N g h i e n cmi Y hgc Y Hpc TP. Ho Chi Minh * T4p 20 * So 5 * 2016
BAO CAO M O T TRLfCJNG HOP SARCOM M 0 VIEM SAU PHUC MAC Duang Thi Thdy Vy*, Phqm Quoc Thdng*, Phan Ddng Anh Thu*, Ngo Quoc Dqt*
T O M T A T
Chdn dodn u sau phuc mqc co the gap nhiiu khd khan. Viudvi tri ndy co the xudt ngu'dn tie nhiiu loqi mo hoc khde nhau. Trong do, sarcdm ma Id loqi sarcdm thiecmg gap nhai a viing sau phuc mqc. Vi vdy, nhdn bdo cdo mot truang hap sarcdm ma viem, chung tdi Kdi cuu y vdn ve mot so'van de co dnh huang deh viec chuan dodn sarcdm ma sau phdc mqc. Do ti le tdi phdt vd tie vong cao, sarcdm ma sau phtic mqc (ian mgt phuang phdp dieu tri hiiu qud vd Hch cue. Trong do, phdu thudt ddng vai Ho chu yeii Hong dieu tri sarcdm md sau phuc mqc.
Tie khoa: u sau phtic mqc, sarcdm md, sarcoma ma viim.
SUMMARY
RETROPERTTONEAL INFLAMMATORY UPOSARCOMA: A CASE REPORT Duong Thi Thuy Vy, Pham Quoc Thang, Phan Dang Anh Thu, Ngo Quoc Dat
* Y Hoc TP. Ho Chi Minh * Vol. 20 - No 5 - 2016:88 - 91 ReHoperitoneal tumor diagnosis can be difficult. Because tumors in this locaHon can belong to many different histohgical types. Liposarcoma is the most common type of sarcoma in the reHoperitoneum. Ther^re, beside of reported a case of reHoperitoneal inflammatory liposarcoma, we revieiv literature fbr some issues that affect the diagnostic of reHoperitoneal liposarcoma. Because of high recurrence and mortality, reHoperitoneal liposarcoma need effecHve and aggressive Heatment. Surgery plays a major role in the Heatment of reHoperitoneal liposarcoma.
Keywords: reHoperitoneal tumor, liposarcoma, inflammatory liposarcoma.
TONG QUAN i'^°'^°)/ thap hem so vol nhiing lo§i mo hpc khac lal5d&i34%P>.
Nhung loai u thuong gap nhat o vimg sau , , phuc mac la sarcom mo (41%), sarcom co tron BAO CAO CA BENH
(28%), u mo bao - spi ac tfnh (7%), sarcom sgi Benh nhan nu, 76 tuoi nhap vien vi bung to.
(6%) va u bao spi than kinh ngoai bien (3%)'5'. Chan doan lam sang: U sau phiic mac nghi Sarcom mo thuang phat tri&i cham khong sarcom mo.
trieu chung cho deh khi dat kich thuoc Ion, dac n ' t h ' ' biet la 6 vimg sau phiic m?c, u co the dat den
duong kinh > 20 cm truac khi dupe phat hienii). U kich thuoc 9x5x3 cm, m | t cat mau vang Ti le tai phat co nhieu thay doi tuy theo vj tri u, ^^' ^° ^ ' ^ S ^^S ^^•
trong truang hpp u sau phiic mac ti Ie tai phat Vi the
cao do kha nang ck rpng han ehe va kha nang y gom eae te bao mo eo nhan di dang, tang xuat hien thanh phan hoi biet hoa cao. VI vay ti ^i^^ ^^ ^ ^ g ^ g ^ 5 ^ ^ ^^^ g ^ ^ c^, le hi vong khi theo doi hr 10-20 nam co the Ien ^^y^ ^^^ ^^^ ,^ ^ g thoai hoa nhay, voi d ^ 80% va thai gian Hi vong tnmg bmh la 6 ^^^ j a i bao di dang, xen ISn la cac te bao viem d m 11 nam(^'. Tuy nhien, di can xa voi ti le thap
* Bp mon Giai Phau Benh - D?i hoc Y Dupe TP. HCM
Tdc gid lien lac: PGS. Ngo Quoc Dat DT: 0903.619.468 Email: [email protected] man, eo noi t?o nang lympho.
88
Y Hoc TP. Hb Chi M m h * Tap 20 * So 5 * 2016 N g h i e n cim Y hpc
Hinh 1: Vi the. (A) Tebdo viem mqn thain nhap lan toa, cd ISc, (C, D) Nguyen bdo tna vdi nhan tang sac, di dqng rdi rdt BANLUAN
Theo so U^u thong ke lai eae truong hpp sarcom phan mem ciia Vien ung thu quoc gia Hoa Ky Hx nam 2009 den 2013, sarcom mo ehiem ti le eao nhat trong cac loai mo hpc ciia u phan mem va u sau phiic mac - phiic mae<'>. Vi vay, co the noi vi tri dong vai tro rat quan trpng trong viec xac dinh ban chat oia u sau phiic mac.
