signet ring cell carcinoma of the colon radiologically...

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CASE RECORD Signet ring cell carcinoma of the colon radiologically simulating ileocecal tuberculosis WC P ETER KW1\N, MD, FRCPC, HJ FR EEMAN, MD, FRCPC, FAC P WCP KWAN, HJ FREEMAN. Signet ring cell carcinoma of the colon radiologi- ca lly simulating ileocecal tuberculosis. Can J Gast roenterol 1992;6(6): 341 - 344. A 65 -ycar-o ld Eas t Indi an woman with episodic vomiting a nd anem ia had radiological eva luations sh owing calci fi ed lymph nodes in the abdomen anJ chest, with narrowing of the il eoceca l va l ve and ascending co l on suggest ing chc possibility of ileoccal tuberc ulosis. Evaluations subseque ntl y proved this to be a signet ring ce ll ca rc inoma of th e co l on with infiltration of the te r minal il eum. Key Words: Crohn's dis e ase , Ileal st enosi s, Inflammatory bowel diseas e, Intestinal ruberculosis, Si gnet ring cell col on cancer Cancer des cellules en bague du colon simulant la tuberculose ileo-caecale a la radiologie RESUME: Une Ancillaise de 65 ans prcsentant des ep i sodes de vomi sse mc n cs et de ! 'anemic a subi des analyses radiol ogiq ues qu i one revelc Jes gang l ions lymphatiques calcif ies au ni veau Jc !'abdomen et du thorax, ainsi qu' un retrec i ~emenc de la va lv ul e ileo-caeca le et du c0lon ascendant, ce qui laisse ~urposer un diagnost ic possible de tuberculosc il eo-caeca le. Les examens su bsequents conf i rment qu'il s'ag it en fait d'un cancer des ce llules en bague du co lon avec infil trat i on de l'ilcon ter minal. Dep arrmenr of Medicine (( ;,lqrn,'mero/ogy), Unr wrsiry I /rJ.1/)i w/ and L/11il'Cr.111y of B nr1.1h Columbia . \/anrnrr wr. Brirish Co/111n/,i 11 Corres / )() nclen ce mu/ re/> ri nr s: Dr \\'IC Per <'r Ku •cm. Gmr mc ntcm/ og:,.•, ACU-F- 1 'l7, L' n ivers ir y Hospiwl ( UB CJ, 22 1 / \\'le.,hrook Mall , Vcmc<>11wr, Brit is h Co/wnhrci \/6T I \X/5 . re1~ 11ho11e (604) ,~22-72 16 R ecei ved fo r /mhlica ru111 ./m11wry / 0, / 992. Acce/)( <'ll }uly 15, 1992 CAN J GA~TROl::NTl:RL)l Vm 6 Nt) 6 N<.WEMBER/D l·t HlflER 1 992 S ll , NET RI N<, Ct\RCINOMA or Tl IE col on is a n unusual colonic muc in - llUS adcnoca rcinoma. Rec ause of its in- filtrntive nature, it ma y produ ce rndiological ch,m ge~ that mimic o th er diseases such as ischemic co liLi ~ nr C rohn\ disease. In the prese nt report , a case of signet ring ce ll ca rcin oma ti the cec um invo lving th e il eoceca l valve and term inal ileum that simulated ileoce ca l tuhe rculosis is described. CASE PRESENTATION A 65-year -o ld Ea~t Indian woman who emigra ted t ,> C, inada nin e year~ rre v1ous ly ITom India pre~enced with n five-year hi, tnry of rec urre nt na usea, vomiting a nd diftu,e ahdnminal dis- comfort. Diagnnstic lap,m ltomy do ne in a co mmunit y h llS piral at the onset o( sy mptlllns 1dent ifi eJ no c n1,e. No harium enema examina tio n lir endn- sco pi c , tudie s were performed prior to surgery. Th e re wa, no history of rectal bleedin g, altered hnwcl habit , f eve r or we ight loss. In 1 98 1 , he wa~ exposeJ tO a ~ister wi th active pulmonary cuber- HI

