Chordoma involving the skin: an immunohistochemicai study of 11 cases Imnitinoliisloc-hc-mical |)ropc-rtic-s were studied in 16 k-sions from 1 1 patients v\ith ehordoma inv-olving the skin. There were iiineme-n and twe) women ranging fre)ni 21 te) 62 years e)ld (mean, 42.6). I he- initial tumor was saeroeoccygeal in 10 casc-s and naso|)haryngc-al in I case-. Three- lesie)iis represented a direct extension fre)m the- primary tume)r te) the skin, and 13 lesie>ns were- e-xamples eif local re-eurreiices in the skin. Iiiuiitniohistochemic-ally, all lc-sic:)ns showed |)ositivity lor keratin, whc-reas 1-1 Ic-sions were |)ositiv-e for v-imentiii and 12 for protein S-l()(). I'^pithc-lial nic-mbraiu- antigc-n was ]:)ositiv-e in fotir instanees and carcinoc-mbryonic antigen was negative in all studic-s. No significant dillc-rc-nce was observed between the iinniuno])rofile ol ease-s of elirc-et exte-nsion and those of loeal recnrreiiees. Overall, the- re-markable- trii)le- ])ositivity for keratin, vimentin, and pre)tein S-lOO was obsc-rved in 11 lesions from eight dillerent cases {7'.V/o). l'his study c-onfirmed the- utility e)f imniune)histe)chetiiistry in the dillerential diagneisis of chordoma from tumors with similar histologic characteristies encountered in the- skin.
Eric J. Gagne, W. P. Daniel Su
Cagiic'- I'J, Su WPD. Chordoma inv-olving the skin: an immtinohistochemieal sttidy of 1 1 cases. J Cutan Patliol 1992: 19: -16947.'"). © Mimk.sgaard 1992.
W. P. Daniel Su. Mayo Clinic. 200 First Street SW. Rochesfer. MN 55905. USA.
Chordomas are rare malignant tumors that were first dc-sc-ribc-d bv' Lu.schka in 1857 (I). They arise Irom rc-mnaiits of the embryonic notochord (2—5), which are normally situated within the vertebral bodies, intervc-rtc-bral disks, or less often within tlie l)re-sac-ral soft tisstic-. This notoehordal origin is supportc-d by the c-x|5eriinc-ntal prodtietic:)n of a chordonia-like neoplasm after punettire of the nueleus |)ul])osus of intervc-rtebral disks (6) and by the rather limitc-d anatomie distribtition e)f chordotiias, which are only found in the axial skeleton (2, 4). In addition, their pattc-rns of dillerentiation are similar to those of the embryologic notochord (7). Most chotdomas atise from the remnants loeated in tlie bonc-s. Thc-y are slow-growing but aggressive tumors, producing local bone destruction and frec^uently extending into the adjacent sejft tissue, including fhe skin (1, 2, 4, 5, 8-10). The pathologic differential diagnosis of chordoma tnay be rather c-xtensive, becau.se all tumors with a component of clear cells or an abundant mueoid matrix must be considered (5, 11). 'Die achievetnent of the eorreet diagnosis has been greatly simplified with the availability of imnnmohistocliemical techAlthough histopathologie and immuno-
Department of Dermatology. Mayo Clinic and Mayo Foundation. Rochester. Minnesota. USA.
Accepfed April 22. 1992
chemieal studies have been done for i:)rimary- chordoma, these studies are rarely reported for cutanee)us lesie)ns. The purpeise e)f this study was to verify- the immuiiohistochciiiical jjrofilc- of known cases cjf chordoma inv-olviug the skin and to com])are the results vvith other tumors that need to be considc-red in the- differential diagiie)sis. Material and methods From 1965 te) 1985, 157 casc-s of clie)rde)ma were reviewed at the Mayo Clinic. The skin was inv-e)lv-e-d in 18 instances (11%). Histe)pathole)gic-material for immunostaining was available- fe)r 16 lesie)ns fre)m 1 1 dillerent patients. Clinical infe)rmatie)n was e)btained fre)ni i:)atients' records and surgical pathe)logy reports, riiree lesic^ns represc-nted direct c-utaucous extension from the primary tumor, and 13 represented local cutaneous recurtenccs. I hese rectirrenees were observed 6 months to 5 years (mean, 2 years) after the initial surgical therapy. Seetions stained v\'ith hematoxy lin and ee)sin were reviewed lor each lesion. Immunohistochemieal studies were performed on sections obtained from
469
Gagne & Su hit;.
I f(.'iiM':)l.
l,ou-pe>we-r
\ lc-\\ e)l p e - r i p l l e - i y o i I t i n i o r a l ne)dnle-s. Neilicee!e-e-e-j)t 1\ e- p u s h i n g
heirele-rs
a n d | ) a l l o r o l I iiiiic i r a l c c - l l s wliie h arc- e-mhc-cldc-d i n innc-mons
lualiix
(llcS:!',. X ()1).
.,3 the- p a r a f r m - c - n i b c - e l d e - e l t i s s u e - b y the- i n e l i r c e t noperoxidasemc-thod. Bric-lly, 4 - ^ . m sc-etions m e ) t i n t e - d e)n s i l a n e - ( l ^ - a n i i n o p r o p y l t r i e t h o x y s i l a n c - ) trc-atc-cl g l a s s slidc-s wc-rc- d r i e d a n d thc-n c:lc-paralliiu/,c-d. N o n - s p c - c i l i e - c - n d o g c - n o i i s i ) c - r o x i d a s c - a c t i v i t y
•
.
A
.^
w a s b l o c k c - d w i l h O.li'Xi h y e l r o g c - n pe-re)\iele- i n r n c - t h ane)l f o r !')() m i n . P r i m a r y a n t i b e i d i c - s le) k e - r a t i n (Al':i/Al'::5, l / 7 5 : H M |1 124161 |), v i m e n t i n ( I / I O : I ) A K ( ) | M 7 2 5 | ) , e-pithelial m e m b r a n e antigen ( 1 / 4 0 : D A K O | M ( i i : 5 | ) , protc-in S-lOO ( 1 / 8 0 0 : f i S C
•/ /';!.'. 2 ((Ja.sr 4). High-povvc-r vic-\v of cytologie details of Uimoral cells. Sc-v-eral largctnultivacuolalc- pliysaliphoroLts c-c-lls ate- vtsthlee&K, X 2.50).
470
Chordoma involving skin Table 1. Chordoma: Immunohistochemicai findings. Case
Tissue examined
1
1st recurrence 2nd recurrence 3rd recurrence Recurrence Recurrence Recurrence Recurrence Recurrence Direct extension Recurrence Direct extension 1st recurrence 2nd recurrence 3rd recurrence 4th recurrence Direct extension
2 3 4 5 6 7 8 9 10
11
Results
Keratin Vimentin S-100 EMA CEA
\i\ l i g h t mic-rosc-opy, c h o r d o m a s m a y a p p e a r d e c e p tivc-ly c - u c - a p s u l a t e d ( I ' i g . 1). I h e t t i m o r a l n o d u l e s vsc-rc- u s u a l l y s i i b d i v - i d e d i n t o se-veral s m a l l e r le)btiles b y d e l i c a t e - e-onncetiv-e- tissue- se-])ta t h a t s u | ) | : ) o r t c d t h e v a s c u l a r s t i p p l y t o the- t u n i e ) r a l c e l l s . 1 h e le)bules e - o n t a i n e d v a r i a b l e - |)rc)|)ortic)iis o f v a e u o l a t e o r n o n v a c - i i o l a t e p o l y g o n a l ce-lls l y i n g s i n g l y o r in s m a l l c l u s t e r s w i t h i n a n a b u n d a n t niue-e)icl m a t r i x . O c c a sie)nal |5hy\saliphe)rotts ( b u b b l y ) e-c-lls c h a r a c - t c - r i s t i e o f c h o r d o m a eeiulel be- i e l e n t i l i e d ( F i g . 2 ) , b u t the-y were- n o t a l w a y s pre-se-nt in all c a s e s .
