Surgery FINAL
Surgery FINAL
● Young female: overlies the 2nd to the 6th ribs and their
costal cartilages and extends from the lateral margin of
TOPIC OUTLINE
the sternum to the midaxillary line.
I. Breast = Mammary Gland
● UPPER LATERAL EDGE: Extends around the lower border
II. Congenital Anomalies of the Breast
of the pectoralis major and enters the axilla.
III. Infectious and Inflammatory Disorders of The
● MIDDLE-AGED MULTIPAROUS: Maybe large and
Breast
pendulous
IV. Common Benign Disorders of the Breast
● MENOPAUSAL: Adipose tissue reduced, and
V. Breast Cancer
hemispherical shape is lost
● The mature female breast extends from the level of the
second or third rib to the inframammary fold at the sixth
BREAST = MAMMARY GLAND or seventh rib.
● The breasts are specialized
accessory glands of the skin that DESCRIPTION
secrete milk. ●
Child/Men: rudimentary
●
Female: after puberty, enlarges → hemispherical shape
●
The breast has a protuberant conical form.
LOCATION ●
The base of the cone is roughly circular, measuring 10-12
● Lies in the superficial fascia covering the anterior chest cm in diameter.
wall ● Considerable variations in the size, contour, and density
of the breast are evident among individuals.
● Rests on the fascia of the
pectoralis major, serratus
anterior, and external oblique
abdominal muscles, and the
upper extent of the rectus
sheath
● Cushioned in fat. ANATOMY
DEVELOPMENT OF BREAST
NERVE SUPPLY
● Lateral cutaneous branches of the 3rd-6th intercostal
nerves provide sensory innervation of the breast (lateral
mammary branches) and of the anterolateral chest wall.
These branches exit the intercostal spaces betweenslips ● Birth: major ducts; only one nipple left behind, the rest
of the serratus anterior muscle. should have not developed
● Cutaneous branches that arise from the cervical plexus, ● Puberty: enlarges due to hormones → proliferation of
specifically the anterior branches of the supraclavicular epithelial and connective tissue elements
nerve, supply a limited area of the skin over the upper ● Pregnancy: becomes fully developed
portion of the breast. ○ Rapid increase in length and branching of the duct
● The intercostobrachial nerve is the lateral cutaneous system.
branch of the 2nd intercostal nerve and may be ○ Secretory alveoli develop at ends of smaller ducts.
visualized during surgical dissection of the axillary. ○ Vascularity increases
Resection of the intercostobrachial nerve causes loss of ○ Nipple enlarges and areola darkens.
sensation over the medial aspect of the upper arm. ○ Later: Colostrum
○ Absence of the breast (amastia) is rare and results
Macromastia ● Diffuse
hypertrophy of
one or both
breasts
occasionally
occur at
puberty in
otherwise
normal girls
GYNECOMASTIA
MYCOTIC INFECTION
● Blastomycosis or sporotrichosis
● Candida albicans – scaling
● Intraoral fungi that are inoculated into the breast tissue
by the suckling infant initiate these infections, which
present as mammary abscesses in close proximity to the
MANAGEMENT nipple-areola complex.
● Androgen deficiency, then testosterone administration ● Dx: scrapings – fungal elements like filaments and
may cause regression. binding cells
● Due medications, then these are discontinued if ● Occurs in immunocompromised women.
possible. ● Management
● Endocrine defects are responsible, then these receive ○ o Removal of predisposing factor
specific therapy (anti-estrogen) ○ o Topical or systematic
● Progressive and does not respond to other treatments,
surgical therapy is considered (local excision, HIDRADENITIS SUPPURATIVA
liposuction, or subcutaneous mastectomy (most ● Usually involves the nipple-areola complex or axilla.
commonly done) ● Chronic condition
● Attempts to reverse gynecomastia with danazol have ● Management: Antibiotic, I&D (incision and drainage),
been successful, but the androgenic side effects of the excision with grafts
drug are considerable (weigh the benefits and risks) ● Chronic inflammatory condition that originates within
the accessory areolar glands of Montgomery or within
INFECTIOUS INFLAMMATORY DISORDERS OF THE BREAST the axillary sebaceous glands
● Common in postpartum period (due to the milk, which
is a good medium for bacterial growth)
● Intrinsic: secondary to abnormalities within the breast
● Extrinsic: secondary to an infection in an adjacent
structure (periductal mastitis or infected sebaceous
cyst)
BACTERIAL INFECTION
● Staphylococcus aureus and Streptococcus species (most
common species)
● Typically breast abscesses are seen in staphylococcal COMMON BENIGN DISORDERS OF THE BREAST
infections and present with point tenderness, ABERRATIONS IN THE NORMAL DEVELOPMENT AND
erythema, and hyperthermia INVOLUTION (ANDI)
● Breast abscess (erythema, tenderness, hyperthermia) ● Basic Principle:
INVOLUTION
● Dependent on specialized stroma
● MACROCYST - stroma involutes quickly, alveoli remain
and from microcyst (precursor to microcyst) - DOES NOT
REQUIRE TREATMENT
● SCLEROSING ADENOSIS - disorder of proliferative and
involutional phases
● DUCT ECTASIA – dilated subareolar ducts with thick
nipple discharge → stagnation of secretions, epithelial
*See last page for bigger and clearer photo. ulceration and leakage of duct secretion into periductal
tissue
● PERIDUCTAL MASTITIS → Periductal fibrosis
Fibrocystic Disease
● Non-specific; fibrocystic change, cystic mastopathy,
chronic cystic disease, fibroadenomatosis, etc.
● Refers to a spectrum of histopathologic changes.
● Diagnostic term to describe symptoms, to rationalize
the need for breast biopsy and to explain biopsy results.
Galactocele
● In lactating women
- A milk-filled cyst that is round, well circumscribed,
and easily movable within the breast.
- Generally, occurs after the cessation of lactation or
*See last page for bigger and clearer photo. when feeding frequency has declined significantly,
although galactoceles may occur 6-10 months after
FIBROADENOMA breastfeeding has ceased.
- Pathogenesis of galactocele is unknown, but
inspissated milk within ducts is thought to be
responsible.
- The cyst is usually located in the central portion of
the breast or under the nipple.
- Needle aspiration produces thick, creamy material
that may be tinged dark green or brown. Although it
appears purulent, the fluid is sterile.
- Treatment is needle aspiration; withdrawal of the
Intraductal Papilloma
● Arise in major ducts.
● Most commonly associated with NIPPLE DISCHARGE
● Premenopausal women
● <0.5cm – 5cm Management of Benign Breast Disorders/Diseases
● Complaint: NIPPLE DISCHARGE – serous or bloody (not Cyst
automatically cancer) ● Breast imaging – UTZ: differentiate tumor if solid or cystic.
○ What we usually do is express the breast and check
● Needle biopsy – 5-10 ml non-bloody aspirate (or more)
in which quadrant is the intraductal papilloma
diagnostic & therapeutic (exclude residual mass)
because sometimes we cannot feel any mass
● SIMPLE CYST vs COMPLEX CYST (residual mass) - biopsy.
○ Diagnosis: press the breast quadrant per quadrant,
then identify which of the quadrants produces
Fibroadenoma
bloody nipple discharge
● Self-limiting
● Rarely undergoes malignant transformation (unless ● UTZ + core needle biopsy (risk is low, not needed)
accompanied by atypia) ● MORE THAN 2 CM: surgical removal, cryoablation,
● Multiple papillomas in younger women less frequently vacuum assisted biopsy or OBSERVATION
associated with nipple discharge –susceptible to
malignant transformation.
● Management: Quadrantectomy
Cysts
● Round, movable lump, which might also be tender to the
touch, suggests a cyst.
● Fluid-filled, round or oval sacs within the breast.
● Most often found in women in their 40’s, they can occur
in women of any age.
Trans 1 | ESCORPIZO | FIALIWAN | GALLANONGO | GUILLEM | KINDICA | LAMORIN Page 10 | 30
● Monthly hormone changes often cause cysts to get bigger increased risk of breast cancer- about 1 ½ times the risk of
and become painful and sometimes more noticeable just women with no breast changes.
before the menstrual period. ● Complex fibroadenomas seem to increase the risk slightly
more than simple fibroadenomas.
