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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

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