CEA. carcinoembiyonic antigen; EMA. epithelial membrane antigen.
(S-10()|), a n d j^olyc-lonal c-arcinoembryonic a n t i g e n (l/cSOO: D A K O | A 1 1 5 | ) wc-re usc-d. A sc-c-ondary a n t i b o d y c-c)n|ugatc-d with biotin was then a d d e d , Ibliowc-d by AECl (!5-amino-9-c-thylcarl)a/,olc-) s u b strate-, which was tise-d as a c h r o m o g e n . Histologicslidc-s were- exaniine-d lor |)ositiv-ity, a n d the- iute-nsity of the- reac-tie)n was grade-d from 1+ (v\-eak) to '.] + (strongly jjositive).
Iablc- 1 s u m m a r i / . e - s the- i n i n i u n e ) h i s t o e - | i e - m i c a l re-stilts a e e - o r c h n g t o the- type- ol Ic-sioii a n a l v / . e d : t i s s t i e Iroiii dire-et e-xte-usion o f the- p r i m a r y t u m o r o r f r o m a loc-al rc-ctirre-nc-c- w a s statne-el. , \ l l It) ttime)rs t e s t e d w e r e positive- for kc-ratin ( f i g . '.'>), w h c - r e a s 14 a n d 12 Ic-sions v\e-re- peisitive- l o r v-inu-ntin ( l ' i g . 4 ) a n d j ^ r o te-in S - 1 0 0 (l-"ig. 5 ) , rc-s|)e-ctiv-elv. I n a d d i t i e ) n , f o u r tunie)t-s we-re- pe)sitive- for c-|)ithe-lial m e m b r a n e a n t i ge-n ( I ' i g . t)). N o re-aetiv itv' tei e-are-ine)embrye)nie- a n t i ge-n w a s n o t i c c - d . O v c - r a l l , triple- ])e)sitiv-ity fe)r ke-ra t i i i , v i n i e - n t i n , a n d ])re)te-in S-lt)t) v\ a s e)bse-rv-e-d in
I 1 Ic-sions from eight patients (T.Wo). In general, we did not obsc-rv c- a n y significant chflc-rc-iu-e- in the immimoliistoc-lic-mical l i n d i n g s bc-twc-c-n casc-s of direct extc-nsioii from the priniarv' ttinior a n d eases of local 1 c-c-urrencc.
jpiiS^Ss I m m n n o s t a i t i i t i g lor f.c-iatin
AEl/AE:i Notice- strong c-ytoplastuic marking with cytoplasmic- borclc-i iute-nsirtcatiou (X ItiO).
471
Gagne & Su
I iiminnc i s t a m i n g lor \-iiiic-ntiii. M i l d
|)e-t-iphe-ial
m a r k i n g is j)ie-se-iit ( X Mill).
•
.
^
\
^
-„,
4. J
» - '
Discussion ('hordomas arc- locally aggrc-ssivc- tumors; they produce bone destruction and as a rule extend to thcsurrounding soft tissue. Clinically, pain resulting
•{: '/i^l'
.:5b V .
from bone destruction a n d from pressure- e)n thcnc-rvc-s is the usual s y m | i t o m . Histologieally, c h o r d o nias arc- usually c-om|)osc-cl of several t u m o r a l noeltiles subdivided into smallc-r lobules by dc-licatc- eonneetive tissue t r a b e c u l a e (2, 4, 5 ) . The- lobules eou-
>>^'?-i'.y ' •'^''' r
/•'(.t;. .') fCn.K- tl). I m m u n o s l a i i u ' i i g I'eir prole-iii S-HK). Neitice s t r o n g t n a r k m g ol e-ytopiastnicl)ordc-r (X lt)(l).
472
Chordoma involving skin /•'/l.'. (i (Casr ID). I m m i i i i e ) s l a i i i i n g le)r e-|)illic-hal aillige-n.
ine-iiiliraiicI'l e-se-nee- eil s l l c t n g
pc-riphc-ial and cytoplasmic marking ( X KID).
..-*>
tain vaiiable proi:)orlions of vactiolate e)r n o n - v a c - t i o l a t e p o l y g o n a l eells l y i n g w i t h i n a n a b u n d a n t homogc-nc-otis iniic-oid m a t r i x (2, I, 6 ) . Oc-casieinal p h y s a l i p h o r o i i s cells c a n be- idc-ntilieel, a l the)ugli t h e y m a y be- a b s e n t in se)nie c a s e s ( 1 2 , 1!)). O u r in)nitmohiste)e-lie-mie-al r e s u l l s a r e e-onsisteiit w i t h e)ther i m m u n o h i s t o c h e m i c a i o b s e r v a t i o n s r e |:)orted in t h e l i t e r a t t i r e for p r i m a r y chorele)mas ( 3 , K5—25). In e)ur sttiely, all t t t m o r s w e r e pe)sitiv-e fe)ikc-i-atin, wherc-as rc-ac-tivity le)r v i m e n t i n a n d p r o t e i n S-lOO w a s observe-d i n ' 1 4 a n d 12 l e s i o n s , rc-spee-tivc-ly. In a d d i t i o n , four t t i m o r s were- peisitive fe)r e|)itlielial m e m b r a n e a n t i g e n . O v e r a l l , t h e i n t e r e s t i n g triple- pe)sitivity for k e r a t i n , v i m e n t i n , a n d p r o tc-in S-l()() w a s prc-sent in 11 Ic-sions from e i g h t i)at i e n l s [I'.VYo). T h i s i m m u n o s t a i n i n g | ) r o n i e s u i ) | ) o r t s
Table 2. Histologic differential diagnosis of cfiordoma. Primary fumors From fhe skin Mixed fumor of fhe skin (chondroid syringoma) Melanocytic lesions with a balloon cell component From the soft tissue Myxopapillary ependymoma Skeletal and extraskeletal myxoid chondrosarcoma Clear cell sarcoma of fendons and aponeuroses Myxoid liposarcoma Chondroma Metasfatic fumors Clear cell adenocarcinoma MucinotJS adenocarcinoma Signet-ring cell carcinoma
the- d i a g n o s i s of e-lie)rde)nia. Ne) |:)ositiv-ity feir c a r c i noc-mbryonie- a n t i g e n w a s ne)ticed. l',xce-pt for oiic rc-|)c)rt (1) t h a t s h o w e d rc-ac-tivitv for c a r c - i n o c m bryonic- a n t i g e n in all e h o r d o m a s s t u d i e d , it se-e-ms t h a t eai-citie)cmbrye)tiic a n t i g e n is e-ither r a r e l y ex|)rcsse-el ( I I , K'i) o r cousisle-ntly ne-gativ-e- (!), 1:5. 1(), 20). 