A rare gastric polyposis: Cronkhite-Canada syndrome

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RADIOLOGIC PATHOLOGIC CORRELATION / Gastrointestinal imaging

A rare gastric polyposis: Cronkhite-Canada syndrome

C. Sellal

^a^,∗

, C. Lemarié

^b

, F. Jausset

^a

, A. Babouri

^c

, V. Laurent

^a

, D. Régent

^a

aDepartmentofAdultRadiology,hôpitalBrabois,rueduMorvan,54511 Vandœuvre-lès-Nancy,France

bDepartmentofPathology,hôpitalBrabois,rueduMorvan,54511Vandœuvre-lès-Nancy, France

cHepatogastroenterologyDepartment,hôpitalBrabois,rueduMorvan, 54511Vandœuvre-lès-Nancy,France

KEYWORDS Gastrointestinal polyps;

Gastricpolyposis;

Cronkhite-Canada syndrome

Case report

A 78-year-old man, without personal or family antecedents, consulted for acquired anorexiaandprofuse diarrhoeawitheightto10 bowelmovementsper dayfor thelast 6weeks,appearingsuddenlywithoutanepidemicormedicinalcontextandnotrelieved bysymptomatictreatments.Hisgeneralconditionwaspreservedbutthepatientreported the loss of 6kg. The clinical examination detected onychomadesis (detachment of the nails)(Fig.1)andalopeciaofthescalp.Hisabdomenwassupple,withoutpalpablemass orhepatosplenomegaly.Thelaboratorytestsdetectedahypoalbuminemiaat23g/Landa macrocytosisat102fL.Thebloodcount,theinflammatory(sedimentationrate,reactive proteinC, electrophoresis of the plasmaproteins and fibrinogen), immune andthyroid assessments were normal. The faecal culture and the parasitology examination of the stoolsprovedtobenegative.Thegastroscopyfoundcongestiveantralgastritiswithlarge veryerythematouscerebriformfolds,withaninfiltratedappearanceofthebulbandthe duodenum(Fig.2aandb).Thecolonoscopyrevealedagreatmanyraspberry-like,rednon- ulceratedandnon-hardenedsessilepolypsalongtheentiresurfaceofthelargeintestine associatedwithacongestivemucosawithoutlesionssuspectedofmalignancy(Fig.2cand d).

∗Correspondingauthor.

E-mailaddress:[email protected](C.Sellal).

doi:10.1016/j.diii.2012.04.006

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800 C.Sellaletal.

Figure1. Onychodystrophyofthepatient.

Thegastric andsigmoid-colicbiopsiesrevealcysticand tortuousdilationoftheglandsofthegastricmucosa,coated withawell-differentiated and non-dysplastic singlestrat- ified epithelium (Fig. 3a—c). The glands are irregularly dispersed in an abundant, focally haemorrhagic chorion, consistingofapolymorphousinflammatoryinfiltrateoflym- phoplasmocytarypredominance (Fig.3d). Thepatientalso underwentacolon-MRIandCTenteroclysisthatfoundmul- tiplepolypsalong theentire gastric antrumand thelarge intestine, with more severe impairment of the sigmoid (Fig.4a—d).Adoubtremainedastotheimpairmentofthe smallintestinebyCTenteroclysis,althoughtheendoscopic videocapsule carried out as a complement found a jeje- unumandileumwithahealthymucosa,freeofanypolyps.

Themanifestationsoftheinteguments(partialalopeciaand onychotrophia),associatedwith gastricpolyposis, support thediagnosis ofCronkhite-Canada syndromeraised during theanatomopathologyexamination.Aninitialtreatmentby intestinal anti-inﬂammatory consisting of 5-aminosalicylic acidderivatives(5-ASA)wasfoundtobeineffective.Atrial treatmentwithcorticoidsandnutritionalmeasureswasini- tiated and improved the clinical symptomatology in two

months. The patientwas informed of the possibilityof a colectomyincaseofclinicalaggravationorinefﬁcacyofthe drugtreatments.

Discussion

Cronkhite-Canada syndromeis araredisorder ofunknown pathogenesis.It wasdescribedin 1955by LeonardWolsey Cronkhite Jr, an internist and Wilma Jeanne Canada, a radiologist [1]. This syndrome associates non-hereditary gastrointestinal polyposis, onychotrophia, alopecia and skin hyperpigmentation. The incidence is estimated at 1/1,000,000with400casesdescribedintheworld,75%of which arein Japan [2]. The mean age of thediagnosis is 59years[3].Theclinicalpictureisdominatedbyabundant diarrhoea,protein-losingenteropathy,frequentlyresponsi- ble for severe undernutrition and hydroelectric disorders thatconditiontheprognosisofthisdisease.The5-yearrate ofsurvivalis55%.

Polyposisischaracterisedbysessile,red,raspberry-like polyps of variable size that affect the entire digestive tube except for the oesophagus. Initially described as adenomateous polyps [1], in reality the lesions corre- spondtohamartomateousjuvenilepolyps.Thehistological examination shows a preserved surface epithelium, cys- tic proliferationand dilation of theglands of the mucous membrane enclosing mucus. The chorion is inﬂammatory, abundantandenclosesapolymorphouscellinﬁltrate.Cases ofcancerousdegenerationhavebeenreportedalthoughthe origin is not certain since, classically, juvenile polyps do not degenerate [4,5]. The hypothesis of cancerisation of co-existantadenomateouspolypsismostlikely.Therefore, regularannualendoscopicmonitoringisrecommended[3].

JuvenilepolyposisandthePeutz-Jegherssyndromearethe maindifferentialdiagnoses[6].Giventherarityofthisdisor- der,thetreatmenthasnotbeenclearlyestablished.Dietetic measuresandcorticoidtreatmentareusuallyproposed.The durationvariesaccording totheclinicalresponse[3].Sur- gical treatment by subtotal gastrectomy or colectomy is reservedforthecomplicatedforms(gastricocclusion,can- cer)orformsresistanttomedicaltreatmentduetoitshigh morbidity.

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Figure2. a:gastroscopy:polypofthegastricantrum(whitearrow);b:gastroscopy:voluminouscerebriforminﬂammatoryfoldsofthe gastricantrum;c:colonoscopy:multiplessessilepolypsofthesigmoidcolon;d:colonoscopy:raspberry-like,hamartomateouspolypofthe sigmoid(arrow).

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802 C.Sellaletal.

Figure3. a:histologicalexaminationofapolypofthegastricantrum:dilatedandtortuousappearanceoftheglandsofthegastricmucosa (HES×2.5);b:detailoftheglandularepitheliumofthegastricantrum:ornateappearancewithoutdysplasia(HES×10);c:histological examinationofapolypofthesigmoidcolon:cysticdilationoftheglandsandcryptsofthecolonmucosa(HES×10);d:sigmoidcolon:

tortuousglandsedgedbyanon-dysplasicepithelium(blackarrow)dispersedinanabundantandinﬂammatorypolymorphouschorion(black star)(HES×20).

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Figure4. a:colon-MRI:polypofthetransversecolon(arrow);b:colon-MRI:sessilepolypsofthesigmoidcolon(arrows);c:CTenteroclysis:

polypoidthickeningoftherightcolicangle(arrows);d:CTenteroclysis:multiplesessilepolypsofthesigmoidcolon(arrows).

Disclosure of interest

Theauthorsdeclarethattheyhavenoconﬂictsofinterest concerningthisarticle.

References

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