Andrew's Chapter 28 Fibrous Tissue Abnormalities Flashcards

Collagen
Terms Definitions
keloid histo
myofibroblasts and college whorled and no elastic tissue(similar to hypertrophic scar)
CENTRAL THICK HYALINIZED COLLAGEN BUNDLES
mucopolysaccarides increased
mast cells
Keloid vs hypertrophic scar
Keloid: central hyalizined collegen bundles
keloid: claw-like cheloids
keloid: extention beyond initial injury
keloid claw like projections
cheloid
atypical keloid appearing lesion
carcinoma en cuirasse
need bx
keloid growth factor?
Transforming Growth Factor B (TGF-B)
Triamcinolone decreases prolif and collagen production assoc w decrease TGF-B1 in normal and keloid fibroblasts
Verapamil keloids
decrease IL-6 and VEGF in dx cells to inhibit cell growth
keloids s/p excision
triamcinolone
IFN-alpha 2b
CO2 laser
Dupuytren Contracture
fibromatosis of palar aponeurosis
30 to 50 yr old men
myofibrolats androgen receptors
5alpha dihydrotestosterone increases fibroplast prolif
no B-catenin and APC gene mutations (less aggressive than deep fibromatosis)
Dupuytren Contracture associated illnesses
ETOH Cirrhosis
Plantar Fibromatosis
DM
Chronic eplilepsy
Peyronie's Disease
Knuckle pads
Dupuytren Contracture vs deep Fibromatosis
Dupuytren NO B-CATENIN and NO APC ADENOMATOUS POLYPOSIS COLI GENE MUTATIONS
less aggressive
Ledderhose's Disease
Plantar Fibromatosis
plantar Dupuytren's
clinical, bx, MRI
tx: ILK, excision, radiotx
Heloderma
Knuckle Pads
Heloderma, hearing loss (sensorineural and conductive), and leukonychia
auto-dom disorder
Heloderma assoc w?
auto-dom epidermolytic palmoplantar keratoderma (mutation keratin 9)
camptodactylia
irreducible flexion contracture one or more fingers
assoc with heloderma
Heloderma histo
fibromas
free moving
Patchydermodactyly
benign fibromatosis of PIPs
teens
mult fingers
5 types
1. classic
2. localized
3. transgrediens (extends to mc)
4. assoc with tuberous sclerosis

collegen and mucin = swelling
no tx
peyronie's disease
penis fibrous intercavernous septum
nodules or plaques, fibrous chordee
assoc w dupuytren's contracture
injection of IFN
extracorporeal shock tx
desmoid tumor
muscular aponeurosis
women preg
5 types
1. abdominal
2. extra-abdominal
3. intra-abdominal
4. multiple
5. Garder syndrome/familial adenomatous polyposis

tx: wide local excision, radiotx, high dose tamoxifen w sulindac
mesenteric desmoid tumors tx
antiangiogenic tx w toremifene and IFN alpha 2b
collagen fibroma
desmoplastic fibroblastoma
desmoplastic fibroblastoma
collagenous fibroma
deep, slow, benign, deep sc, fascia, aponeurosis, muscle
HYPOCELLULAR, BANDS OF COLLAGEN skeletal muscle, no metastasize
CHROMO TRANSLOCATION (2;11), (q31;q12)
Desmoid Tumors 5 types
1. abdominal
2. extraabdominal
3. intraabdominal
4. multiple
5. assoc with Gardner's/familial adneomatosis polyposis
Collagenous Fibroma genetic?
chromo translocation
(2;11), (q31;q12)
aponeurotic fibroma
juvenile aponeurotic fibroma (calcifying fibroma)
slow-growing cyst well-demarcated structures on hands/feet
collagenous stroma w acid mucopolysaccharides infiltrated w plump meenchymal cells with oval nuclei
hyalinized areas (chondroid or osteoidmetaplasia)
STIPPLED CALCIFICATION on ROENTGENOGRAMS
tx surgery
Infantile myofibramtosis
mc fibrous tumor infancy
50% head and neck
60% at birth or soon after
congenital generalized fibromatosis
uncommon
multiple firm dermal and sc nodules
50% skeletal lesions metaphyseal long bones
if only bone and skin then good prog resovle 1 to 2 years old (congential multiple fibromatosis)
females
Widespread infantile myofibromatosis
auto dom
organs, gi, breast, lun, liver pan, tongue, serosal ,lns. kidney
spindle cells whorled pattern, nodules of myofibrolasts
mortality 80%
if survive 4 mths spon regression
low dose chemotx
juvenile hyaline fibromatosis
infantile systemic hyalineosis
allelic auto recessive
mult sc skin nodules, haline deposition, gingival hypertrophy, osteolyctic bone, joint contractures

nodular tumors scalp, face, stremtities
pink confluent papules paranasal folds and periauricular and perianal

chromo q4q21 15 different mutations in gene encoding capillary morphogenesis protein 2 transmemb prtein induced during cap morphogenesis binds laminin and ollagen 4
juvenile hyaline fibromatosis
infantile systemic hylanosis
gene
chromo 4q21 15 different mutations encoding capillary morphogenesis protein 2
transmemb protein induced durin gcapillary morphogenesis binds LAMININ and COLLAGEN 4

fibroblasts w intracytoplasmic eosinophilic granules in homeogeneous eosinophilc dermal ground substance
defective synthesis of collagen deposited fibrogranular material
Infantile digital fibromatosis
infantile myofibroblastoma, inclusion body fibroma

rare neoplasm of infancy/childhood
dorsal or lateral DIP toes fingers
NO THUMB OR GREAT TOE
asym firm red smooth nodules 1st mth/yr
no metastasize but infiltrate
dermis prolif myofibroblasts and collagen
eosinophilic cytoplasmic inclusions fibroblasts

conservative nonsurgical
spon regression
mohs
inclusion bodies tumor cellss tain with trichrorme and smooth muscle actin stain
fibrous hamartoma of infancy
solitary dermal or sc firm nodule upper trunk, axillae, upper arm, upper grunk, inguinal region, genitals
uncommon skin changes (hair, eccrine, pig)
boys
whrols or bands
fibramatosis of colli
fibrous prolif infiltrating lower 1/3 SCM, fine needle aspiration confirm dx
spon regression, wryneck deformity
wryneck deformity
fibromatosis of colli
Diffuse Infantile Fibromatosis
within 3 yrs
muscles of arms, neck, and shoulder
/ 35
Term:
Definition:
Definition:

Leave a Comment ({[ getComments().length ]})

Comments ({[ getComments().length ]})

{[comment.username]}

{[ comment.comment ]}

View All {[ getComments().length ]} Comments
Ask a homework question - tutors are online