1 he- dillere-ntial diagiie)sis eif e - h o r d o m a i n c l u d e s all t u m o r s t h a t h a v e , h i s t o l o g i c a l l y . a c-lc-ar cc-ll c o m p o n e n t o r a n a b u n d a n t myxe)id m a t r i x ( 5 , 11). Tab l e 2 s u m m a r i z e s the- tume)i-s t h a t mtist be- e-e)nsidere-el in the- histe)le)gie- difle-re-ntial d i a g n o s i s of c-liordoiiia. Myxo]:(apillary e p e n d y m o m a a n d m v x o i d v a r i a n t of c - h o n d r o s a r c o m a (1) llrst have- te) b e e o n sidere-el, neit o n l y because- of tlic-ir h i s t o l o g i c s i m i l a r i t y b u t a l s o bec-attse of t h e i r sjjecifu- i-)ro|)c-nsity to arise- in t h e s a c r a l a r e a , a s deies c h o r d e ) m a . M y x e ) p a p i l l a r y e p e n d y m e ) m a s e)e-e-iir ahiie)st c-xc h i s i v e l y in the- r e g i o n of the- c o n n s n i e d u l l a r i s anel liluni te-rminale-, altlie)ngh the-y e-an inv-olvc the- soli t i s s u e s of t h e b u t t o c k (2t)). T h e i r d i s t i i u - t i v e h i s t o l o g i c Ic-attircs c o n s i s t of the- presenee- of pa|:)illae l i n e d by o n e o r sevc-ral lav e r s of c u b o i d a l cc-lls a n d s t i r r o u n d c - d bv a n a b u n d a n t m u c i n e ) u s stre)ma. Thefusie-)n e)f t h e i ) a | i i l l a e r e s n l t s in a se)lid o r r e t i c t i l a t e d a p p e a r a n c e t h a t c a n be- e a s i l y c-onfiise-d w i t h a e-he)rd o m a . l m m i i n e ) h i s t e ) e l u - m i c a l l y , t h e t u m o r ee-lls s h o w s t r o n g rc-activity for glial fibrillarv ac-idic p r o tein anel arc- often jiositivc- for p r o t e i n S-U)O ( 2 7 ) , b u t k e r a t i n is n o t e-xpre\sse-d ( 1 9 ) . .-\lthotigh s o m e e a s e s of c h o r d o m a h a v e b e e n r e p o r t e d t o s h o w a
473
Gagne & Su rc-activity for glial fibrillary a c i d i c |3rotein ( 2 8 ) , t h e c eic-xprc-ssion of k e r a t i n rc-mains helpful t o d i s c r i m i n a t e bc-iwc-c-n the- twcj c-ntities. I'Lxtraskc-lc-tal myxoid chcjiidrosarcomas, also ealled chordoiel s a r c o m a s , o c c u r m a i n l y in t h e soft tissties of the- e-xtremities ( 2 9 ) . Histe)le)gically, t h e y she)w s t i a t i d s a n d r o w s of s m a l l c u b o i d a l c-ells, w i t h oc-casioually v a c u o l a t c d a c i d o j j h i l i c cyto]:)lasm, c-nibc-ddc-cl in a n a b u n d a n t m y x o i d m a t r i x ( 2 9 ) . l)c-s|)ite thc-ir m o r p h o l o g i c r e s e m b l a n c e to c h o r d o m a , m y x oid c h o n d r o s a r c - o m a s a r c i m m u n o h i s t o c h c m i c a l l y rc-activc- for vime-iuiii anel p r o t e i n S-100 ( 2 2 , 3t)), b u t n e g a t i v e for kc-ratin ( 1 4 , 15, 19, 2 4 , 2 5 ) . L e s s often, a m y x o i d c-liondre)sare-e)ma will o e e u r in b o n e ; t h e hisloleigie- as|)c-e-t anel inimuiie)histcjelieniical profile arc-, liowc-vc-r, s i m i l a r to t h e extraskelc-tal form ( 2 5 ) . , \ mixc-d t u m o r of the-skin (c-liondroid s y r i n g o m a ) e a n be- c-onlusc-d w i t h c - h o r d o m a ( I I ) . Ihc- e h a r a c t e r i s t i c histologie- Icaturc-s c o n s i s t of n u m e r o u s s m a l l e l u s t e r s of c-pifhelial cells seafterecl t h r o t i g h a m y x oid o r cliondroiel s t r o m a . Se-ve-ral gre)U|:is e)l cells she)vv a ele-linite- eliietal elille-rentiation, g i v i n g t h e t t i n i o r a m i c r o t t i b i i l a r p a t t e r n t h a t is n o t o b s e r v e d in ehordoma. Immttnohisloc-hc-mic-ally, ihc- diic-tal c-pithc-lial ecimponc-ut is positive- lcii- kc-ratin, e-|)ithe-hal nic-mbrane- antige-n, anel careinoc-nibryonieantige-n, v\he-re-as the- myoe-pilhc-lial c-oni|)onc-iit is |:)ositive lor kc-ratin a n d protc-in S-lt)O (!il). CIc-ar cc-ll sarc-omas of Ic-ndons a n d a p o n e u r o s e s c a n rc-sembic- a c h o r c l o m a Instologie-ally, althejtigh e-liiiieally the-y arise- in the- c-xl rc-mitie-s ol y o u n g j^atie-nts. These t u n i o i s are- eeiniposed ol laseicles a n d solid nc-sts o f fiisiform a n d c u b o i d a l cc-lls w i t h c l e a r c y t o p l a s m a n d nuclc-i w i t h large- nuele-oli {'.V2). l m m t m o h i s t o c l i c n i i c - a l l y , the- (uine)r cells a r e p o s i t i v e for vinie-iitin anel prote-in S-l()() {'.')'.'i) a n d n e g a t i v e for the- c-|)ithc-lial markc-rs. Mc-lanoc-ytic- Ic-sions, c-spc-cially w h e n a c o m p o nc-nt of b a l l o o n cc-lls is prc-sc-nt, a l s o h a v e t o b e rulc-c;l o u t . l u thc-se c a s e s , the- iiniiiuiiohistoc-hc-uiical | ) r o lilc- s h o w s a r e a c t i v i t \ ' Ibr vime-ntin a n d |)rotc-in S-lt)O, a n d t h e r e s u l t s for c-pithc-lial markc-rs a i u l c-arc-inoc-nibryonie a n t i g e n will be- n e g a t i v e . ( n i o r d o m a s m a y simulate- the- histeileigic a s p e c t of a m e t a s t a t i c a d e n o e a r c i n o m a to t h e s k i n . T h e n i i c-rosc-o|)ic- cliflc-rc-ntial e h a g n o s i s iuc-lttde-s cle-ar cell a d e n e ) c a r c i n o m a s , me)stly of r e n a l o r i g i n , a n d s i g n e l r i n g eell o r m u c i n o u s a d e n o c a r c - i n o n i a s , m o s t l y
ttnless
arc- e-spe-eially inisle-acling wlie-ii the- tumoral
elusfers an- surrounded by lakes c:>f muciti. However, lmniunolustoc-hc-inieal studies will clc-moustrate- pos-
is e)l r e n a l
e)rigiii.
m
is the rule (!15). Preitein S-lflf) is ustially not e-xpressed in ade-ne)carcinomas (20) as opposc-d to c h o r d o m a s , a l t h o u g h occ-asional re]:)orts ol a positivereaction c a n be fotind ('.U')).
Conclusion ' T h i s s t t i d y c o n l i r m s the- u t i l i t y o f i i i m i u n o h i s l o e l i c - mistry from
in
t h e dilTc-rential
tumors
with
similar
e n e e ) u i i t e r e d in t h e s k i n .
diagnosis histologic-
of
chorcloma
charactc-risties
The- i n i m i m e ) h i s t e > e - h e - n i i c - a l
|)rofilc- o f t r i p l e - i ) o s i t i v i t y l o r k c - r a t i u , v i m e - i i t i i i , a n d |3re:)teiti S - 1 0 0 w a s fe)uiiel |Datients rather
(73%).
'This
in
II
le-sions
iinmtinostaining
c h a r a c t e r i s t i c - a n d is h c - l p l i i l
from
c-ight
profile-
is
t o s u p p o r t thc-
d i a g n o s i s o l c - | i e ) r d o m a , c-vc-ii m the- s k i n .