Diagnosis
● Diagnosed based on symptoms, such as breast lumps, Treatment
swelling, and/or tenderness or pain. ● Many doctors recommend removing fibroadenomas
● Tend to be worse just before your menstrual period especially if they keep growing or change the shape of the
begins and may change as you move through different breast, to make sure that the cancer is not causing the
stages of your menstrual cycle. changes.
● An ultrasound may be done to see if the lumps are solid ● Sometimes these tumors stop growing or even shrink on
or are just filled with fluid (called a simple cyst.) If the their own, without any treatment. It’s important for women
ultrasound shows the lump is solid or if the cyst has both who have fibroadenomas to have regular breast exams or
fluid and solid components (a complex cyst), a biopsy may imaging tests to make sure that the fibroadenomas are not
be needed to make sure that it’s not a cancer. growing.There is no definite medicine yet for
● Neither fibrosis nor simple cysts increase your risk of later fibroadenomas
developing breast cancer. ● Sometimes one or more new fibroadenomas can grow
after one is removed. This means that another
Treatment fibroadenomas has formed- it does not mean that the old
● Cyst fluid doesn’t need to be removed unless it’s causing one has come back.
discomfort.
● Mild discomfort from fibrosis, you may get relief from
well- fitted, supportive bras, applying heat, or using over Phyllodes Tumor or Phylloides Tumor
the counter pain relievers (paracetamol or ibuprofen) ● These are rare breast tumors that start in the connective
● It can be drained by putting a thin, hollow needle into the (stromal) tissue of the breast.
cysts which might be done to confirm the diagnosis. ● Phyllodes tumors are most common in women in their
● For cysts that continue to come back and cause 40’s, but women of any age can have them.
symptoms, surgery to remove them might be an option. ● Most phyllodes tumors are benign (non-cancer; really big
tumors but are benign), but about 1 out of 4 these tumors
Fibroadenomas are malignant (cancer).
● Fibroadenomas are common benign (non-cancerous) ● First called cystosarcoma phyllodes
breast tumors made up of both glandular tissue and stromal ● Histologically, benign phyllodes tumors are similar to
(connective) tissue. fibroadenomas, but the whorled stroma forms larger clefts
● Fibroadenomas are most common in women in their 20’s lined by epithelium that resemble clusters of leaflike
and 30’s, but they can be found in women of any age. They structures.
tend to shrink after a woman goes through menopause. ● The stroma is more cellular than in a fibroadenoma, but
● Fibroadenomas can often feel like a marble within the the fibroblastic cells are bland, and mitoses are infrequent.
breast. Some fibroadenomas are too small to be felt, but
some are several inches across. Diagnosis
● Fibroadenomas tend to be round and have clear cut ● Painless lump, but some may hurt. (expansion/
borders. You can move them under the skin and they’re compression from other tissue) They tend to grow quickly
usually firm or rubbery but not tender. A woman can have and stretch the skin.
one or many fibroadenomas. ● Ultrasound or mammogram - hard to tell from
● Easy to remove since they are encapsulated. fibroadenomas;
● mammography has round densities with smooth borders
Diagnosis and are indistinguishable from fibroadenomas.
● Some fibroadenomas can be felt, but some are only found ● Ultrasonography may reveal a discrete structure with
on an imaging test (like a mammogram or ultrasound). cystic space.
● A biopsy (taking out breast tissue to check it in the lab) is ● An excisional biopsy- to know for sure that it’s a phyllodes
needed to know if a tumor is a fibroadenoma or some other tumor, and whether it’s malignant or not.Also used to
problem. differentiate it from fibroadenoma.
● Women with simple fibroadenomas have a slightly
BREAST CANCER
Facts
WHO/International Agency for Research on Cancer Also highest in INDIA.
Leading killer of women ages 35-54
1M develop the disease without knowing it
Phils – highest incedence rate in Asia
Phils – 9th highest in the world
Breast Quadrants
Inspection
arms up in the air
hands on her hips (with and without pectoralis muscle
contraction)
Leans forward to accentuate skin retraction.
NOTE:
o Symmetry (it is normal that it’s slightly asymmetric)
o Size and shape
o Presence of edema; nipple or skin retraction or
erythema
IN SITU CANCERS
DUCTAL CARCINOMA IN SITU
o Also known as intraductal carcinoma
o Is a non-invasive or pre-invasive breast cancer.
LOBULAR CARCINOMA IN SITU (LCIS)
o May also be called lobular neoplasia
Not a cancer, though the name can be
confusing
o Cells that look like cancer cells are growing in the
lobules of the milk-producing glands of the
breast, but they don’t grow through the wall of
the lobules.
TYPES OF THERAPY
Depends on:
o Stage of the disease
o Biologic subtype
o General health status of the patient
o Clinical stage
o Histologic characteristics
o Biomarkers
TOPICOUTLI NE Neur
obl
ast
oma 8
I. PRI NCI PLES OF PEDI
ATRIC
ONCOLOGY Sar
coma 7
I. BENI
I GNTUMORSI NCHI LDHOOD
a. NonMal i
gnantTumor softheLiver Wi
l
m’st
umor 6
b.Hepat i
cHemangi oma
c. Mes enchymalHamar toma Os
teos
arc
oma 5
d.Hepat ocell
ularAdenoma
e. FocalNodul arHyper plas
ia Ret
inobl
ast
oma 3
f. Gas t
rointest
inalStr
omalTumor s
g.Ter atoma and ot her Ger m Cell Li
vert
umor 1
Tumor s
h. SacrococcygealTumor s EPIDEMI OLOGY
II
I. MALI GNANTTUMORSI NCHI LDHOOD ● Chi ldhood c anc eri nc i
denc ei sgr eatestdur ing
a. Wi l
msTumor/Nephr obl ast
oma the1s tyearofl ife
b.Neur oblastoma ● Peaksagai nat2- 3year st hens lowl ydec lines
c. Livertumor s unt i
lage9
● I nc reas ess teadi l
yagai nt hroughadol es cence
I.PRI NCI PLESOFPEDI ATRI CONCOLOGY ● V ari
e s withg e nd er an dr a c
e
● Canc erinc hildr eni sunc ommon ○ Var iati
ons by gender ar e al s
o s een.
● Onl yabout2%ofal lcanc erc as es. Dis t
r i
but i
onbyr ac e.Whi tec hildrengener all
y
● Sec ond mos tc ommon c aus e ofdeat hi nc hi
ldren ha ve5 0% grea ter in ci
d enceo f ca ncer thand o
oldert han1yearofage blac kc hil
dr en.
● Canc eri st he s econd l eadi ng c aus e ofdeat hi n
childrenaf tert raumaandac count sforappr oximately CHI LDHOODCANCERANDHEREDI TY
11%ofal lpedi atricdeat hsi nt heUni tedSt ates. ● Cons t
itutional gene mut ations t hat ar e
● Sever alf eat ures di stingui sh pedi atri
cf rom adul t he r
e ditaryf or ex amp lep assedo nb yap aren tto
canc er s,i nc l
udi ng t hepr esenc eoft umor st hatar e ac hil
dornonher edi taryi nwhi cht her eisde
predomi nant l
y s een i n c hil
dr en, s uch as no vo mu tatio n in t h es p erm o fo ocyteb efore
neur obl as toma and ger m c el lt umor s , and t he fertil
ization- co ntri
b ute stoa ne stima ted1 0%t o
favor abler es pons et oc hemot herapyobs erved for 15%ofpedi atricc anc ers.
many pedi at ri
cs olid mal ignanc i
es ,even i n t he ● Co ns t
itutiona lc h romo s omalabnor mal iti
es ar e
pres enc eofmet astas es. the res ul
t of th e ab no rma l structu ral
● Leukemi a-mos tc ommonc anc er rear rang eme n to ft h e norma l 4 6 chromo so me
● Braint umor-mos tc ommons olidt umorofc hil
dhood andmaybeas soc i
at edwi thapr edi spos i
ti
ont o
● Lymphomaar et henextmos tc ommonmal i
gnanc yin canc er .
children f ollowed by neur obl astoma,s of tt i
ssue ○ E g.l eukemi as een wi tht risomy21 ( Down
sarc oma, Wi lm’ s t umor , ger m c ell t umor, s yndr ome )
osteos arcomaandr etinobl astoma ○ ger mc ellt umor swi thKl inef elters yndrome
● 15- 19year s-Hodgki nandGCT( Ger m Cel lTumor ) (47 XX Y).
aret hemos tf requent lydi agnos edmal ignanc y.
