ee nena
_VS SUSI GTS UN SANA SAL JU SVS RGA UN SHUI
164
CHAPTER 126
Vascular Tumors
Tara ier
Hora J. Frieden
Voscule anomalies aze common bith
marks! Ther chesifeation bas ofen
been problematic ‘with contradictory
and confusing descptive nomenclature.
‘Acamiletion sytem bese propor by
‘Mulliken and Glowacki was revised in
1996 by the Inemaonal Sociey for the
Study of Vascular Anomalies based on
tlie, radilogi, and. herdyeaenie
Charictersse, into vascular malforma
tion and vascular tumors? Vasculatmal-
Focrstons (sce Chap. 178) re enor of
morphogenesis wheres hemangiomas
and other vascular tomers grow y cell:
In proliferation,
“There ate several ypes of vascular eu
more, most of which occur in child
hood. These tamer have alto had con
fesingnosclogy? with dexeriptve but
imprecise terminology sich sw
fey, capt, and cavernous. Several
‘ype of hemungiemts have been de
Sctbed (ntantle, sapidly inveluting
Congenital, lobular capillary, te), 50
Srhenever pone, hemagines shoud
hot be used as a standalone noun but
rather qualified by an adjective based on
the specie condition,
Hi nramisrownoous
Epidemiology
Infantile hemangiomse (IH) are. the
most common benign tumors of child
hood, occuring in upto 10 percene of
thaldren by I year of age I co
fther types of hemangiomas and vase
Isr maltomatons Il have 9 character
inte proliferative phate folowed by 9
slower involution phase. They ate more
ommon in females @-3: ato) and in
premature infants, especially those
treighing lees than 1500 grams? Other
‘ik factrs ae Caucasian face and mul
tile gestation pregnancy
Etiology and Pathogenesis
IH are primarily composed of endothe
lal cell bu ako contain Snobs,
pevcyes, imerstial cells, and mast
Cells. although the precise pathophysio
logis mechanioms ofthe growth end i
volition of endothelial els emai un
own, ceveal recent ditcoveies have
sdvanced the understanding of IH. Clu
osesransportert (GLUT) has been
‘denied se an immunabittochemical
marker expresed in TH, regardless of
Hoge, and inthe mirovesculute ofthe
pcenta GLUT te not expressed
Scher yevelar tumnces en mabe
‘ons thus making ita specife and use
ful marker for IH. IH and the placens
share other vascular angens och 25
FoR, Lewis Y antigen, and. Meron;
the p00 alo have similar gene expres
sion profes bsseé on DNA based mi
(rosa? Both inti” and Texte
Sic" hypotheses exist to explain these
findings A somatic mutation may cause
fal aveuar precirsor col (gic
ast) to improperly differentia to
ward a placental microvascular pheno
typeof, alemativly, hemargiomss
‘may ofginate from te placental vascu
Tneue. IH lack placental tophoblaric
marker, suggesting that embolization
of the placenta iaek (as distinct from
Placenta microvasculture) is not the
Foure of."
Prolferating TH expest CD81 and
(CD54, known maskers for endothelial
cil in adsiton to CD 183, exposes in
Frimitve ell populations" This co
Expression suggests that endothelial
progenitor cell) are involved in the
Pathogenesis of IH, However, IH alo
express myeloid markers CDI, COIS,
{CB52, and CaS suggesting that these
endothelial cells are diatinc from those
formal vasculature While met IH
fe negative for lymphatic markers such
Prot, podoplanin, and D240, some
TH expres the bmmphatic marker IYVE-
1.8 This immunophenotype is simile
to the immature endothelal cells in the
celina vein, sthich can diferente
into ether Iymmphaic or blood vessels
Expression of CD81, CD34, CD13,
and LYVEs1 is markedly reduced or a
dent in other vasclir Gumors and inal
formations and ducng the ivoluing
pPhise of IH. The concept of hemang
feu call being immsrare ard core
pletely ciferenited could help explain
their pid growth daring the Bist few
‘onthe of life, fllowed by their even
tual selédestncion via apoptosis. i
Clinical Findings
STORY The cinical history is one of
fhe most important keys to diagnosing
an IH (Table 1261) Absence ae bir
fr presence sa premonitory mat, usu
ally an area of pall, elangiectasis, or
INFANTILE
HEMANGIOMAS
NCEA es
luskiness ie characteristic, whereas a
fully formed sofissue mass atthe time
ofits mow likely not an I, but an
other vascular anomaly or ether disease
[CUTANEOUS LESIONS Growth Character
tes, Though IH ae not present a ith
ss fly Formed tumors, they almost a
‘BLE 125-1
History
orn icon,
‘Var te ile pono?
‘asl arp estan?
‘Completion dung fm pronany ar
sates?
+ Ova han ng post matter
‘te ci ein wel and gating
‘ei array?
‘Hoe ere ban any estas
nr freses?
tis "bra
‘ia se ate tr?
‘os chara sas?
‘Goh ung penne
groan a cil’ sratic
ayonasittl gov, abo
hiking nse? Sa tetas
‘sector ati)
‘A empath 3 pin, hes
‘ cuca?
yer weatreis?
Fay ist
‘lst fami hitoryooranias
cater nse arrars?‘A FGURE 126-1 Vso is sgn ate arena, ee pas he, ga cason AA mats pat pati
a, ge? yrs, cig pas, apie sale
ten mistakenly diagnosed as wacheoms
Jacia, upper respiratory infection, or
croup If the hemangioma contin
enlarge, espratony distress can ensue
Snd become hfe-threstening. Prompt
‘raltton by a pedate tsacyegae
stand treatment is essential Heman-
flomat can also involve the parotid
flan, and often equie treatment due
fo deformity of adjecene structures, a,
Incare cases, high-output congestive
bear le 338
umbosacrl and Perineal Hemangionas.
Segmental hemangioras oven the
teotbonacnl ren tan have aoc
spinal, bony, and genitourinary anome
lies, Most fequenty, posterior midline
THibave a prceacy high sak of spinal