References 1. H o u r o p o u l o u
\ \ He)sse- .A, keie-ssiie-i
.\, et .il. I n i i i m i i o h i s -
te)chetiiical investigatiott ol ehoteleimas: histogeuc-lic- a n d d i l f c - r e n t i a l d i a g n o s l i e - a s p c - c t s . ( I n r t - T o p l ' a t h o l li)!)l): 8 ( 1 : l H ; i . 2.
Dahliti DC!, U n n i
K K . Bone- t u m o r s : gc-ncral a s p e c t s a n d
d a t a o t i H,.512 c a s e s . S p r i n g l i e l d , 1 1 , : C C : ' I ' h o m a s , I ' l l l d : ;i7(l. 3.
Maccloiialel ehordosis
K I , , De-ck J I I N .
physalipheira
lmniuiie)liisteic hc-mistry ol'e-c-
anel cliordoiii.i. C a n J Nc-nrol S c i
1090: 17: 120. 4 . S c l u - i t h a t t c - t - B V V . P a t h o l o g y o f t h e - p i l n i t a r y aticl sc-llat- tc-g i o n . I n : . S o m m c - i s . S C , K o s e - n I ' l ' , l - ' e - e h n c - r R l l , c-cls. P a t h o l o gy a n n u a l
198.''). V o l . 2 0 , p a t t
I . N o r w a l k . ('.'!': . \ p p l c - | e i n -
C i e u t u r y - C i r o l ' t s , l!)f{.^): 1 0 1 . .•). S u n d a r e . s a u N . C h o r d o m a s . C l i n O i l h o p
l ' ) H ( , : 2 0 1 : Ki.").
(). F r i e d m a n t i I , H a r r i s o t i I ) I ' ' N , B i r d T S . T h e - l i n e - s t t t i c t t t r c - c i f e h o r d o m a w i t h p a r t i c u l a r rc-ie-ic-uc-c- l o t h e - p h \ s a l i p h o r o n s c e l l . , I C l i n I ' a l h o l l ' . l ( ) 2 : I.''): I K ) . 7. Hc-a1eni ] M , I n r n c - r I ) R . K c - n e - c l i o n s e m u e ) i e ) e - h o r c l a l d i l l c - i c - n t i a l i o n a r i s i n g frotii a s t u d y ol c h o r e l e n i i a s . 1 l i s t o p a t l i o l o g y 1985:
9: .543.
8 . A t i e l I M , V c - r d i i (.'•. C h o r d o m a : a n a n a l y s i s e)l t w e - n t y c a s c - s t t c - a t e d o v c - r a t v v e i i t y - y c - a t s p a n . , ] S n r g O i i c e i l l')7.''): 7 : 2 7 . 9 . CJray SVV, . S i u g h a b h a n d h u
B,Smith
R,A, S k . i n d a l a k i s J1',.
S a e t - o c o c e y g e a l e h o t - d o t u a : r e - p o r t o l a c a s e - a n d ie-\ie-\\ eil ihel i t e r a t u r e . S u r g e r y l!)7.'): 7 8 : .''>7:i. 10.
l l i g i n b o t h a t n N L , P h i l l i p s R K , l'ari IIVV, l l i t s l i i I K ) . C h o r d o m a : thirty-rive--yc-ai s t u d y a l M e i u o r i a l lle>spil.il. C a u c e - r
1967: 20: 1841. I I . ( lhaml)c-rs I ' W , .Schwinn ( ' P. (! horde nii.i: a c-limc-oj)alhologic-
study of metastasis. Am.) Clin Patliol 197'.): 72; 7l).''i.
c-oming from the rectum. Mucinotis adenocar(-inomas
t h e aclene)eareine)iiia
w h i c h e-ase- t h e e e ) e . \ ] i r e - s s i o i i o f k c - r a t i n a n d v - i n i e - n l n i
12.
K l l i o l t EC;, M c K i n n c - y S , B a n k s I I , l-'iilks R M . . A s p i r a t i o n c y t o l o g y c)l t D c t a s t a t l e - c l i o r d c u n a : a e-asc- rc-port. /Xcta C y t o l
I 9 8 : i : 2 7 : (K")!!. K5. Pc-rasole .'\, t u l a u t o l i u o I ) , S p i g a r i o l 1'. A s p i r a t i o n c y t o l o g y
ili\'ily lor fhe (-pithelial m a r k e r s keratin a n d vpi-
a n d i m i n u i i o c - y l o c h c - m i s U - y of .sacraf c-lioreloma w i l h
theltal nie-nibrane" antige-n, in asse)eialie)n with a pos-
mc-casCases-. a c a s e rc-port. tOiagti C y t o p a t h o l 1 9 9 1 : 7 : 2 7 7 .
ifivify lor c:arcinoembryonic anfigen (Hi, 20, 34). The
474
expre-ssie)n
e)f v i m e n t i n
is u s u a l l y
tiot
f'otmel
lt\-c-r
14. A b c - i i o z a P, Siblc-y R K . ( i h o r d o n i a : a n i i n i n i i n o l i i s l o l o g i c s t u d y . H u m P a t h o l I98(i: 17: 7 4 4 .
Chordoma involving skin I.V Coindrc- ] - M . R i v e l j , T r c ) j a n i M , l)e Ma.scarel I, De Ma.sca-
Ki.
2().
casc-s. A u i J C l i u P a t h o l
llarreiwe- 1)|. l'ayle)r CR. Immuueipe-roxidasc- stainitig I'ot,iiiligc-ii
m ceileinie- c a t c - m o n i a .
27.
ostc-osat--
2eS.
()iu-ol
Me-is I M . ( i i r a l d e ) . \ . \ . ( ' h e i i c i o m a : a n i i i i i i i n i i e ) l i i s l e ) c - h c - n i -
M i e - l t i i i c - i i . \ 1 , l.c-hio \ ' - P . D a h l D , X ' i r t a n c - t i 1. D i l l ' e t e n t i a l
aidc-el b y anti-inte-rnic-eltate- h l a n i c - i U a n t i b o d i c - s . . \ m
29.
mvxe)-
stucK
o l .V2
Onol'rio BNt. M y x o p a p i l -
.1 clinic-opatheilogic-
atid
imtiiunocyto-
198'): .')(): 8 8 ; ! .
S. P o s i t i v i t y
to glial
tti b o n e , cartilage-, a n d e-he)rde)ma.
libiillaiA |
Siirg
1989: I I : 2 2 .
l-',nziiigc-r F M . S h i i a k i M . l-',xttaske-le-t.il n i \ xe)id c h e i n d r o s a r c o i n a : a n a n a K s i s ol S I cases. H c i m P . i t h o l 1 9 7 2 : .r. 1 2 1 .
:>().
I ' l e t c h e - r C D . M . Pouc-11 C , .McKc-c- P H . K x t i a s k c l e t a l c h o u e l r o s a r c - o m . i : a histochc-mie-.d
|
I 9 « : f : I 12: 11)0.
i c a l stctdv. 1 l i s t o p a t l i o l o g y 111.
and
mvxoid
immunohistoche-tii-
I98(): 10: 189.
P a l t n e r R M , L u c a s R B , K i i i g h t J . C l u s t e t s o t i B. l m m n n c ) c - \ -
M i c - t t i n e i i . \ l . Che)rcle)m.i: a n t i b o d i e s te) c - p i t h c l i a l t u c - t n b r a n e
t o c h e t i i i e a l i d c - n t i l i c a t i o n eiT cc-ll typc-s iti p l e o t i i o r p h i c - a d e -
a n t i g c - n a n d c at c i n o e - n i b i yeinic a u t i g c - n i n elille-re-titial e l i a g -
noma.
ue>sis. . \ r c - l i P a t h o l l . a h Mc-cl 1 9 8 1 : 108: 8 9 1 .