GENETI CSCREENI NG
● Al ong wi th an i nc r
eas e under s tandi ng oft he
FrequencyofCancerDi agnos esi nchi l
dhood mol ec ularbas isofher editaryofc hildhoodc anc er
hasc omet heoppor tuni t
yt oI dent ifyc hil
dr enwho
Typeofcancer APer cent ageofTot al
areathi ghr iskofmal ignanc y
Leukemi a 30 ● An di ns omec ase s toi n t
e rvenebef oret hec anc er
devel opsorwheni tiss ti
llcurable.
Br ainTumor s 25 ● Fami lialadenomat ouspol ypos i
s
○ Mut ati
onsoft headenomat ouspol ypos i
sc oli
Lymphoma 15 (APC)geneonc hr omos ome5q21.
○ Leadt ot hedevel opmentofi nvas i
vec olorec tal
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LAMORI
N
carci
noma wit
hanabdomi nalpai
norabdominalmas
s
○ Hence,i
dentif
icat
ioninthispati
ent/indi
vi
dual
, ● Fet
alhydropshave been i
denti
fi
ed bypr enatal
Prophyl
act
iccolectomyisrecommended ul
tras
onography in some fetuses wit
h l iver
○ Alsoati
ncreasedriskofhepatobl
astoma. hemangi
oma.
● Famil
ialThyr
oidCancer. DIAGNOSIS
○ 100% as soci
ati
on between ger
mli
ne RET A.Laborator yTes t
mutati
ons ○ Ser um al phaf etoprot ei
n( AFP)i spres enti n
○ Prophylacti
cthyroi
dect
omyisr
ecommended veryhi gh c oncent r
ations( nor mally,nor mal
i
nnewbor n)atbir t
h( 48,000-35, 000ng/ ml )
II
.BENI GNTUMORSI NCHI LDHOOD ■ Rapi dlydec li
nest o adul tl evelsofl ess
than10ng/ mlby8mont hsofage
A.NonMal ignantTumor soft heLi ver ■ Mar kedlyel evat edAFPl evelsinac hi l
d
● Pri
maryl i
vert umor sc ons ti
tutel esst han 3% of withal ivermas sal mos tc ertai
nlymeans
tumorss eeni nthepedi at r
icpopul ati
on thatt hemas si smal ignant ;ifassoc i
at ed
● Onlyonet hir
doft hoset umor sar ebeni gn with a mas s,i t usual lyi s a par t of
○ Epi thelial (which c ompr i
ses f oc al nodular Kasabac h-Mer it
t s yndr ome r esulti
ng
hyper plasi
a, hepat ocel
lular adenoma) from presenceo f l
iver h ema ngioma
Mes enc hymal (hepat ic hemangioma, ○ Thromboc ytopenia
mes enc hymalhamar toma) ○ Hypot hyr oidism mayal s ooc curi nmul tipl
eor
○ Ot her( teratoma,inflammat oryps eudotumor ) diff
us ef or msofl iver.Thyr oi
df unc ti
on t es t
s
○ Nonpar asi
ti
c c ysts al so i ncluded bec aus e should be done r out i
nelyi nt hesec hil
dr en
alt
houghi ti snotaneopl asiabuti tisals
oa bec ausehypot hyroidisms ignificantlyi mpac t
s
commonnonmal ignantt umoroft heli
ver theirmoney?
Cl
inicalMani f
estation B.ImagingTechni ques
● Mos tc hi l
dren wi t h beni gn livert umor spr esent ○ Supi ne r adiogr aph oft he abdomen ( initi
al
withapai nlessr ightupperquadr antabdomi nal ima ging )
mas sorhepat omegal y. ■ Cal ci
f i
c ati
onswi thint hemas s
● Gas troint esti
nalc ompr ession,s uchasc onst i
pation, ○ Abdomi nalul trasonogr am wi thDoppl er
anor exi a,orvomi tingmayal s obepr esent. ○ Comput edt omogr aphy( CT)wi thi ntravenous
● Ift hemas sispai nf ul,t hapai nus uallyisdul land cont rast
achi ng and i sc aus ed byexpans i
on oft hel i
ver ○ Magnet icr esonanc ei magi ng( MRI )
caps ul eorc ompr es sionoft henor mals urroundi ng ■ When s urgi c
alr esec ti
on i spl anned and
struc ture. mor e det ail
ed i nf ormation about t he
● J aundi ceandwei ghtl ossar eunc ommon vas cul aranat omyr elati
vet ot het umori s
○ Exc ept i n i nf ant s wi th s ympt omat ic des i
r ed
hemangi omas ■ Ori ni nfant swi thhemangi omai nwhom
○ Rai ses uspiciont hatt hel esioni smal i
gnant anot her di agnos i
si s bei ng c ons i
der ed,
● Ac uteabdomi nalpai nmaybec aus edbybl eeding bec aus et he MRIappear ance may be
i
nt ot hemas sori nt ot heper i
toneum par ti
cularly diagnos t
ic
i
nhepat ocell
ularadenoma ○ Ar t
er i
ogr aphyr es ervedf orc hildrenwi thl i
ver
● Al thought hiscondi ti
onar erarelys eeni nc hil
dr en, he ma n gio ma o r A V ma l
forma t
ion w it
h a
childrenmaypr es entwi thc onges tivehear tfail
ure conges ti
ve hear tf ail
uref orembol ization of
(CHF)andt hromboc ytopeni a thebl oods uppl yt ot het umori sneededf or
○ Known as t he Kas abac h- Mer r
itts yndr ome treatment
whenas soc i
at edwi thavas cularanomal ys uch
asl iverhemangi oma B.Hepat i
cHemangi oma
● Cut aneoushemangi omasar es eeni nabouthal fof ● Mos tcommonbeni gnl i
vert umori nc hildren
children wi th al i
ver hemangi oma and r apid ● Mos tar ei dent i
fiedi nt henewbor nper iodor
enlar gement of t he l i
ver wi th a di f
fus el i
ver dur i
ngpr enat alul trasounds creening
hemangi omac anc aus eAbdomi nalCompar ment ● Cl
t ass
ifi
edas :
Syndr ome and r es pirator y di stress.Cut aneous ○ Foc al
hemangi oma maybe c l
ues i n di agnos ing l i
ver ○ Mul tif
oc al
hemangi oma es pec i
al l
yi ft he pat ientpr esent s ○ Diffuse
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LAMORI
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y c ys t
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-- ○ Orpr edomi nant lyvas cular- >pres entwith
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fi
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● TX:Compl et eoper ativer es ection( treatment
l
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○ Managementmus tbe t emper ed by t he
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ldrentreatedwi thanabol i
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seonT2-
● Multi
plebloodt ransfusi
onsforc hronicanemi a
weighted
● Chi
ldrenwi thtype1gl ycogens t or
agedi s ease
sequences
-thedevel opmentofhepat oc ellul
aradenoma
i
n pat i
ent wi th type 1 gl yc ogen s t
or age
di
seaseincluding:
○ r egi
onali mbalanc eininsul
inandgl ucagon
metabol i
sm bec auset hesehor monesar e
i
mpor t
anti nr egulati
onoft hehepat ocyte
prol
if
erationandr egenerationand
○ t heset umordevel op as a r es pons et o
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LAMORI
N
glyc ogenover loadand commont hangonadals itewher easi nadul t
sonl y
○ par tl
ys ec ondar yt oonc ogeneac tivat ion 10%abnor mal
● Res embl esHCCbutwi thoutmi tos es ● Ext ragonadalt umors i
tesabnor malmi gr ationof
● Ser um AFPandl i
verenz ymesar ewi thi nnor mal thepr imor dialger mc ellsr es ultsi nt hedepos it
ion
range ofc ellsint he
● Propens i
ty f or i nt ra abdomi nal s pont aneous ○ Sacr ococcygeal r egi on, r etroper itoneum,
rupt ur eandbl eedi ng medi as ti
num andpi nealgl andoft hebr ain
● TX:Compl etes ur gic alr es ec tion ● Ger m c el lt umor demons trate a bi modalage
