Mesenchymal tumours Flashcards
what are mesenchymal tumours
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…
what are mesenchumal tunours composed of
different CT of body: fibrous tissue. vartilage, bone, muscle , fatty tissue, vv
mesenchymal are …. and epithelial are
histoid
organoid
whre are lesions of these tissues embryonically derived form
mesoderm beside peripheral nerves, they come form ectoderm
etiology and pathogenesis
- history of previous trauma
- receal chromosomal abnormalities and mutations in genes
- most of soft tissue occur sporadicqlly: (occasionaly or irregualr)
exmaples of compnents of gentic syndromes (sporadically
neirofibromatous 1,
li-fraumeni syndrome
osler-weber-rendu syndrome
mesenchuamøa classification
- fibrous tumor tissue (fibrous reticulate)
- fibrohistiocytic tumors
- tumors of fat cells
- tumors of cartilage, synovium and bone
- vascular tumors
- tumors of muscle cells
- tumors of dorsal chorda
- tumors of schwann cells
- cells of lymphoid and hematopoietic bone marrow origin
interms of tissue it can be divided into
soft tissue
Bone tumors
lacation of mesenchymal tumors
- 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
general feauture sof soft tissue
- superficially located tumors, tend to be bening(but deep-sided lesions are mostly malignant)
- large sized tumors generally more malognnat
- rapidly growing tumors-behave malignant
- maligant tumoras have frequently increased vascularity
- 40%-lower extremitites, 20% upper, turunk and peri-30, ehaf and
but marcinek says 70 IN LOWER
- more men than women
- 15% in choldren- rhabdomyosarcoma and synovial sarcoma
categories of mesenchymal tumors according to dignity
- bening:
- dont recur and removed by excision-lipoma - semimalignant without metasisatis
- semimalignant with low rate of metasasis
- malignant
bening mesenchymal
99%: superficial, above muscle fascia
- palpabel or visble mass
lipoma, fibrohystiocystic, vascular, nerve sheta tumors
malignant mesenchymal tumors=sarcomas, where do they come form
- originate form primitive mesenchyamla cells(capicity to differentiate aling different cell pathways)
- sjeldent kommer fra malignnat transfomation of preexistoing bening tumor
how do sarcomas and carcinomas move
Sarcoma:
- hematogenoys route
- in lungs, liver, bone and brian
carcinomas:
- lymohpgenous
-often late
localisation of sarcomas
75%-lower extremeties
- thihg-because largest muscle
10% chest and retroperitoneum
where ate sacromas usually placed in deep tissue
1/3 -superficial
2/3-deep sited
what are paraneoplatic feauters
-complications caused by some products synthesized by tumor cells
- sacroma domt uaslly make these features
- some features: cachexia, anemia, pale, sleepiness, some bleeding condiitons
what is the most common features of malignancy
-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
most common site of distant metastasis
lunsg
mwhich diagnostic methons should be used
first ultrasound, then MRI or spiral CT
if sarcoma can be problemati to resect, what can we use
prefeered radiotherapy and the chemothrapy(most sarcomas are resistant to chemo)
what cells does tumors arise form
stem cells fordi de er litt immature
fortsatt i fasen hvor de fifferentiate og spnt. tidlig og noe kan skje til dem
diagnosus of soft tissue tumors
- mophology of tumor cells
- if it reseble ant tissue
- should be proved by immunohistolchemistry - 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 - Genetic abnormalities
- 1/3 of sarcomas
typing of sarcomas
determiantion of tumor type
grading of sarcomas
-aggressivity
- grade of malignant
- 2grade, 3 grade
assesment:
1. differentiation
- resembles of normal
- good=low grade tumor and ooppsye
- mitotic actvity
-nr of mitosses
- ^mitoses=more aggressive - necrosis
staging of sacromas
TNM:
T:
pT1: <5cm (superficial-pT1A, deep-pt1B
pT2: >4cm, (superfical samme opplegg)
N:
N0: absent
N1: present
M:
M0: absent
M1: distal
what is firbroma
- tumor of fibrous tissue
- ex: hyperplastic fibrous tissye
- gibrous growth with mesenchymal tissue elements-neurofibroma, fibromyoma
Fibrous growth with mesenchymal tissue elements: ex
- fibroma durum
- fibroma mole or fibrolipoma
- elastofibroma
fibroma durum
-bening, pedunculated and weel circumsied
- skin
-body surfaces and mucosal membranes
- rich collagenous fibrous connective tissue
fibroma mole or fibrolipoma
- soft fibroma
- bening, mixed with mature fibrous CT and adult type fat
elastrofibroma
-rare bening
- subscapular region
- collagen bundles and branching elastic fibers
what is fibromatosis
tumor like lesions of fibrous tissue which continue to proliferate activly and can be difficulat to differentiate from sarcomas
- semimalignant
how do fibromatosis look
- invade locally and recur after surgical excision
- like myofibroblasts
what do we divide fibromatosis into
- infatile and juvenule
- adult
adult type of fibromatosis
- 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%
desmoid fibromatosis
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
- extra-abdominal: men, upper and lower extr, chest wall, back, buttocks, head and neck
- intarabdomainal: root of small bowel mesentery sassocated with Gardner syndrome,
solitary, large, firm , infiltating muscle
fibromatoses
infiltartion
non metastasing
active proliferation
recur after removal
painful and deformaing
fibrosarcoma
- 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
Fibrohistocystic tumors
- presence of cells wirh fibroblastic and histiocytisc feature in varying proportions
- bening, low grade malingnad or malignnat
bening fibrohistiocytsi tumors
dermatofibroma, slceronsing ehrmngionma, fibroxanthoma, giant cell tumor of tendon sheath
low grade malingna tof fibrocystic tumors
dermatofibrosarcoma protuberanse
-trunk
malingnat firbohystiocytsic tumors
- 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
tumors of adipose tissue
lipomas-bening
liposarcoma-malignant
lipomas-AT
- 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
liposarcoma-AT
- 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
skeletal mucle tumors
rhabdomyoma
rhadomyosarcoma
rhabdomyoma
- bening,r are
ehad and neck, upper neck, tomng, larynd and pahrunc
-
rhabdomyosarcoma
-malignant
-most common soft tissue tumor in children and young adults
- arising form rhabdomyoblsts
-types: embryonal, botryoid, alveolar, pleomorphic
tumros of uncertain histogeneiss
- 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 - alveolar soft part sarcoma
- granular cell myobalstoma
- epitheloid sarcoma
- clear cell sarcoma
tumor like lesions
- nodular fascitis
- 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