P a t h o l 198."): I K i : 2 1 3 .
Nakajinia
1 . \ \ a l a i i a h c - S. Sate) N', K a m e - y a
Shiiiie)sate) ^ . :\\i distribution
I', l l i r o t a
T,
32.
immuneipc-ie)\tc:lase- s t u d y o l S - 1 0 0 p r o t e - i n
i n n o r m a l a n d iie-o|)lastie- tissnc-s. . \ m | S t t t g
x\ith
p.irlicnl.ir
rerc-ic-ncc- to t n y o e p i t h e l i a l
cells. J
K c k a r d t j j , P r i t c h a t d D J . S o n i c K I L C l e a r cc-ll saic-onia: a e l i n i e - o p a t h o l o g i c s t t i d y 0 1 2 7 case-s. Cance-r 1 9 8 3 : ~)'2: 1 182.
33.
Pathe)! 1 9 8 2 : I i : 71.'^).
K i n d b l o m L - ( ; , L o d d i i i g P, /Xiigc-rvall L . CUc-ar-ec-ll s a r c o m a e)l te-nde)tis a n d a p o t i e u r o s e s : a n t m m u u o h i s t o c - l i c - m i c a l a n d
( ) k a | i i n a K . llemcla I, K i t a g a w a
I'. I m n i u n o h i s t o c h c - m i c - a l
e-le-c-|re)n miereisc-opic a n a l y s i s i n d i c - a t i t i g tic-nral crc-st o r i g i t i .
elisli i b i i t i e i n o f . S - l t l O p r o t e - i n i n t u m o r s ,tncl t u m o r - l i k e - le-sieiiis o T b o i i e - anel e-artil,ige-. (!ane-e-r 19e'!8: ( i l : 7 9 2 .
21.
c-hmc-opathe)le)gic
1984: 8 1 : !.')().
\ ' . Sluiaugshoti
h m i i . i n c i i o r e l e i m . i . ) P a l l i e i l I ! I 9 1 : 1 (i 1: II.'').
Palhol
2!i.
K.isamikctl
acidic- |)re)tc-tti
d i a g n o s i s eil c - h o r d o i n a . c h o n d r c i i c l . a n d c - p c - n d y i n a l t u m o r s
22.
e-pe-udymoma:
\ ' i r l a n e - i i I. l',.\pie-ssie>ii eil elillere-nt eyteikc-tat i n subclassc-s i n
as
a
Se)ime-land P R l , . Schc-ithanc-r B\\. lary
ic-al s i n e K e,l 2 0 ca.sc-s. A r c h P a t h o l L a b M c - d 1 9 8 8 : 1 12: ^t'V.'..
21.
e-pe-nelymonia:
ehe-tnical s t t t d y o l ' 7 7 casc-s. C.iticc-r
17. 1 Ic-ikiuhc-imo K, Pc-t-sson S, Kindblom I,-C, Morg.iii PR.
20.
| B . . S u b c n l a n c - o t i s s a e i e)e-e)e-c-\ g c - a l
I98(i: l.'')ll: d l .
e-oma, and ehejidoma. j Sitrg Otie-ol 1981: Ki: 1.
19.
l . B . Ste-rn
paptllary
c a l cm e )e-nil n yeiiiic
18.
Ile-lwig
le-i . \ . 1 nmmne)luste)le)gic-al stnci) in choi-domas. J l'athol
Rtithc-rroord ( i S , l)a\ic-s
\ ' i r c - h o \ \ s .\re-h | . \ | 1983: 1 0 1 : 10!). 31.
. \ ( ; . (ihordonia.s—ulttastruelnre
Quenlme-ie-t
. \ . Mc')lle-r
P. Sc h w a r / . \ ' . .\be-| I ' . S e h l a g P.
C ' a r e m e ) c - m b r y o n i c - a n t i g e n , C.A 1 9 - 9 . a n d ( ! . \ 12.') i n n o r m a l
aiicl i m m t i n o h i s t o c h c - m i s t t y : a i c - p o r t b a s e d o n t h e e x a t u -
a n d c a t c i t i o t i i a t o c t s l u m i a t i c o l o r e c t a l tisstic-. C a t i c e t - 1987:
i n a l i o n o i ' s i x casc-s. 1 l i s t o p a t h e i l o g y 1 9 8 7 : 1 1 : 77.'').
(iO:
S a l i s b i i i ) J R . Is.iacson P ( ; . D c - m o n s l i aticiti o f c y t o k e t a t i t i s
22(il.
3.''). W a l d h c - r r
R. Schwc-chhc-imc-r K . C o - e \ p r c - s s i o u o f cytokct--
aiiel a n e-pilhe-hal m e - n i b r a i i e - a n t i g e - n i n e h e ) i c l o n i a s a n d h u -
a t i n . i i u l \ i m c - n l i n mu-rinc-diatc--si/.c-d l i l a n u - n t s i n ic-iial ce-ll
m . i i i le-tal ue)te)c-horcl. . \ t u J S l u g P a l h o l 198.'): 9 : 7 9 1 .
earciiiomas:
2.'"). W i c l s
M R , Bntgc-ss | H , M a u i \ - c - l
--chordoid
s a t c - o m a ':
| { ' . .\
nllrastruetur.il
r e a s s e s s m c - m eil
and
inimimohislo-
c hc-inic-al e-oiitpariseiii w i l h c - h o i c l o m a a n d skc-lc-tal m y x o i d c - h o t i d r c i s a t c - o t i i a . .Meicl P a l h e i l 1 9 8 8 : I : !:!.!.
c c i m p a r a l i \ c- s t u d y
o f die- iutet-tiu-diatc--si/e-cl
lilatuc-tit d i . s t r i b t i t i o n i t i tc-tial cell c a r c i t i o t i i a s a n d t i o t n i a l h u m a t i kicluc-\. X ' i r c h o u s .Arch | . \ | 198.'): 108: I.''). 3().
l ' a k a s h i M , 1 l a i m o l e ) 11, Mttrasc- I , M i t s t i v ,\ 11, K a t o K . .An ittminne)c-hc-mical a n d i n i i n u n o h i s l o c l u - m i c a l s t u c k ol .S-1 0 0 pie)te-it\ i n r e n a l cc-ll c a i c i u o m a . C . n i c e r
1 9 8 8 : t'l 1: 8 8 9 .
475
Eric J. Gagne, W. P. Daniel Su
Cagiic'- I'J, Su WPD. Chordoma inv-olving the skin: an immtinohistochemieal sttidy of 1 1 cases. J Cutan Patliol 1992: 19: -16947.'"). © Mimk.sgaard 1992.
W. P. Daniel Su. Mayo Clinic. 200 First Street SW. Rochesfer. MN 55905. USA.
Chordomas are rare malignant tumors that were first dc-sc-ribc-d bv' Lu.schka in 1857 (I). They arise Irom rc-mnaiits of the embryonic notochord (2—5), which are normally situated within the vertebral bodies, intervc-rtc-bral disks, or less often within tlie l)re-sac-ral soft tisstic-. This notoehordal origin is supportc-d by the c-x|5eriinc-ntal prodtietic:)n of a chordonia-like neoplasm after punettire of the nueleus |)ul])osus of intervc-rtebral disks (6) and by the rather limitc-d anatomie distribtition e)f chordotiias, which are only found in the axial skeleton (2, 4). In addition, their pattc-rns of dillerentiation are similar to those of the embryologic notochord (7). Most chotdomas atise from the remnants loeated in tlie bonc-s. Thc-y are slow-growing but aggressive tumors, producing local bone destruction and frec^uently extending into the adjacent sejft tissue, including fhe skin (1, 2, 4, 5, 8-10). The pathologic differential diagnosis of chordoma tnay be rather c-xtensive, becau.se all tumors with a component of clear cells or an abundant mueoid matrix must be considered (5, 11). 'Die achievetnent of the eorreet diagnosis has been greatly simplified with the availability of imnnmohistocliemical techAlthough histopathologie and immuno-
Department of Dermatology. Mayo Clinic and Mayo Foundation. Rochester. Minnesota. USA.