dist
r i
but ionwi thpeaksat2and20year sofage
E.Foc alNodul arHyper pl as i
a
● I
rregul ar lys haped,non- tenderl i
vermas s Classi
fi
cat ion
● Frequent lyf oundi nc i
dent al lyatl apar otomy ● Semi nomaordys germi nomai sapr imiti
veger m
● Femal e- to- mal er at ioi sappr oximat ely4: 1 cellt umor t hat l ac ks t he abi l
ityf or f ur t
her
● CTt ypi cal lys howsahyper vasc ularl es i
onwi tha diff
er ent iation
dens es tellat ec ent rals car ○ I ffol l
owsnor malmi grat ioni ts ettleswi thint he
○ t hes e l esion r ar ely bec ome mal ignant or gonads i f abnor mal i t depos i
ts i n t he
hemor rhagi c ther ef or e expec t ant i s ext ragonadals i
tes .Ifi tunder goesneopl asti
c
appr opr iate when r emoval mi ght be differ ent i
ationandi tal readyl ackst heabi l
it
y
as s oc iat ed wi ths igni ficantmor bidityi ft he to di f
ferent iate t hes e c ells ar e c al
led
c hildi sas ympt omat icandt hedxhavebeen semi nomaordys ger mi noma,i ti sunus uali n
made c onc lusivel y by r adiogr aphi cs tudies childhoodandoc cur smos tfrequent lyint he
bei ngnor malAFPandbi ops y ○ Medi as ti
num,pi nealgl and and t he gonadal
● Arter i
ogr aphy or magnet ic res onanc e sitesdur ingt headol escentyear s
angi ogr aphy ● I fther ei sdi f
ferent i
ationi tbec omes Embr yonal
○ Hyper vas cular mas s wi th f eedi ng ar t
eries carcinomai sc ompos edofc ell
sc apabl eoff ur t
her
ent er ingt heper ipher yandc onver gi ngont he diff
er ent iationi nt oembr yoni corext raembr yoni c
c ent r alpor t i
onoft het umor ● Ter at omasar et hemos tc ommonger mc elltumor
● Mic ros c opi c exami nat ion s hows pr olifer at i
on of andar ec ompos edofel ement sf rom oneormor e
hepat oc ytes and bi l
e duc ts and t he oft he embr yoni c ger m c elll ayerand c ont ai
n
pat hognomoni cc ent ralf i
br os i
s ti
ssuef or eignt ot heanat omi csiteofor i
gin
● Tx:expec t antmanagement ● Mat ur eand i mmat uret erat omasar ec ons i
der ed
beni gnl es i
onsi tishoweveri mper ativet ohavea
F.Gas troi ntes t i
nalSt romalTumor s thoroughandac c uratepat hol ogi cr eviewbec ause
● Rar e mes enc hymal t umor s, or i
ginat e f rom 25% oft hes eger mc ellt umor si nc hil
dhoodar e
i
nt es tinalc el lofCaj al mixedwi t hmor et han1hi stolicf eat ure
● Cellul ar s pindl ec el l,epi thel i
oid,or oc c asional
pleomor phi c mes enc hymalt umor st hatexpr es s
theKI T( CD117)
● Mar ker ss uc h as CD34,s moot h mus cle ac tin,
des mi n,andS- 100pr otei n
● Themos tc ommon s itei st hes tomac h( 50% t o
70%) ,f ollowedbyt hes mal li ntes ti
ne( 20t o30%) ,
colonorr ec tum ( 10%) ,andes ophagus( 5%)
● Gener al izedabdomi nalpai n,dys peps i
a,andoc c ult
GIbl eedi ng,I DA( s houl dpr omptani nves tigation
toexc ludeGIt rac tmal ignanc yasac aus e)
● Palpabl eabdomi nalmas sori ntes tinalobs truc t
ion
● Plainabdomi nalXr ay,CT,endos c opy
● Tx:c ompl etes ur gic alexc is i
on
Classi
ficat i
ons ystem f orDevel opmentofGer m Cel l
B.Ter at omasandOt herGer m Cel lTumor s Tumor s
● 20%ofpedi atricger mc el ltumor sar emal ignant
● 1%t o3%ofal lmal ignantt umor si nc hil
dhoodand
adol es c enc e
● I
nc hildr en,t heext r agonadalt umors i
t ei smor e
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LAMORI
N
St
ageI
I-I
Vtes
tes Surger
yandpost
-op
Chemo- PEBx3c
ycl
es
St
ageI
-IIext
ragonadal COG,AGCT0132
Hi
ghri
sk Surgeryandpos t-
op
St
ageI
II
-IVext
ragonadal chemot herapywi t
h
St
ageI
Vovary plati
num- based
etopos i
dephos phate
andbl eomyc i
ns ul
fate
(PEB)pos t-opor
adjuvantchemot herapy
H.SacrococcygealTumor s
● Mos tc ommonext ragonadalt umorinneonat esas
compar edt oadul tswhi charet hegonadalt umor
ofager mc el
ltumor
● Ac c
ount i
ng f orup t o 70% ofal lt eratomasi n
chil
dhood
● 3t o4:1f emaletomal erati
o
● A newbor n specifi
call
y pr esent with a mas s
prot r
udi ngf r
om t hes acralregion,andmanyar e
detec tedwi thprenat alult
rasonography
● I
famas shasbeennot edatbi rthandl eftinplace,
● Ger m c ellt umor demons t
rat e a bi modalage
ani ncreas erateofmal i
gnancyhasbeenobs erved
di st
ribution
● Tx:Compl etes urgicalexc i
si
on ass oon asi tis
● wi thpeakat2and20year sofage
diagnos ed
● Thepr es enceofani ntersexdi sorder→ i sknown
(increas ed r i
sk i n devel oping) f actor f or
CLASSIFI CATION AND ASSOCI ATION WI TH
GONADOBLASTOMAi sani ns i
tuger mc elltumor
MALI GNANCY
wi th an abi l
ity t o di fferentiate i nto a
● I nt he s tudy per for med by Out mann and
dys ger minoma, i n mat ure t eratoma, or yol k
coll
eagues ,t hey have f ound out t hat t he
subt umororc arci
nomawhi char eoft hemal ignant
mal i
gnanc yr at
ei nc reased wi th the mor e
for m
hidden ort ype 3 or4 l esions.Thi ss urvey
● One r i
s k group i nc l
ude t estos ter
one def iciency,
notedt hel ow r ateofmal ignanc yinneonat es
andr ogen i nsens i
tivi
tys yndr ome and 5- alpha
andyoungi nf antsl es st han2mont hsofage,
reduc t
as edef i
ciencywhi c har eandr ogendef icient
andhi gherr at einol deri nfantsandc hildren
mal es
mor ethan2mont hsofage.
● Gonadaldys genes isi sassoc iatedwi t
ht her iskof
● Sever als ubs equents tudies have c onfirmed
mal ignanc ywi th10% at20,and19% at30year s
thisand not ed mal ignanc yr at
esashi gh as
ofage
90%.
● Undes cended t esteshave an i ncreased ris k of
mal ignanc ywi thar atehighes tf orintraabdomi nal
● Thet ype1out mannc las si
fic
at i
onofSCTwoul d
tes t
is appr oximat ely .4% of al l males have
meanexc l
us ivelyout sidet het umor ,mos toral lof
undes cended t estishoweveri twasobs erved i n
thet umori snot edout s ideoft hes acrum.
3. 5to12%oft es t
icularcanc erpopul ati
on
● Type 2 - par tl
y ext ra- orpar tlyi nt
ra- sacral=
partlyintrapel vic
Risk-bas edScheme
● Type3-maj or it
yoft het umori sintrapelvic
Lowr i
sk Sur ger yalone ● Type4-al loft het umor i sl ocatedwi thi
nt he
Stage1ovar y COG,AGCT0132 pelvis
Stage1t es t
es
I
mmat uret eratoma *hi
gherr ateofmal ignanc yf ormor ehi ddent umor s
andf ort hoset umor sthatwer enotexc isewi thint he
I
nt ermedi ater i
sk fi
rsttwomont hsofl ife.Itisver yimpor tantthatwhen
StageI I-I
IIovar y yous eet hesepat ient sint henewbor nper i
odori nt he
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LAMORI
N
f
ir
stt
wo monthsofli
fe,you r
ecommend t
hatt
he oft
het
umor
t
umormus
tber
emoved.