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Page 1: Signet ring cell carcinoma of the colon radiologically ...downloads.hindawi.com/journals/cjgh/1992/656898.pdf · diseases such as ischemic coliLi~ nr Crohn\ disease. In the present

CASE RECORD

Signet ring cell carcinoma of the colon radiologically simulating

ileocecal tuberculosis

WC PETER KW1\N, MD, FRCPC, HJ FREEMAN, MD, FRCPC, FACP

WCP KWAN, HJ FREEMAN. Signet ring cell carcinoma of the colon radiologi­cally simulating ileocecal tuberculosis. Can J Gastroenterol 1992;6(6):341 -344. A 65-ycar-o ld East Indian woman with episod ic vomiting and anemia had radiological evaluations showing calcified lymph nodes in the abdomen anJ chest, with na rrowing of the ileocecal va lve and ascending colon suggesting chc possibility of ileoccal tuberculosis. Evaluations subsequently proved this to be a signet ring cell carc inoma of the colon with infiltration of th e terminal ileum.

Key Words: Crohn's disease , Ileal stenosis, Inflammatory bowel disease, Intestinal ruberculosis, Signet ring cell colon cancer

Cancer des cellules en bague du colon simulant la tuberculose ileo-caecale a la radiologie

RESUME: Une Ancillaise de 65 ans prcsentant des episodes de vomissemcn cs et de !'anemic a subi des ana lyses radiologiques qui one revelc J es ganglions lymphatiques calcifies au niveau Jc !'abdomen e t du thorax, a insi qu'un retreci ~emenc de la va lvul e ileo-caecale et du c0lon ascendant, ce qui laisse ~urposer un diagnostic possible de tube rculosc ileo-caecale . Les examens subsequents confirment qu'il s'agit en fait d'un cancer des cellules en bague du colon avec infiltrat ion de l'ilcon terminal.

Deparrmenr of Medicine (( ;,lqrn,'mero/ogy), Unrwrsiry I /rJ.1/)iw/ and L/11il'Cr.111y of Bnr1.1h Columbia . \/anrnrrwr. Brirish Co/111n/,i11

Corres/)()nclence mu/ re/>rinrs: Dr \\'IC Per<'r Ku•cm. Gmrmcntcm/og:,.•, ACU-F- 1 'l7, L'niversiry Hospiwl ( UBCJ, 221 / \\'le.,hrook Mall , Vcmc<> 11wr, British Co/wnhrci \/6 T I \X/5 . re1~11ho11e (604) ,~22-7216

Received for /mhlica ru111 ./m11wry /0, /992. Acce/)( <'ll }uly 15, 1992

CAN J GA~TROl::NTl:RL)l Vm 6 Nt) 6 N<.WEMBER/D l·t HlflER 1992

S ll ,NET RIN<, Ct\RCINOMA or Tl IE

colon is an unusual colonic muc in­llUS adcnocarc inoma. Recause of its in ­filtrn t ive nature, it may produce rndiologica l ch,mge~ that mimic othe r diseases such as ischemic coliLi~ nr C roh n\ disease. In the present report, a case of signet ring cell carcinoma ti the cecum involving the ileocecal valve and term inal ileum that simul ated ileocecal tuherc ulosis is desc ribed.

CASE PRESENTATION A 65-year-old Ea~t Indian woman

who emigrated t,> C,inada nine year~ rrev1ously ITom India pre~enced with n five-year hi , tnry of recurrent na usea, vomiting and diftu,e ahdnmina l dis­comfort. Diagnnstic lap,m ltomy done in a community hllSpiral at the onset o (

symptlllns 1de nt ifieJ no c n1,e. No ha rium enema examination lir endn­scopi c , tudies were performed prior to

surgery. The re wa, no history of rec tal bleeding, a ltered hnwcl habit , fever or weight loss. In 198 1 , he wa~ exposeJ tO

a ~ister wi th active pulmonary cuber-

H I

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KWAN AN!) FRl:EMAN

Figure l) Barium enema $howing fixed and srenoric renninal ilet1m, cect1m ancl ascemling colon. Calcified ahclominal /:;mph nodes are Jlresenr

Figure 2) Detailed radiographic view of ileoce­cal region showing feawreless renninal ile11m wich rigid stenosis

culosis. The deta ils of her surveillance were not avai lab le but she d id not receive amituberculous tre(l tmen t and she was not deemed to have active pul­monary tuberculo~is. In March 199[, a harium swallow suggested multiple gastric erosiom hut gastroscupy fa iled to demonstrate ;iny lesion.