Accepfed April 22. 1992
chemieal studies have been done for i:)rimary- chordoma, these studies are rarely reported for cutanee)us lesie)ns. The purpeise e)f this study was to verify- the immuiiohistochciiiical jjrofilc- of known cases cjf chordoma inv-olviug the skin and to com])are the results vvith other tumors that need to be considc-red in the- differential diagiie)sis. Material and methods From 1965 te) 1985, 157 casc-s of clie)rde)ma were reviewed at the Mayo Clinic. The skin was inv-e)lv-e-d in 18 instances (11%). Histe)pathole)gic-material for immunostaining was available- fe)r 16 lesie)ns fre)m 1 1 dillerent patients. Clinical infe)rmatie)n was e)btained fre)ni i:)atients' records and surgical pathe)logy reports, riiree lesic^ns represc-nted direct c-utaucous extension from the primary tumor, and 13 represented local cutaneous recurtenccs. I hese rectirrenees were observed 6 months to 5 years (mean, 2 years) after the initial surgical therapy. Seetions stained v\'ith hematoxy lin and ee)sin were reviewed lor each lesion. Immunohistochemieal studies were performed on sections obtained from
469
Gagne & Su hit;.
I f(.'iiM':)l.
l,ou-pe>we-r
\ lc-\\ e)l p e - r i p l l e - i y o i I t i n i o r a l ne)dnle-s. Neilicee!e-e-e-j)t 1\ e- p u s h i n g
heirele-rs
a n d | ) a l l o r o l I iiiiic i r a l c c - l l s wliie h arc- e-mhc-cldc-d i n innc-mons
lualiix
(llcS:!',. X ()1).
.,3 the- p a r a f r m - c - n i b c - e l d e - e l t i s s u e - b y the- i n e l i r c e t noperoxidasemc-thod. Bric-lly, 4 - ^ . m sc-etions m e ) t i n t e - d e)n s i l a n e - ( l ^ - a n i i n o p r o p y l t r i e t h o x y s i l a n c - ) trc-atc-cl g l a s s slidc-s wc-rc- d r i e d a n d thc-n c:lc-paralliiu/,c-d. N o n - s p c - c i l i e - c - n d o g c - n o i i s i ) c - r o x i d a s c - a c t i v i t y
•
.
A
.^
w a s b l o c k c - d w i l h O.li'Xi h y e l r o g c - n pe-re)\iele- i n r n c - t h ane)l f o r !')() m i n . P r i m a r y a n t i b e i d i c - s le) k e - r a t i n (Al':i/Al'::5, l / 7 5 : H M |1 124161 |), v i m e n t i n ( I / I O : I ) A K ( ) | M 7 2 5 | ) , e-pithelial m e m b r a n e antigen ( 1 / 4 0 : D A K O | M ( i i : 5 | ) , protc-in S-lOO ( 1 / 8 0 0 : f i S C
•/ /';!.'. 2 ((Ja.sr 4). High-povvc-r vic-\v of cytologie details of Uimoral cells. Sc-v-eral largctnultivacuolalc- pliysaliphoroLts c-c-lls ate- vtsthlee&K, X 2.50).
470
Chordoma involving skin Table 1. Chordoma: Immunohistochemicai findings. Case
Tissue examined
1
1st recurrence 2nd recurrence 3rd recurrence Recurrence Recurrence Recurrence Recurrence Recurrence Direct extension Recurrence Direct extension 1st recurrence 2nd recurrence 3rd recurrence 4th recurrence Direct extension
2 3 4 5 6 7 8 9 10
11
Results
Keratin Vimentin S-100 EMA CEA
\i\ l i g h t mic-rosc-opy, c h o r d o m a s m a y a p p e a r d e c e p tivc-ly c - u c - a p s u l a t e d ( I ' i g . 1). I h e t t i m o r a l n o d u l e s vsc-rc- u s u a l l y s i i b d i v - i d e d i n t o se-veral s m a l l e r le)btiles b y d e l i c a t e - e-onncetiv-e- tissue- se-])ta t h a t s u | ) | : ) o r t c d t h e v a s c u l a r s t i p p l y t o the- t u n i e ) r a l c e l l s . 1 h e le)bules e - o n t a i n e d v a r i a b l e - |)rc)|)ortic)iis o f v a e u o l a t e o r n o n v a c - i i o l a t e p o l y g o n a l ce-lls l y i n g s i n g l y o r in s m a l l c l u s t e r s w i t h i n a n a b u n d a n t niue-e)icl m a t r i x . O c c a sie)nal |5hy\saliphe)rotts ( b u b b l y ) e-c-lls c h a r a c - t c - r i s t i e o f c h o r d o m a eeiulel be- i e l e n t i l i e d ( F i g . 2 ) , b u t the-y were- n o t a l w a y s pre-se-nt in all c a s e s .
CEA. carcinoembiyonic antigen; EMA. epithelial membrane antigen.
(S-10()|), a n d j^olyc-lonal c-arcinoembryonic a n t i g e n (l/cSOO: D A K O | A 1 1 5 | ) wc-re usc-d. A sc-c-ondary a n t i b o d y c-c)n|ugatc-d with biotin was then a d d e d , Ibliowc-d by AECl (!5-amino-9-c-thylcarl)a/,olc-) s u b strate-, which was tise-d as a c h r o m o g e n . Histologicslidc-s were- exaniine-d lor |)ositiv-ity, a n d the- iute-nsity of the- reac-tie)n was grade-d from 1+ (v\-eak) to '.] + (strongly jjositive).
Iablc- 1 s u m m a r i / . e - s the- i n i n i u n e ) h i s t o e - | i e - m i c a l re-stilts a e e - o r c h n g t o the- type- ol Ic-sioii a n a l v / . e d : t i s s t i e Iroiii dire-et e-xte-usion o f the- p r i m a r y t u m o r o r f r o m a loc-al rc-ctirre-nc-c- w a s statne-el. , \ l l It) ttime)rs t e s t e d w e r e positive- for kc-ratin ( f i g . '.'>), w h c - r e a s 14 a n d 12 Ic-sions v\e-re- peisitive- l o r v-inu-ntin ( l ' i g . 4 ) a n d j ^ r o te-in S - 1 0 0 (l-"ig. 5 ) , rc-s|)e-ctiv-elv. I n a d d i t i e ) n , f o u r tunie)t-s we-re- pe)sitive- for c-|)ithe-lial m e m b r a n e a n t i ge-n ( I ' i g . t)). N o re-aetiv itv' tei e-are-ine)embrye)nie- a n t i ge-n w a s n o t i c c - d . O v c - r a l l , triple- ])e)sitiv-ity fe)r ke-ra t i i i , v i n i e - n t i n , a n d ])re)te-in S-lt)t) v\ a s e)bse-rv-e-d in
I 1 Ic-sions from eight patients (T.Wo). In general, we did not obsc-rv c- a n y significant chflc-rc-iu-e- in the immimoliistoc-lic-mical l i n d i n g s bc-twc-c-n casc-s of direct extc-nsioii from the priniarv' ttinior a n d eases of local 1 c-c-urrencc.
jpiiS^Ss I m m n n o s t a i t i i t i g lor f.c-iatin
AEl/AE:i Notice- strong c-ytoplastuic marking with cytoplasmic- borclc-i iute-nsirtcatiou (X ItiO).