StagingofWT
I
II
.MALI
GNANTTUMORSOFCHI
LDHOOD Table 30-1 Chi
ldren’sOnc
ology Gr
oup (COG)and
Sociétéint
ernat
ionaled'
oncol
ogi
epédiat
ri
que(SI
OP)
A.Wi l
msTumor/Nephr oblastoma StagingSystems
● Wi l
ms ’t umor i st he mos tc ommon pr i
mar y
malignantt umoroft heki dneyinc hil
dr en
COGWi
l
lms
’TumorSt
agi
ng
● 2nd mos tc ommon abdomi nalt umori nchil
dr en
(only2ndt oneur obl as toma) St
ag Cr
iter
ia
● Mos tar edi agnos edbet ween1and5year swi th e
thepeaki ncidenceatage3.
● Thet umor sus uallydevel opi not her wiseheal thy I Thet umorisli
mitedt ot hekidneyandhas
chil
drenasanas ympt omat i
cmas sint hefl
ankor beenc ompletel
yr esected
upperabdomen Thet umorwasnotr upturedorbi opsi
ed
● Frequent l
yt he mas si sdi sc
over ed by a par ent pri
ort oremoval.
whilebat hingordr es singt hec hil
d Thereisnopenet rationoft herenalcaps
ule
● Others ympt omsi nc l
udeHyper tension,hemat uria, orinvolvementofr enals i
nusvessels
.
obstipati
on,andwei ghtl oss
● occas i
onally ,t he mas si sdi scovered f ol
lowi ng I
I Thet umorext
endsbeyondt hec apsuleof
bluntabdomi naltrauma thekidneybutwasc omplet
elyres ect
ed
● Near l
y97% ar es por adi c-i nt hattheyyoc curi n withnoevidenc eoftumoratorbeyondt he
theabs entofher itabl eorc ongeni t
alc auseorr i
sk mar gi
nsofresecti
on
factor Thereisnopenet r
ationoftherenalc apsul
e
● I
fwi thher i
tabler i
s kf ac t
or-pr esentatanear li
er orinvasi
onoft herenalsi
nusvess els
.
age( theaf fectedc hildof10%) ,andt het umor s
aref r
equent lybi l
ater al. I
II Grossormi cr
os copicres idualtumorr emai ns
postoper ati
vely,incl
udi ngi noperablet umor,
Geneticpredispositi
ont oWI lms’tumor posi
tives urgicalmar gins ,t
umors pi l
l
age
● WAGRs yndrome surf
ac es,regionallymphnodemet astases,
- Wi l
ms’ t umor , Ani ridia, Gyni t
our
inar
y posi
tiveper it
onealc ytol ogy,ortrans ected
abnormal it
ies,andment alRet ardati
on tumort hrombus .
- Knownt or esultsfr
om adel eti
onofonecopy Thet umorwasr upturedorbi opsiedpr iorto
eachoft heWI lms’tumorgene,WT1 removal.
● Bec kwith-Wi edemanns yndr
omei sanover gr
owth
syndrome I
V Hematogenousmetast
asesorl
ymphnode
- Vi scer
omegal y, macroglossia, and metast
asesoutsi
detheabdomen(
e.g.
,l
ung
hyperinsuli
inemi chypoglycemia l
iver
,bone,br
ain)
- Mut at
ionsatt he11p15,locus
V Bilat
eralrenalinvol
vementi
spr
esentat
● Hemi hyper tr
ophy
diagnosis
,andeac hsi
demaybeconsi
dered
tohaveas tage.
Preoperat
ive evaluat ion bef ore oper at
ion, pati
ents
suspect
edofWi ll
’stumors houldunder go: Ni
cet
oknow!
● Abdomi nalc hes tcomput er i
zedt omography
○ Char ac terizethemas s
SI
OPSt
agi
ng
○ I dentif
yPr esenceofmet astases
○ Pr ovide I nformati
on on t he oppos i
te St
age Cr
iter
ia
kidney
○ Pr esenc eofnephr ogeni crestswhichisa I Tumori sli
mi t
edt otheki dneyor
precurs orr eason t ot he developmentof sur r
oundedwi thaf i
brousps eudocapsul
e,
Wi l
l’
st umor . i
fout sidet henor malcont oursoft
he
● Abdomi nalUS kidney.Ther enalcapsuleor
○ Eval uat et he pres ence ofr enalvein or ps eudoc apsulemaybei nfil
tr
atedwiththe
vena caval ext ension whi ch i s a tumor ,buti tdoesnotr eac htheouter
contraindi c
ationofac ompleteresec
tion
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LAMORI
N
surface,anditisc ompletel
yr esected.The uri
narybladdermus tbec ompl
etel
yremoved.
tumormaybepr otr
uding( bulgi
ng)intothe Al
sot hes uspect
edenlargedlymphnodemus t
pelvicsystem anddi ppingintot heureter, betakent ogetherwit
ht hespeci
men.
buti ti
snotinfil
trati
ngt hewal ls
.Theves sel
s ● Compl et
er emovalofkidneyuptodist
alur
eter
ofther enalsi
nusar enoti nvolved.Intr
arenal +LNs ampl ing
vesselsmaybei nvolved.
B.Neur oblas toma
I
I Thet umorext endsbeyondt hekidneyor
● Mos tcommonabdomi nalt umori nc hildren
penet r
at est hrought her enalc apsule
● Third mos tc ommon pedi atric malignanc y-
and/orf ibrousps eudoc aps uleintot he
10%ofal lc hi
ldhoodc anc ers
perir
enalf at ,buti tisc ompl etelyresected.
● Patientshaveadvanc eddi s easeatt het imeof
Thet umori nfil
tratest her enals i
nus
present ationunl ikewi tht heWi l
m’ st umori n
and/ori nvadesbl oodandl ymphat i
c
whic hc ur ei sexpec ted i nt hevas tmaj ori
ty.
vesselsout sidet her enalpar enchyma,but
The over alls urvivalof Neur obl
as toma i s
i
tisc ompl et elyres ected.Thet umor
si
gni f
icant lylowerc ompar edt oWi lm’ st umor.
i
nf i
l
tratesadj acentor gansorvenac ava,
● 80%ofc as espr esentbef or et heageof4year s,
butitisc ompl etelyres ec ted.Thet umor
andt hepeaksat2year sofage
hasbeens urgicallybi ops ied( wedge
● Arisefrom t heneur alcr estcel ls
biopsy)pr i
ort opr eoper ative
● Originatesf r
equent lyatt heAdr enalgl ands,
chemot her apyors urger y.
posteriormedi astinum,nec korpel vis( butcan
I
II Ther ei sincompl eteexc i
s i
onoft het umor, ari
sei nanys ympat het i
cgangl i
on)
whi chext endsbeyondr es ec ti
onmar gi
ns ● Thec l
inicalpr esent ationdependsont hesite
(gros sormi c r
osc opictumorr emai ns oft he pr imar yt umorand t he pres enc e of
pos toper ati
vely). met astases .
Anypos it
ivelymphnodesar einvol ved.
Tumorr upturesbef oreordur ings ur gery Cl
ini
calpresent ation
(i
rres pec t
iveofot herc ri
ter i
af ors tagi ng). ● ⅔ of t hist umor s ar ef irs
t not ed as an
Thet umorhaspenet ratedt heper i
t oneal Asympt omat icabdomi nalmas s .
surfac e.Tumori mpl antsar ef oundont he ● The t umormay c r
os st he mi dl ine and t he
peritoneals urface.Thet umort hrombi majori
tyofpat i
ent swi llalr
eadys how Si gnsof
presentatr esection,mar ginsofves selsor metastat i
cdi s
eas e-pai nfrom t het umormas s
ureterar etransec t
edorr emoved orfrom bonymet astases
piecemealbys urgeon. ○ I fyous eeac hil
dlimpi ng,c ompl aining,
unabl et owal korc ompl aini ngofany
I
V Hematogenousmet astas
es(l
ung,l
i
ver
, j
oi ntpai nwhi chisver yunl ikel
yf ora
bone,brain,etc.)orlymphnode child,wemi ghtbeabl et opal pat ean
metast
as esar eoutsidethe abdomi nalmas s.A neur obl astoma i s
abdominopel vicregi
on. highlyc ons i
der ed.
