - bening, osteoid and heterotopic bone formation in soft tissye
    - trauma of skeletal m or its tnedon
    -richly vascularized granulation tissue replaves the affected muscle or tendom, then foolow developemt of oestoid and bone at periphert

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