471
Gagne & Su
I iiminnc i s t a m i n g lor \-iiiic-ntiii. M i l d
|)e-t-iphe-ial
m a r k i n g is j)ie-se-iit ( X Mill).
•
.
^
\
^
-„,
4. J
» - '
Discussion ('hordomas arc- locally aggrc-ssivc- tumors; they produce bone destruction and as a rule extend to thcsurrounding soft tissue. Clinically, pain resulting
•{: '/i^l'
.:5b V .
from bone destruction a n d from pressure- e)n thcnc-rvc-s is the usual s y m | i t o m . Histologieally, c h o r d o nias arc- usually c-om|)osc-cl of several t u m o r a l noeltiles subdivided into smallc-r lobules by dc-licatc- eonneetive tissue t r a b e c u l a e (2, 4, 5 ) . The- lobules eou-
>>^'?-i'.y ' •'^''' r
/•'(.t;. .') fCn.K- tl). I m m u n o s l a i i u ' i i g I'eir prole-iii S-HK). Neitice s t r o n g t n a r k m g ol e-ytopiastnicl)ordc-r (X lt)(l).
472
Chordoma involving skin /•'/l.'. (i (Casr ID). I m m i i i i e ) s l a i i i i n g le)r e-|)illic-hal aillige-n.
ine-iiiliraiicI'l e-se-nee- eil s l l c t n g
pc-riphc-ial and cytoplasmic marking ( X KID).
..-*>
tain vaiiable proi:)orlions of vactiolate e)r n o n - v a c - t i o l a t e p o l y g o n a l eells l y i n g w i t h i n a n a b u n d a n t homogc-nc-otis iniic-oid m a t r i x (2, I, 6 ) . Oc-casieinal p h y s a l i p h o r o i i s cells c a n be- idc-ntilieel, a l the)ugli t h e y m a y be- a b s e n t in se)nie c a s e s ( 1 2 , 1!)). O u r in)nitmohiste)e-lie-mie-al r e s u l l s a r e e-onsisteiit w i t h e)ther i m m u n o h i s t o c h e m i c a i o b s e r v a t i o n s r e |:)orted in t h e l i t e r a t t i r e for p r i m a r y chorele)mas ( 3 , K5—25). In e)ur sttiely, all t t t m o r s w e r e pe)sitiv-e fe)ikc-i-atin, wherc-as rc-ac-tivity le)r v i m e n t i n a n d p r o t e i n S-lOO w a s observe-d i n ' 1 4 a n d 12 l e s i o n s , rc-spee-tivc-ly. In a d d i t i o n , four t t i m o r s were- peisitive fe)r e|)itlielial m e m b r a n e a n t i g e n . O v e r a l l , t h e i n t e r e s t i n g triple- pe)sitivity for k e r a t i n , v i m e n t i n , a n d p r o tc-in S-l()() w a s prc-sent in 11 Ic-sions from e i g h t i)at i e n l s [I'.VYo). T h i s i m m u n o s t a i n i n g | ) r o n i e s u i ) | ) o r t s
Table 2. Histologic differential diagnosis of cfiordoma. Primary fumors From fhe skin Mixed fumor of fhe skin (chondroid syringoma) Melanocytic lesions with a balloon cell component From the soft tissue Myxopapillary ependymoma Skeletal and extraskeletal myxoid chondrosarcoma Clear cell sarcoma of fendons and aponeuroses Myxoid liposarcoma Chondroma Metasfatic fumors Clear cell adenocarcinoma MucinotJS adenocarcinoma Signet-ring cell carcinoma
the- d i a g n o s i s of e-lie)rde)nia. Ne) |:)ositiv-ity feir c a r c i noc-mbryonie- a n t i g e n w a s ne)ticed. l',xce-pt for oiic rc-|)c)rt (1) t h a t s h o w e d rc-ac-tivitv for c a r c - i n o c m bryonic- a n t i g e n in all e h o r d o m a s s t u d i e d , it se-e-ms t h a t eai-citie)cmbrye)tiic a n t i g e n is e-ither r a r e l y ex|)rcsse-el ( I I , K'i) o r cousisle-ntly ne-gativ-e- (!), 1:5. 1(), 20). 1 he- dillere-ntial diagiie)sis eif e - h o r d o m a i n c l u d e s all t u m o r s t h a t h a v e , h i s t o l o g i c a l l y . a c-lc-ar cc-ll c o m p o n e n t o r a n a b u n d a n t myxe)id m a t r i x ( 5 , 11). Tab l e 2 s u m m a r i z e s the- tume)i-s t h a t mtist be- e-e)nsidere-el in the- histe)le)gie- difle-re-ntial d i a g n o s i s of c-liordoiiia. Myxo]:(apillary e p e n d y m o m a a n d m v x o i d v a r i a n t of c - h o n d r o s a r c o m a (1) llrst have- te) b e e o n sidere-el, neit o n l y because- of tlic-ir h i s t o l o g i c s i m i l a r i t y b u t a l s o bec-attse of t h e i r sjjecifu- i-)ro|)c-nsity to arise- in t h e s a c r a l a r e a , a s deies c h o r d e ) m a . M y x e ) p a p i l l a r y e p e n d y m e ) m a s e)e-e-iir ahiie)st c-xc h i s i v e l y in the- r e g i o n of the- c o n n s n i e d u l l a r i s anel liluni te-rminale-, altlie)ngh the-y e-an inv-olvc the- soli t i s s u e s of t h e b u t t o c k (2t)). T h e i r d i s t i i u - t i v e h i s t o l o g i c Ic-attircs c o n s i s t of the- presenee- of pa|:)illae l i n e d by o n e o r sevc-ral lav e r s of c u b o i d a l cc-lls a n d s t i r r o u n d c - d bv a n a b u n d a n t m u c i n e ) u s stre)ma. Thefusie-)n e)f t h e i ) a | i i l l a e r e s n l t s in a se)lid o r r e t i c t i l a t e d a p p e a r a n c e t h a t c a n be- e a s i l y c-onfiise-d w i t h a e-he)rd o m a . l m m i i n e ) h i s t e ) e l u - m i c a l l y , t h e t u m o r ee-lls s h o w s t r o n g rc-activity for glial fibrillarv ac-idic p r o tein anel arc- often jiositivc- for p r o t e i n S-U)O ( 2 7 ) , b u t k e r a t i n is n o t e-xpre\sse-d ( 1 9 ) . .-\lthotigh s o m e e a s e s of c h o r d o m a h a v e b e e n r e p o r t e d t o s h o w a
473