● Sever ewat erydi arrheaduet othes ecretionof
V Bi
lateralrenaltumorspresentat VIP (vas oactive i ntes t
inalpept i
de) by t he
di
agnos i
s.Eachsidehastobes ubstaged tumor
ac
c ordingt oaboveclas
sif
icati
ons. ● Cer ebellarataxi aorops oclonus
The COG s tagi
ng doesnoti nvol
ve a pr eoperat
ive Di
agnos
ticEval uation
chemot her
apy,itmeansthatthet umorbepr imaril
y ● Sympat het i
c ner vous s ystem - el evat
ed
resec
tedandt henanadjuvantchemos houldfoll
ow. catecholami nesandt heirmetaboli
tes
The SI OP pr ot
ocol, neoadj uvant, meani ng ○ Ser um cat echolmines - Dopami ne,
preoperati
vechemother
apymus tbei nst
itutedbefore Nor epinephri
ne
resec
tionofthetumor. ○ Ur ine cat echolamine met abol
i
tes -
VMA ( Vanil
l
ylmandelicAcid)andHVA
(Homovani ll
icAcid)
● BMA
Sur
gicalTr
eatment ● Abdomi nalCT-t ocharacteri
zethetumorand
● Radicalnephr
our
eter
ect
omy - meaning t
he i
tss usceptibil
ity
ent
ir
ekidneyuptothedis
talur
eterneart
he ● Met ast
at i
cwor kup
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LAMORI
N
○ Chestxray/CT ( any abnor
mal
it
y on ● Elevated AFP i n 90% of c hildren -
ches
txrays houldbefoll
owedbyches
t Hepat oblas
t oma>>HCC
CTscanforitsconfi
rmati
on) ● Abdomi nalCT t o ident i
fyt he lesion and
○ Bonemar rowbiopsy i
dent i
fythedegr eeoff ocalinvasi
venes s
○ Boneradionucl
idescan ● Biopsy- mus t be per f
ormed f or mal i
gnant
appear i
ng les i
onsunl esst he l
esion c an be
Pr
ognosti
cI ndicator s resect
edeas il
y.
● Anopenbi ops yisr equi redi nordert opr ovi de ● Hepat oblas
t oma i s usuall
y unifocali fitsis
ti
ssuef oranal ysist oc ategor izewhet hert he removemaj orit
ys urvi
ved
tumorhasf avor abl eorunf avor ablef eat ure. ● HCC i sof t
en ext ens i
velyinvasive and mul t
i
● ShimadaCl ass i
fi
cat ion centri
cbutonl yami nor i
tyofpat i
entshave
● Degr ee of di ffer ent i
at i
on, t he mi tos i
s- l
es i
ons amenabl et oc ompl eteresec t
ion at
karyor r
hex isi ndex, and t he pr es enc e or diagnosis.
abs enceofs c hwanni ans troma
● Ingener al,c hil
drenofanyagewi thl ocalized
neur oblastoma.Ani nf antyoungert hat1year St
agi
ngofPedi
atr
icl
ivercancer
ofagewi thadvanc eddi s easeandf avor able
diseas ec harac teristic shaveahi ghl i
kelihood
ofdi seasef rees ur vival . St
ageI No met as
tas
es, t
umor c
ompl
etel
y
● By c ontras t ol der c hildr en wi t
h advanc e
r
esec
ted
diseas ehaveas igni ficant l
ydec reas edc hanc e
ofc uredes pitedes pit eint ens i
vet her apy
● FAVORABLE
St
ageI
I Nomet astas
es ,t
umorgr os
s l
yr esec
ted
○ Hyper diploid DNA, Loc alized
tumor ,Les st han1yearol d with mi
croscopicresi
dualdisease( ie,
.
● UNFAVORABLE Posi
ti
vemar gins)ort umorr uptureor
○ N- mycampl i
fi
cation,Advanc ed tumorspi
llatthetimeofsurgery
diseas e,Ol dert han1yearol d.
● Ageofi mpor tanc e.
St
ageI
II No dist
ant met astases
, t umor
C.Li verTumor s unresec
table or r
esec t
ed wi t
h gr
oss
● >⅔ ofalllivert umor saremal ignant res
idualtumororposi
tivelymphnodes.
● 2maj orhi stologics ubgroups
a. Hepat oblastoma
- Mos tc ommon l i
ver mal i
gnancy in St
ageI
V Dis
tant metas
tasi
sr egar
dles
s of t
he
chil
dr en ext
entofli
veri
nvolvement
- Di agnos edbef oreage4
b. HEpat ocellularcarci
noma
- Peaksbet ween10and15year sofage ● Stagi ngs ystem bas edonpos tsurgicalext ent
● Theageofons etofl i
vercanc erinc hi
l
drenis oft umorands urgicalr es
ectabil
ity.Theover all
rel
atedtot hehi stologyoft het umor . survivalr at ef orc hi
ldr enwithhepat obl astoma
● Mali
gnantmes enc hymomaands ar
comasar e i
s70%,buti ti sonl y25% f orhepat ocellul
ar
muc h les s c ommon but c onsti
tut
e t he carcinoma.Chi ldren di agnosed wi ths tage I
remainderofmal ignanc y. and I Ihepat obl ast
oma have a c urer ate of
● The f i
nding of t he l i
ver mas s does not great ert han90% c ompar edt o60% f ors tage
necessaril
yi mpl yt hatmal ignanc yispresent
. I
IIandappr oximat ely20%f orstageI V.
Nearly 50% of al l mas s es ar e benign ● I
n c hi l
dren di agnos ed wi th hepat ocellul
ar
hemangi omasar et hemos tc ommon carcinoma,t hos e wi t
hs tage Ihave a good
out come,wher eass tagesI I
IandI Var eus uall
y
Cli
nicalpresentati
on fatal. The f i
br olamell
ar var iant of
● Pai nl
essabdomi nalmasswhichthe par
ents hepat ocellularc arc
inomamayhavea bet t
er
note whilechanging t
he chi
l
d’scl
othes or prognos is
whilebathi
ngthec hi
l
d.
● J aundic
e,anorexi
aandweightl
oss Goali
nmanagement
Evaluation ● Compl
ete Sur
gic
alRes
ect
ion- pr
imar
y goal
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LAMORI
N
andises sent ialf orcure SAMPLEX
● Controlofmet as tasi
sandr ecur
renc e 1. Al l ar e t rue about Fami li
al Adenomat ous
● Adequat epal l
iat i
on-f orends t
agec anc er Polypos isexc ept
● Fortumor st hatar eunr es ec t
able,preoper ati
ve a. Mut ations of t he adenomat ous
chemot herapy s hould be admi ni
s t
er ed t o pol ypos i
s c oli ( APC) gene on
reducet hes i
z eoft het umorandi mpr ovet he c hromos ome5q21.
possibili
tyf orc ompl eter emoval . b. Lead t ot hedevel opmentofi nvas i
ve
● Chemot herapy i s mor e s ucces s
f ul f or c olor ec talc arc inoma
hepat oblas toma t han f or hepat oc ellul
ar c . Pr ophyl ac tic thyr oidec t
omy is
carci
noma. r ecommended
● Areasofl oc al l
yi nvasivedi s
ease,s uchast he d. Al so at i nc reas ed ris
k of
diaphr agm,s houl dber es ectedatt het imeof hepat obl as toma.
surgery. 2. Among15- 19year sol d,t hes ec anc ersar ethe
● Forunr esec tabl et umor s,l i
vert r
ans plantat i
on mos tf requent lydi agnos edmal ignanc y.