Neu so sanh voi eae loai u phan mem khac, hoa mo va hoa mo mien dich chi dong mpt vai tro han che trong chan doan u mo. Men tang chah doan cho hau het cac truang hap chii yeu dua vao lam sang, hinh anh hpc va tieu ban H&E truyen thong. Trong do, nguyen bao ma la mpt dac diem ehia khoa ve mo hgc giiip chuan doan. Cac tieu chuan chan doan nguyen
ti tqo thdnh nang lympho, (B) Viing tebdo u xip dang xoay ong md dim
bao mo bao gom: (1) nhan tang sac, vi^n nhan 16m hoac dgng v6 so sac net, (2) bao tuong chua nhiing gipt giau Hpid (chat beo trung tinh) va (3) dac diem mo hen "phu hpp" chan doan sarcoma ma. Tuy nhien, cac tieu ehuah nay van can can than khi danh gia vi co nhieu truong hpp te bao giong - nguyen bao mo va te bao - gia nguyen bao mo nhu; thue bao rai rac trong mo mo hoai tii, te bao mo teo det nang, te bao di dang chiia nhay trong cac u ac tinh khae u mo va anh gia do co dinh hoae sau dieu tri u ac tinh. Ben c^nh do, mot so truang hpp benh lanh tinh van c6 su hien dien eua nguyen bao mo nhu: u mo di dang/te bao hinh thoi, u mo dang sun va u nguyen bao sun. Vi vay, hien dien nguyen bao mo don dpc khong dii de chuan doan sarcom mo va ehah doan u mo co
N g h i e n cmi Y hpc Y Hpc TP. Hb Chi M i n h * Tap 20 * So 5 * 2016
the dat ra trong truong hpp khong thay su hien dien eua nguyen bao ma (nhu trong truong hgp sarcom mo biet hoa ro va mgt so truong hgp sarcom ma nhayi*'.
Bdng 1: Thdng ki cua Vien ung thu qud'c gia Hoa Ky tie nam 2009 deh 2013 ve cdc loqi md hgc thuang gap cua u phdn niim vd u sau phiic mqc - phuc mac^>
Lo^i m6 hQC Sarcom spi U mo hko so[ ac tinh
Sarcom soi bi loi Sarcom mo Sarcom ca tron Sarcom co vSn Sarcom co' v3n dang
phoi Sarcom mach mau U mach chu bao ac
tinh Sarcom Kaposi U vo bao soi than
l<inh actinh U nguyen bao than
kinh Sarcom mang khap
U tnjng mac ac Sarcom xiKyng ngoai
tnjc va dai bdo Sarcom nhay Sarcom NOS Sarcom dang bilu
m6 Sarcom te bao sang,
NOS
Phin mgm S6 (uwng 1.018
982 652 2.230 1.775 381 165 525 73 116 184 84 608 434 88 2.736
194 80
Tile 7.6%
7,4%
4,1%
16,8%
13,3%
2,9%
1,2%
3,9%
0,5%
0,9%
1,4%
0,6%
4,6%
3,3%
0,7%
20,6%
1,5%
0,6%
Sau phuc mac vd phuc mac S6 Ju>ang
21 461 409 16
109 412
163 Til6 1,0%
22,1%
19,6%
0,8%
6,2%
19,7%
7,8%
Phan loai u mo theo To chiie y te the gioi nam 2013 doi chieu voi phan loai nam 2002 co mpt so thay doi.
Trong do, thuat ngu "Sarcom mo biet hoa ro" khong xuat hien cimg voi thuat ngii "U mo khong dieh hinh", khong con thuat ngu
"Sarcom mo te bao tron" (dong nghia vdi
"Sarcom mo nhay") va ciing khong con phan nhom "Sarcom mo loai hon hop".
Bdng 2: Phan loai uma theo VmO 2013 vd 2002 WHO 2002**' 1 WHO 2013"^'
Ldnh tinh Umo' B^nh u mS nhieu nci B$nh u mff nhieu noi cua spi
than kinh U nguyen bao m&/benh nguyen
bao mo' nhieu noi U mo" madi meiu
Um&co U md dang sgn U m& Off mgch mau ngoai thgn
U met tuy ngoai thu-iyng than U mo di dang/tl bao hinh thoi
U mo nau
U mo B^nh u m& nhieu noi Benh u m& nhilu noi cua soi thin kinh U nguy§n bao mo U mo mgch mau Umo CO U m6 dgng sun
U m& di dang/tl beio hinh thoi U mo nSu Trung gian {tiln triln t^i chl) U m& khong a i n hinh/sarc^m
m5 biet hoa ro U m& khong dien hinh Ac tinh
Sarcom mo" hli biet hoa Sarcom mo nh^y Sarcom mo te b^o tron
Sarcom m& dj dang Sarcom mo logi hon hop Sarcom mo kh5ng die hi#u
Sarcom mo hoi biet h6a Sarcom mo nhay Sarcdm mo dj dgng Sarcom mo' khong dgc
hieu
"U mo khong di&i hinh" va "sarcom mo biet hoa ro" dong nghia vai nhau vi co chung dac diem hinh thai va di truyen. Nguoi ta nhan thay, Sarcom mo biet hoa ro khong co kha nang di can neu nhu khong co thanh phan hoi biet hoa. Vi vay, vao cum thap nien 70, nguoi ta sii dung thuat ngii "U ma khong dien hinh" cho nhiing ton thirong a nhung vung de dang phau thu|t nhu chi va than minhw. Ngupe lai, doi voi nhiing ton ttiuong 6 nhihig vimg khong the phlu thuat rpng dupe nhu sau phuc mac, trung that, tai phat tai cho gan nhu chSc chan va dan d&i tii vong, ngay ca khi khong co thanh phan hoi biet hoa hay di can. Vi vay, thuat ngii "sarcom ma biet hoa ro" phu hpp hon trong truong hpp nay. Do do, viec su dting thuat ngii nao Ia tiiy thugc vao each trao doi ciia nha giai phau benh va phau thuat vien de tranh nham lan va dieu tri khong phu hap(^.