Gagne & Su rc-activity for glial fibrillary a c i d i c |3rotein ( 2 8 ) , t h e c eic-xprc-ssion of k e r a t i n rc-mains helpful t o d i s c r i m i n a t e bc-iwc-c-n the- twcj c-ntities. I'Lxtraskc-lc-tal myxoid chcjiidrosarcomas, also ealled chordoiel s a r c o m a s , o c c u r m a i n l y in t h e soft tissties of the- e-xtremities ( 2 9 ) . Histe)le)gically, t h e y she)w s t i a t i d s a n d r o w s of s m a l l c u b o i d a l c-ells, w i t h oc-casioually v a c u o l a t c d a c i d o j j h i l i c cyto]:)lasm, c-nibc-ddc-cl in a n a b u n d a n t m y x o i d m a t r i x ( 2 9 ) . l)c-s|)ite thc-ir m o r p h o l o g i c r e s e m b l a n c e to c h o r d o m a , m y x oid c h o n d r o s a r c - o m a s a r c i m m u n o h i s t o c h c m i c a l l y rc-activc- for vime-iuiii anel p r o t e i n S-100 ( 2 2 , 3t)), b u t n e g a t i v e for kc-ratin ( 1 4 , 15, 19, 2 4 , 2 5 ) . L e s s often, a m y x o i d c-liondre)sare-e)ma will o e e u r in b o n e ; t h e hisloleigie- as|)c-e-t anel inimuiie)histcjelieniical profile arc-, liowc-vc-r, s i m i l a r to t h e extraskelc-tal form ( 2 5 ) . , \ mixc-d t u m o r of the-skin (c-liondroid s y r i n g o m a ) e a n be- c-onlusc-d w i t h c - h o r d o m a ( I I ) . Ihc- e h a r a c t e r i s t i c histologie- Icaturc-s c o n s i s t of n u m e r o u s s m a l l e l u s t e r s of c-pifhelial cells seafterecl t h r o t i g h a m y x oid o r cliondroiel s t r o m a . Se-ve-ral gre)U|:is e)l cells she)vv a ele-linite- eliietal elille-rentiation, g i v i n g t h e t t i n i o r a m i c r o t t i b i i l a r p a t t e r n t h a t is n o t o b s e r v e d in ehordoma. Immttnohisloc-hc-mic-ally, ihc- diic-tal c-pithc-lial ecimponc-ut is positive- lcii- kc-ratin, e-|)ithe-hal nic-mbrane- antige-n, anel careinoc-nibryonieantige-n, v\he-re-as the- myoe-pilhc-lial c-oni|)onc-iit is |:)ositive lor kc-ratin a n d protc-in S-lt)O (!il). CIc-ar cc-ll sarc-omas of Ic-ndons a n d a p o n e u r o s e s c a n rc-sembic- a c h o r c l o m a Instologie-ally, althejtigh e-liiiieally the-y arise- in the- c-xl rc-mitie-s ol y o u n g j^atie-nts. These t u n i o i s are- eeiniposed ol laseicles a n d solid nc-sts o f fiisiform a n d c u b o i d a l cc-lls w i t h c l e a r c y t o p l a s m a n d nuclc-i w i t h large- nuele-oli {'.V2). l m m t m o h i s t o c l i c n i i c - a l l y , the- (uine)r cells a r e p o s i t i v e for vinie-iitin anel prote-in S-l()() {'.')'.'i) a n d n e g a t i v e for the- c-|)ithc-lial markc-rs. Mc-lanoc-ytic- Ic-sions, c-spc-cially w h e n a c o m p o nc-nt of b a l l o o n cc-lls is prc-sc-nt, a l s o h a v e t o b e rulc-c;l o u t . l u thc-se c a s e s , the- iiniiiuiiohistoc-hc-uiical | ) r o lilc- s h o w s a r e a c t i v i t \ ' Ibr vime-ntin a n d |)rotc-in S-lt)O, a n d t h e r e s u l t s for c-pithc-lial markc-rs a i u l c-arc-inoc-nibryonie a n t i g e n will be- n e g a t i v e . ( n i o r d o m a s m a y simulate- the- histeileigic a s p e c t of a m e t a s t a t i c a d e n o e a r c i n o m a to t h e s k i n . T h e n i i c-rosc-o|)ic- cliflc-rc-ntial e h a g n o s i s iuc-lttde-s cle-ar cell a d e n e ) c a r c i n o m a s , me)stly of r e n a l o r i g i n , a n d s i g n e l r i n g eell o r m u c i n o u s a d e n o c a r c - i n o n i a s , m o s t l y
ttnless
arc- e-spe-eially inisle-acling wlie-ii the- tumoral
elusfers an- surrounded by lakes c:>f muciti. However, lmniunolustoc-hc-inieal studies will clc-moustrate- pos-
is e)l r e n a l
e)rigiii.
m
is the rule (!15). Preitein S-lflf) is ustially not e-xpressed in ade-ne)carcinomas (20) as opposc-d to c h o r d o m a s , a l t h o u g h occ-asional re]:)orts ol a positivereaction c a n be fotind ('.U')).
Conclusion ' T h i s s t t i d y c o n l i r m s the- u t i l i t y o f i i i m i u n o h i s l o e l i c - mistry from
in
t h e dilTc-rential
tumors
with
similar
e n e e ) u i i t e r e d in t h e s k i n .
diagnosis histologic-
of
chorcloma
charactc-risties
The- i n i m i m e ) h i s t e > e - h e - n i i c - a l
|)rofilc- o f t r i p l e - i ) o s i t i v i t y l o r k c - r a t i u , v i m e - i i t i i i , a n d |3re:)teiti S - 1 0 0 w a s fe)uiiel |Datients rather
(73%).
'This
in
II
le-sions
iinmtinostaining
c h a r a c t e r i s t i c - a n d is h c - l p l i i l
from
c-ight
profile-
is
t o s u p p o r t thc-
d i a g n o s i s o l c - | i e ) r d o m a , c-vc-ii m the- s k i n .
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aiicl i m m t i n o h i s t o c h c - m i s t t y : a i c - p o r t b a s e d o n t h e e x a t u -
a n d c a t c i t i o t i i a t o c t s l u m i a t i c o l o r e c t a l tisstic-. C a t i c e t - 1987:
i n a l i o n o i ' s i x casc-s. 1 l i s t o p a t h e i l o g y 1 9 8 7 : 1 1 : 77.'').
(iO:
S a l i s b i i i ) J R . Is.iacson P ( ; . D c - m o n s l i aticiti o f c y t o k e t a t i t i s
22(il.
3.''). W a l d h c - r r
R. Schwc-chhc-imc-r K . C o - e \ p r c - s s i o u o f cytokct--
aiiel a n e-pilhe-hal m e - n i b r a i i e - a n t i g e - n i n e h e ) i c l o n i a s a n d h u -
a t i n . i i u l \ i m c - n l i n mu-rinc-diatc--si/.c-d l i l a n u - n t s i n ic-iial ce-ll
m . i i i le-tal ue)te)c-horcl. . \ t u J S l u g P a l h o l 198.'): 9 : 7 9 1 .
earciiiomas:
2.'"). W i c l s
M R , Bntgc-ss | H , M a u i \ - c - l
--chordoid
s a t c - o m a ':
| { ' . .\
nllrastruetur.il
r e a s s e s s m c - m eil
and
inimimohislo-
c hc-inic-al e-oiitpariseiii w i l h c - h o i c l o m a a n d skc-lc-tal m y x o i d c - h o t i d r c i s a t c - o t i i a . .Meicl P a l h e i l 1 9 8 8 : I : !:!.!.
c c i m p a r a l i \ c- s t u d y
o f die- iutet-tiu-diatc--si/e-cl
lilatuc-tit d i . s t r i b t i t i o n i t i tc-tial cell c a r c i t i o t i i a s a n d t i o t n i a l h u m a t i kicluc-\. X ' i r c h o u s .Arch | . \ | 198.'): 108: I.''). 3().
l ' a k a s h i M , 1 l a i m o l e ) 11, Mttrasc- I , M i t s t i v ,\ 11, K a t o K . .An ittminne)c-hc-mical a n d i n i i n u n o h i s l o c l u - m i c a l s t u c k ol .S-1 0 0 pie)te-it\ i n r e n a l cc-ll c a i c i u o m a . C . n i c e r
1 9 8 8 : t'l 1: 8 8 9 .
475