342

The patient was admitted ro University Hospital in August 199 I for furthe r investigatio n hecause of persist­ent abdo minal pai n and nausea. Physi­cal examination revealed an obese woman with a normal chest and abdo­minal examination; fecal occult blood testing was positive. S he was anemic with hemoglobin 109 mg/L and a mean cell volume of 68 fl. lron profile con­firmed iron defic iency with fc rritin 8 µ g/L. An a ir contrast barium e nema showed a gross ly abno rmal cecum with contract ion and loss of normal mucosa! outline while the ilcocccal va lve was stenoric and fixed (Figure l ). Cecal ah­normality extended up the ascending colon to the region of the hepatic fl ex­ure. Several calcific densities were pre­sent consistent with calcified lymph nodes. Distal terminal ile um was fcaurre lcss and appeared dilated (Figure 2) . C hes t radiographs revealed a cal­c ified density in the right upper lobe con-i tent with previous granulomarous

disease. Computed tomography scan of the abdomen and pelvis revea led a thick­ened cecum, ascending colon and distal tenninal ileum.

T he·c radiological changes sug­gested possible tuberc ulous involve­ment of the distal terminal ileum and ascending colon. Differential diagnoses included Crohn's disease, amcbias is and a colon ic neoplasm. The patient had a negative tuberculosis skin rest ( 5 TU) and she was not ancrgic.

The carc inoembryonic antigen wa 1.2 ~Lg/L ( normal less than 4 µ g/L). Stoo l cultures and examinations for parasit ic pathogens were negative.

Colo noscopy revealed an ahruptly narrowed lumen in the ascending colon ; the cecum could nm be intubated. The colonic muco a was extremely fr iable and abnorma lly thickened; b iopsies of these chicken ed mucosa! folds demon­strated signet ring cell carc inoma.

Laparotomy revealed a firm 6 cm mas· in the region of the cecum and terminal ileum. There was a 1.5 cm calc ified lymph node in the portal region. The omentum was c losely allied to the tumour but oth erwise there were no o bvious signs of local extension. A righ t hemicolectomy was performed wirh a resection of a port ion of the

involved term ina l ileum. I listolngical­ly, resection margins were free from tumour, and signet r ing celb extended through the wall of the cecum and into distal te rm inal ileum (Figures 3,4). In some sectio ns of term inal ileum, scat­te red signer rings ce lls were seen in­fi ltrating the mucosa! lamina propria (Figure 5 ). T here were tumour deposi t, in two meserneric lymph nodes. Other lymph nodes showed only calcitkar ion (no tumour deposi t or ev idence of prior tubercu losis).

The patient did well fo llowing sur­gery. Since there was lymph node in­volvement, adjuvant chemotherapy with s-nuorourac il and levamisole was offer­ed ( I) , but the patient refused treatment.

DISCUSSION Signet ring cell carc inoma of the

colon is an unusual adcnncnrcinoma cha1 wa~ fi rst de c ribcd in l 95 1 as a ' lini tis plastica' type of colon cancer ( 2). The tumour docs not rip pear t()

follow the hypothetical polyp-cancer sequence suggested for more typical colonic adenacarcinomas. Althm1gh n is known to be uncommon, rh1.: exact incidence of colonic signet ring cell carcinoma has not been determined. O ne series suggested it could he found in approximately l. 5% of all cases of colonic cancer (3), bur figures as low as 0 . l % have been reported ( 4). The clinical and radiographic features ha\'e been rev iewed (5) - most reports ind i­cate that patients with signet ring cell colo n cancer a rc younger than those wi th the usual colonic adenocarc inoma and that t he ini tial clin ical features commonly may mimic innarnmatory or ischemic diseases of the C()lon because of their radiographic aprearance~ on barium enema (6).