U mo khong di«i hinh la nhom thuang gap nhat ciia sarcom mo, chiem ti le 40-45%. B?nh
90
Y Hpc TP. Hb Chi Mhih * Tap 20 * So 5 * 2016 N g h i e n ctru Y hpc
thuang gap 6 nhom tuoi trung nien, nhat la b khoang 60 tuoi. Cac nghien ciiu khong ghi nhan eo sir khac biet ve gioi tmh. U thuang a mo mem sau ciia chi, nhat la dui sau do la sau phiic mac, quanh tinh hoan va tnmg that<i). U CO the phat trieh eham khong gay dau cho den khi dat kich thuoc Ion. Dae biet la o vung sau phiic mac, u eo the dat den duong kinh > 20 cm truoc khi dugc phat hien. U mo khong dieh hinh CO the phan thanh 3 phan nhom dua vao dac diem mo hgc: dang mo, xo hoa, viem"").
Tiiy vao thanh phan mo hpc hien dien ma co cac chah doan phan biet khac nhau: (1) thanh phan xo hoa neu hien dien qua trgi co the dan den bo sot cae vimg biet hoa theo huong mo;
(2) te bao viem ttiam nhap qua miic co the can phan biet vai benh nguyen bao spi co viem, benh Castteman va lymphom Hodgkin va khong HodgkinW. Cac dang mo hpe co the kg't hpp trong cung mgt truang hpp, nhat la vol u 6 sau phiic mac. Nhu da nhac den 6 tren, yeu to tien lupng quan trgng nhat la v; tri oia khoi u.
Ngoai ra, vi tri u va gjai doan ding dan den su khac biet ve kha nang xuat hien thanh phan hoi biet hoa: > 20% vcn. u 6 sau phiic mac va < 2%
voi u 6 chi. l i le tli vong khi theo doi hi 10-20 nam thay doi tu 0% trong truong hap u 6 chi deh 80% doi vai u sau phiic mac, thoi gian tir • vong trung binh la 6 den 11 nam'">.
Phau thuat la phuang phap dieu tri chuan cho sarcom mo. Sarcom mo sau phiie m?c can mpt phucmg phap phau thuat tich arc, bao gom phau thuat nhieu Ian hoac phau thuat cat bo eae co quan khi xam lan hay tai phat. Di can xa voi ti le thap (7%), thap hon so voi ti le di can oia nhiing loai mo hpc khac (15 den 34%)<^'.
KETLUAN
Sarcom mo la loai mo hpc thuang gap nhat ciia u sau phiic mac. Vdn de chan doan sarcom mo chli yeu can dua vao thong tin lam sang, hinh anh hgc va tieu ban nhupm H&E. Trong do, vi tri u dong vai tro quan trgng. Su hien dien eua nguyen bao ma la mgt dau hieu cHa
khoa cho chan doan, tuy nhien can than trpng khi danh gia. Sarcom mo viem sau phiic mac la mpt dang mo hgc thupe nhom sarcom mo biet hoa ro. Nhung do vi tri u sau phiic mac nen co ti le tai phat va tir vong cao, vi vay can mot phuong phap dieu tri tich ctrc.
TAI U E U THAM KHAO
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W H O Classification of Tumours of Soft Tissue and Bone.
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228:355-65.
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1984;200:20fr4.
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9 National Cancer Institute. Soft Tissue Sarcomas-Histologic Type at Parenchymatous and Nonparettchymalous Sites {Soft Tissues, Heart and Mediastinum, Retropentoneum and Pentoneum, Pleura, Spleen) Among Histologically Confirmed Cases 2009-2013 All Races, Both Sexes. Table 34.1
10. Weiss SW (1994) Histologic Typing of Soft Tissue Tumours.
World Health Organization Histological Qassification of Tumours Spnnger Berlin.
11. Weiss SW, Rao VK (1992). Well-differentiated liposarcoma {atypical lipoma) of deep soft tissue of the extremities,retroperitoneum, and miscellaneous Siles. A follow-up shidy of 92 cases with analysis of (he inddence of
"dedifferentialion" A m J Surg Pathol 16 1051-1058.
Ngdy nhdn bdi bdo:
Ngdy phdn biin nhqn xet bdt bdo:
Ngdy bdi bdo duqc ddng:
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