may be of fer ed i n s elect pat i
ent s. The a. Hodgki nsandNon- hodgki n'
s
fi
brolamel lar var i
ant of hepat oc ellul
ar b. Hodgki nandGCT
carci
nomamayhaveabet terout comewi th c . GCTandLeukemi a
l
ivert ransplant at i
ont hanot herhepat oc ellul
ar d. Noneoft heabove
carci
nomas . 3.Pr i
mar yl ivert umor sc ons ti
tut esl esst han_ __of
tumor ss eeni nt hepedi atr i
cpopul ation
a. 1%
b. 3%
c . 5%
d. 10%
4.Allaret r ueofhepat oc ellul aradenomaexc ept ;
a. Tr eat ment i s c ompl ete s urgical
r esec tion
b. Res embl esHCCbutwi thoutmi toses
c . Ser um AFP and l i
ver enz ymes ar e
wi t
hi nnor malr ange
d. As soc i
at ed wi th t ype 1 gl yc ogen
s torgaedi seas e
e. Noneoft heabove
5.Origi
nat ef rom t hei ns tes tinalc ellofCaj al
a. Foc alnodul arhyper pl as i
a
b. Hepat oc ellularadenoma
c. GI ST
d. Ger mc elltumor s
6.Trueoff ollicul aradenomaexc ept ;
a. CTt ypi c all
ys howsahyper vasc ularl esi
onwi th
adens es tellatec ent rals c ar
b. Fr equent l
yf oundi nc ident allyatl apar otomy
c. Femal e- to- mal er at i
oi sappr oximat el y4: 1
d. Regul ar l
ys haped,non- tenderl ivermas s
7. Apr imi tiveger mc el lt umort hatl ackst heabi l
i
ty
forf
ur t
herdi ffer ent i
ation
a. Semi noma/ dys ger mi noma
b. Chor i
oc ar c
inoma
c. Ter atoma
d. Embr yoni cc arc i
noma
8.T/F:Inc hi l
dr engonadalt umors iteisunc ommon
9.Mos tc ommonger mc ellt umor .
10.Whati st he mos tc ommon pr imar y mal ignant
tumoroft heki dneyi nc hildr en?
11.I
nWAGRs yndr ome,As tandsf or ?
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LAMORI
N
a. Anuria
b. Ani
ridia
c. Al
coholism
d. Anaesthesi
a
12.Thefollowingar es
ympt
omsofnepr
hobl
ast
oma,
except
:
a. Hyper t
ension
b. Hemat uri
a
c. Obstipati
on
d. weightloss
e. AOTA
ANSWER:
1. C
2. B
3. B
4. E
5. C
6. D
7. A
8. T
9. Terat
oma
10.Wil
ms ’t
umor
11.WAGRs yndrome
- Wi lms’ t umor , Ani
ridi
a, Gynitour
inar
y
abnormali
ti
es,andment
alRetar
dat
ion
12.E
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LAMORI
N
● In 1 to 4 years of age
TOPIC OUTLINE: ○ Drowning was the leading cause of injury
I. EPIDEMIOLOGY OF CHILDHOOD INJURY death followed by
II. RESUSCITATION AND IMPACT IN OUTCOME ○ Transportation-related injury.
III. RESUSCITATION PRINCIPLES ● 5 to 9 years of age- lowest rate of unintentional
A. Prehospital care death
○ The most common cause of death in this age
B. Primary Surveys and Treatment of Life
group and also those children ages 10-14
Threatening Injuries years was motor vehicle occupant injury
C. Resuscitation Phase ● Male >> Female
IV. INJURIES TO THE CENTRAL NERVOUS ● In all age groups, male children are at higher risk
SYSTEM for unintentional injury than females. This can be
V. THORACIC INJURIES attributed to a variety of factors, including biology
A. Tension Pnerumothorax (differences in temperament), exposure to risky
behavior, gender socialization, and cognitive
B. Hemothorax
differences
C. Traumatic Asphyxia ● Race and ethnicity are also important factors in
VI. ABDOMINAL INJURIES the risk for unintentional injury in children.
A. Small Intestinal Injury ● Decreases in the injury death rate are due to
B. Solid organ injury multifaceted preventive strategy.
VII. CHILD ABUSE ● Intentional injury
VIII. SAMPLEX ○ result in a fatal outcome from homicide, child
abuse, or suicide.
● National and state effort in this regard have less
I. EPIDEMIOLOGY OF CHILDHOOD INJURY have result to considered reduction in homicide,
● Preventable injuries state an enormous financial child abuse and suicide and now this death
emotional and social tool on the injured children represents a smaller percentage of fatalities in
and their families as well as the society as a children in the united states.
whole. ● Recognition of this intent requires referral to the
● Worldwide, Childhood injuries are a growing child protection service for assessment
problem ● Resuscitation of this children is frequently a
● 875,000 children are killed per year challenge because Abuse may be chronic, which
● Nonfatal injuries affect the lives of between 10 to results in a child with a limited physiologic reserve
30 million born globally
● In United States, Unintentional injury is the
leading cause of death
● claiming more than 12,000 child lives annually,
or an average of 30 children each day.
● The leading cause of unintentional related death
varies according to the child's age and is
dependent on the developmental ability and
exposure to potential hazard. In addition to
parental perception is the child ability and injury
risk
● Falls were the leading cause of nonfatal injury-
15 years old. II. RESUSCITATION AND IMPACT IN OUTCOME
● Children less than 1 year of age have the highest ● Resuscitation of the injured child includes the
rate of unintentional injury-related death, with a Actions necessary to reverse and control the
rate more than twice with that of older children sudden alterations in physiologic homeostasis
○ Airway obstruction is a major pillar in this age that occur as the result of injury.
group
ENDOTRACHEAL INTUBATION:
● VASCULAR ACCESS
○ Providing of venous access in a hypovolemic
child is often a challenge due to collapsed
peripheral veins from massive blood loss
C- 4th ICS is the ideal place for thoracostomy tube ○ 2 functioning catheters is ideal
placement ○ Achieve venous access above and below the
diaphragm
○ Given the potential for extravasation of
resuscitation fluids from occult intra-
abdominal venous injuries. Nevertheless, in
children any peripheral venous access is
useful.
○ Two attempts should be made to place large
bore peripheral IVs in upper extremities
○ If percutaneous placement is unsuccessful
WOUND HEALING
● Internal and external factors and circumstances
● Tissue injury–hemostasis–inflammatory
W-PLASTY response
FLAPS
● Flap – vascularized block of tissue, mobilized
from donor site and transferred to another
location
A. Plasmatic imbibition ● Graft vs flap
○ Flaps have own blood supply
○ Occurs within 24 to 49hrs
○ Flaps are used in re-surgery when to repair
○ Blood supply comes from wound bed underlying structures later on from the first
surgery
B. Inosculation ○ Grafts, cannot be separated from the
○ After 48 hrs there will be a fine vascular underlying structures anymore
network between the wound bed and the
graft, “Kissing capillaries”. ● TYPES OF FLAPS
A. By blood supply
○ Random (Blood supply: dermal subdermal
C. Revascularization plexus), use in reconstruction of small full
○ Already have the new vessel growth thickness defect which graft are not
applicable, accepted Length to width ratio is
● These phases usually complete by day 4 or 5. 3:1
Also usually the time we open grafts for ■ Rotation: similar to transposition but
inspection. semicircular
■ Transposition: fashioned adjacent to an
● During the initial few days your graft is most area needing reconstruction and rotated
susceptible to interference, either from infection into the defect (e.g., Z plasty, rhomboid
or from mechanical shearing and hematoma or flap)
seroma. ■ Advancement: tissue is moved forward
● Suspicion for infection or hematoma - you may from the donor site along the flap’s long
open it in 3 days for inspection and try to axis rather than being rotated about a
point (e.g., V-Y advancement flap,
remove the seroma or hematoma, and hopefully
rectangular advancement flap)
there will be more chance for the graft to survive. ○ Axial (BS more discrete well described
Composite Graft vessels)
■ Head and neck flaps
■ Groin flap
■ Posterior thigh flap
B. By composition
○ Fasciocutaneous flaps
■ Supplied from vessels communicating
with the underlying superficial or deep
fascia
■ Thinner compared to musculocutaneous
flaps
■ Do not create a functional loss of muscle
in the donor site
Perforator flaps
● Flap connected to vessel of the subdermal or
subcutaneous plexus (involve a connection to
more distal vascularity than a conventional flap)
● Pink-red
macular
vascular
stains that
are present
at birth and
○ Infantile hemangioma persist
■ Most common birthmark throughout
■ Appears in early neonatalhood usually at life
2 weeks ● Become more verrucous and darker throughout
■ Rapid growth during first 6-8 months of life
infancy (proliferating phase) ● Port-wine stains (CMs of the head and neck)
■ Growth frequently peaks before the first associated with Sturge Weber syndrome
year, and then the lesions enter the (vascular involvement of the leptomeninges and
involuting phase – diminishing ocular pathology)
endothelial activity and luminal ● Pulsed dye laser treatment (better results in
enlargement infancy and young childhoods)
■ Involuted phase (in 50% by age 5, 70%
by 7 years, with continued improvement Lymphatic Malformations/ Lymphangiomas or
until age 10-12) cystic hygromas
■ In 50% of children, near normal skin is
● Never
restored
regress and
■ Treatment is largely observational
have the
■ Oral propranolol: first-line treatment for
potential to
complicated or high-risk infantile
affect
hemangiomas
underlying
muscle and
● When you administer this during the proliferative
bone
phase, the non-selective beta adrenergic
● Expand or
blockade causes rapid involution of your
contract with the flow of lymph, infection, or
hemangioma
intralesional hemorrhage
○ Vascular malformations
● Sclerotherapy - main treatment, usually done
■ Slow-flow lesions: usually these are
before surgery
capillary malformations and
● Although surgery rarely removes the entire
telangiectasias, lymphatic malformations,
lesion, surgical resection is the only possibility
and venous malformations.