T he presented patien t had a pro­longed history of abdominal symptom· consistent with intermittent intestina l obstruction. Laparotomy done fi ve years earlier was 1wrmal. Although it is diffic ul t to accept that an deocccal tumour was present five years earlier, no other explana tion of the ,ymptonb was evident; early tumour in volvement in the ileocecal valve region may have resulted in t1bstructive symptl)m~. Fur­thermore, experience from Ita ly indi -

CAN J CiASTROENTERl) l V Oi 6 N() 6 NOVE~1BFR/Dll 'U,IBEI\ 1992

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Figure 3 ) PhoromicrograJ>li of ileal section showing submucosal mfilrrawm 111iih signer rin~ cells. (1-iemawxylin and eosin X 100)

cares that surviva l in pat ien ts with signet ring cell cancer of the colnn is similar to that of patients with the more typical h bmlogical variety of colonic adenocarcin()ma (6), sugge~-ring the growth rate of ~ignet ring cell colonic carc in­oma may not be exceptionall y rapid.

The patient's harium enema showed impressive contrnctinn of the cecum, narrowing of the ascending colon and a stenotic ileocccal va lve. There were calcified abdominal lymph nodes and chest radingraphs were consistent with prior granulomatous disease.

These radiographs were n(mspecific, hut taken with the country of origin of the patient and her history of exposure to tubercu lns is, i n1 esri nal ruhercu losis uwolving the ile()Cecal region w;1s con­sidered. It was estimated that 80 w 90% of patients wi th int estinal rube rculnsis wi ll have involvement of the ikocecal region, possibly because of the ri ch lym­phatic supply in this region.

However, in a review of 81 cases nf ~bJom inal tuherculosb in Canada, in­cluding patie nts wi th tuherculow, peri­tonitis (7) only 21 <){1 had ileoceca l in­volvement. Of these cases, 59% had c\'iJence of tubercu lo ·is elsewhere. Thus, the absence of pulmonary chan­ges indicative ofactive tuhercul()Sis wi II not necessari ly alle r the initia l radio-

Figure 4) Higher power phowmicrogra/>h of subm11cosa of ilea/ section showing signet rillg cell infiltrating musrnlaris mucosa. ( Hemawx~lin and eosin x200)

logic interpretation. The classic rad io­logical appearance of ilcucecal tubercu­losis is a con ical, shrunken, con tracted cecum with a narrow, ulcerated Lermin­al ileum. Because of mesocolon con­traction, the cecum may be pulled out of iliac fossa. With more advanced dis­ease and str ict uring of the ileocecal valve, dilation of the terminal ileum occurs (8,9), and deep ulcerations, fis­sures and fistulous tracts may develop.

The radiological mimicry of ilcoce­cal tube rcu los is in chis case is produced hy the prope ns ity of th e signe t ring car­cinoma cells to infiltrate the bowel wall resulting in marked narrowing of the colon over a con iderab le distance with rigidity and fixation ( 10, 11). In the presenL patient rhe lumour in fi ltrated the cecal wall , ileocecal valve and ter­minal ileum, with barium enema ch;:m­ges resulting. Alternative radio logical diagnoses included other inflammatory condi tions, such as Crohn's disease or ischemic disease. For ~ignet ri ng ce ll carc inoma occurring elsewhere in th e colon , barium enema changes may sim­ul ate spasm, Crohn's disease, ischemic coli t is with stricture and complica ted diverticular disease (5, 12, 13 ).