for cure.
■ Fast-flow lesions: (if they have an arterial
○ Associated with significant blood loss
component) arterial malformations and
○ Potential exists for regeneration of lymph
arteriovenous malformations (AVMs)
channels and recurrence of the LMs
■ Combined capillary, lymphatic and
postoperatively
venous malformation: Klippel-Trenaunay
syndrome
■ CMs, LMs, and VMs are found; may be
associated with soft tissue and skeletal
hypertrophy in one or more of the limbs
Arterial Malformations
● Goals: remove (or at least reduce) the risk of
● Pure AMs are rare and more commonly present
malignant transformation (eg. for your giant
as AVMs (arterial and venous malformation)
melanocytic nevi, 0-5% risk of malignant
● AVMs: red violaceous skin with a palpable mass
transformation, malignant melanoma), we do
beneath.
surgery to preserve function and improve
● Local warmth, bruit, and thrill are frequently
cosmesis
present.
● Near the skin ng CMN, you expand the skin
● Ischemic changes, ulceration, intractable pain,
there, use an expander for that. Pag tinanggal
and intermittent bleeding
mo yung CMN, since you expanded the nearby
● Four stages: quiescence, expansion, destruction,
skin you can advance the expanded skin to that
and decompensation.
area (where the CMN is).
● Treatment is initiated when signs and symptoms
● You can also have Neurocutaneous
of ischemic pain, ulceration, bleeding, or
melanocytosis: a collection of melanocytes in
hemodynamic instability (stages 3 and 4) are
the leptomeninges.
evident
● Arterial embolization (to occlude nidus 24-72
V. FACIAL FRACTURES
hours before you remove it) before surgical
extirpation
A. MANDIBLE FRACTURES
● Treatment of a
frontal sinus
fracture depends
on the fracture
characteristics as
shown in the
algorithm
D. LE FORT INJURIES
C. ORBITAL FRACTURES
● Produced by splaying
apart of the
nasomaxillary buttresses
to which the medial
canthal ligaments are
● Most common facial fracture (prominent
attached
location)
● Treatment: plating or
○ Intranasal examination (check for septal
wiring all bone
hematoma) important thing to consider
fragments meticulously,
○ If hematoma is present, it must be incised,
potentially with primary
drained, and packed to prevent pressure
bone grafting, to restore their normal
necrosis of the nasal septum and long-
configuration
term mid vault collapse. The septal
hematoma usually looks like a bluish or
F. TRIPOD FRACTURES
purplish collection of blood on the side of
nasal septum. Since the nasal septum is an
●
Zygomaticomaxi-
important support structure in the mid vault.
llary complex
The septum could collapse during necrosis,
fracture, also
and causes saddle nose deformity
known as a
● Closed reduction of nasal fractures may be
quadripod fracture,
performed under local or general anesthesia
quadramalar
● Many, if not most, show some deformity upon
fracture, and
final healing
formerly referred
to as a tripod
I. PANFACIAL FRACTURES
fracture or trimalar
fracture, has four components:
○ lateral orbital wall (zygomaticosphenoid and
zygomaticofrontal sutures)
○ separation of the maxilla and zygoma along
the anterior maxilla (near the
zygomaticomaxillary suture)
○ zygomatic arch
○ orbital floor
● Fractures of multiple bones in various locations
○ May involve frontal and maxillary sinus
G. NASAL FRACTURES
fractures, NOE fractures, orbital and ZMC
fractures, palatal fractures, and complex
mandible fractures
● Difficult to reestablish the normal relationships
between facial features in the absence of all pre-
traumatic reference points
● Without proper correction of bony fragment
relationships, facial width is exaggerated, and
facial projection is lost.
● The key point is first to reduce and repair the
zygomatic arches and frontal bar to establish the
frame and width of the face.
2. True about plastic surgery except 6. Method that can revise and redirect your
a. It defies any definition and has no organ existing scars and to provide additional length
system of its own and is based on principles especially in contractures but lacks the
rather than specific procedures applicability and universality
b. It is organized on a specific organ system a. Z plasty
and has very loose boundaries and has b. W plasty
specific anatomic regions and does not c. Both
overlap with a lot of other subspecialties. d. NOTA
c. Plastic and Reconstructive Surgeons are
called at the end of the operation or 7. How to increase the likelihood of a good scar?
treatment to reconstruct what has been lost. a. Placement of sutures that will not leave
d. AOTA permanent suture marks/ prompt removal of
skin sutures
3. “Plastikos” is a greek word which means b. Wound edge inversion and apposition
a. To hide c. Both
b. To connect d. NOTA
c. To cut 8. Colony of microorganisms enveloped with a
d. To mold matrix of extracellular polymers will cause
4. The most common facial fracture is? a. Latency
a. Tripod fracture b. Antibiotic resistance
b. Mandible fracture c. Increase species diversity
c. Nasal fracture d. Quorum-sensing
d. NOEF e. AOTA
I. Ear Reconstruction
● Small helical lesions - may be addressed by wedge
excision, primary closure
● Larger defects (especially upper and middle thirds of
your ear) - may be approached by antihelical and
● Reconstruction of the nose requires appreciation of
conchal cartilage reduction patterns to reduce the
the nine aesthetic subunits defined by normal
circumference of the helix to allow primary closure.
anatomic contours and lighting patterns.
● At the same time Local flaps may be used to close or
○ Dorsum
re-create the missing tissue.
○ Sidewall (2)
○ Tip
○ Ala (2)
○ Soft triangle (2)
○ collumella
● In general, if a defect involves >/= 50% of a subunit,
the remainder of the subunit should be excised and
included in the reconstruction.
● This is a 1 stage repair for microtia, usually they put in ● Three layers: skin cover, structure support, and
an implant material to recreate the cartilage. mucosal lining
● They expand the skin envelope and insert that ● When a defect or anticipated defect is evaluated, it is
preformed, carved ear rigid framework. useful to consider what layers of tissue will be missing
● They used the antia-buch flap, if you notice this is a so that a reconstruction can be devised that replaces
helical defect which resulted from the excision from each layer.
the lesion. What happened was, there was some ● Healing by secondary intention- used in concavities
cartilage reduction/ helical reduction flaps done, so e.g. alar grooves
that you can still close it to resemble an ear. Usually ● Split or full-thickness skin graft- used in superficial
you will end up with a smaller ear and it may also have defects of the nasal dorsum or sidewall
some cupping because of the decrease size. ● Composite grafts- used for the nasal tip or rim
● Post-auricular flaps created in staged proceduresmay ● Local random pattern flaps- useful in closing small
be manipulated to create a skin tube mimicking the defects of the dorsum or tip
furled helix and bridging the gap of a defect. - May be combined with cartilage grafts if
structural support is needed
● Axial pattern flaps- used for larger defects
Scalp Anatomy