Tuberculous involvement of the colon as ide from the ileocecal region can produce varied rndingrnphic a nd

CAN J GASTRl )FNTEROI Vol 6 No 6 N1.)VH,11\FR/DF<'EMHER 1992

Signet ring cell colon cancer

Figure 5) Plwwmicrop;raph of ilea/ section slwwing signet ring cell camnoma infilrrarinl! lamina J>ro/)ria {Jericryptal region. (Hemawxylin (Ind eosin x200)

endoscopic appemances, including ;,eg­mencal strictures, ulceration;, or hyper­trophic nodular mucosa (14). The en­doscopic appearance of the right colon in the patient comprised ahnormal, hemorrhagic mucosa but because of the degree of bleeding and friability en­coumered, adequate v isu,1liw1 ion was not possible. However, no large ulcer­ation was encounte red.

Signet ring cell carcinoma of the small intestine also is quite rare; how­ever, it has been de cribed in Crohn's disease (15), ileostomy stoma (16,17) and in an ilea! segment following ilco­cysroplasry ( 18) . Spreading of the sig­net ring cells into the terminal ileum in the pat ient was expected, given the biological nature of this tumour as reflected by t he in vitro ohservations demonstrated for the signet ring cell carcinoma cell line, DLD-2; this li ne showed inc reased invasiveness through basement membrane compared with othe r colonic e pi thelial cell lines (19).

T his case illustrates that in signet ring cell carcinoma of the colon, the biological behaviour of the malignant cells can result in an unusual constel­l;;icion of barium radiographic findings that may simulate other inflammatory diseases, part icu larly infection includ­ing ileocecal tuberculosis.

143

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KWAN /\NI) FRl:FM1\N

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JS, ct al. Levasirnolc nnd fluomurncil fo r ndjuvanl therapy of resecteJ colon carcino ma. N Engl J Med I 990;322:352-8.

2. La ufman J-1 , Saphir 0. l'rirnary linitis pla,tica type of carc inoma ,if the colon. Arch Surg 1951 ;62: 79-9 1.

3. Lui IOL, Kung ITM, LeeJMH, Boey JH . Primary colorecra l signet-ring carcinoma in young patients. Report of 3 cases. Pathology 1985; 17:3 1-5.

4 . Fahl JC, Dockerly MD. Judd ES. Scirrho us carcinoma of the cok,n and rectum. S urg Cynccol Obstct 1960; 1 l l :759-66.

5. Kwan W C P, Frecmnn HJ . S ignet-ring cell carcinoma tif the colon radiologically simulming C rohn's colit is. Can J Gastroentero l 199 I ; 5: 7 1 -4.

6. Giacchcm A, A,tc H, Bmacchini P. er al. Primary signet-ring c:ircinoma ,if th.: large bowel. Rcpnrt nf nine crn,cs. Cancer 1985;56:272 3-6.

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H, Endt1 M, T :1kemoto T. The fihercolonoscopic diugno.sis of intestina l tubercul\1sb. Endoscopy l 975;7: l 12-2 I.

l 5. Petra, RE, Mir-Madjbsi SH. Farmer RG. Crohn's dise,1se and mt('stina I c,1rc inoma. Gasrrocntcrology 1987;9'3: 1307 - l 4.

16. J-1hnson CD. Primary mucinow, adenncmcinoma developmg m an ileostomy stoma. G ut l 989; 30:889.

I 7. Sman PJ, Samy S. Wells S. Primary mucinous aJ enocarc moma developing in an ilc(1sromy stoma. G ut 1988;29: 1607- 12.

18. T :ib,aki E, Murahashi I, T,1ynda M, l-fond,1 M, W,, ku S. Signe! ring adenocarcinnma of ilea! segment foll,nving ilencystoplasty. J Urn! 1981; l 30:562- 3.

19. DanekcrGW Jr, r,a.:zn AJ , Steele CD Jr, Mercurio AM. ln1crnc11on~ of hum,111 culorecw I c:i rcmoma cc I b with hascmcnl mc111 hrnnes. Analy,1s (llld corrcl:HllHl with differential inn. A rch Surg l 979; l 24: 181- 7.

CAN J l~N,TRl\ENTFR( l l Vl lL 6 Nu 6 N\ Wl ~ !BI-R/Dl-l l·M lllcR 1992

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