3 Clin Pathol 1997;50:350-358

Short reports

Late stage congestive gastropathy

Ornella Leone, Magda Zanelli, Simonetta Piana, Donatella Santini, Domenico Marrano

AbstractGastric mucosal abnormalities resultingfrom portal hypertension are defined as"congestive gastropathy". A case of con-gestive gastropathy with unusual features,in a 63 year old man with a history ofexcessive alcohol intake and cirrhosis, isdescribed. The patient underwent a sub-total gastrectomy because of profusebleeding from a gastric ulcer, providing alarge surgical specimen for examination.Unusual gross and histological findingsincluded prominent arterial intimal hy-perplasia, and diffuse duplication andfocal fragmentation ofthe internal laminaelastica. The differential diagnosis of thiscondition includes primary angiodysplas-tic gastropathy such as Dieulafoy's dis-ease. The similarity with Dieulafoy-likeangiodysplasia emphasises that clear cutcriteria to define gastric vascular lesionsdo not yet exist.(7 Clin Pathol 1997;50:350-358)

Keywords: congestive gastropathy; cirrhosis; primaryangiodysplasia

sia, arteriovenous malformation, haemangiomaor angioma.'We report a case of congestive gastropathy in

a patient with portal hypertension focusing onthe histopathological aspects of a long standingdisease.

Case reportA 63 year old man, with a long history ofexcessive alcohol intake and with symptomaticcirrhosis, was admitted urgently to our institu-tion suffering from haematemesis andmelaena. At admission, his haemoglobin con-centration was 69 g/l, red blood cell count2200 x 109/l, packed cell volume 0.2, mean cellvolume 91.3 p3, white blood cell count8.82 x I09/l, and platelet count 68 000 perlitre. The gastroscopy, urgently performed,disclosed an ulcer (1.5 cm in diameter) on theposterior wall of the stomach. After three days,owing to a second episode of haematemesis,the patient was transferred urgently to the sur-gical department. A further gastroscopyshowed an active, profuse bleeding from thegastric ulcer prompting a subtotal gastrectomy.

Institute ofAnatomicPathology, UniversityofBologna, OspedaleSant' Orsola, ViaMassarent 9, 40138Bologna, ItalyO LeoneM ZanelliS PianaD Santini

Insitute of Surgery ID Marrano

Correspondence to:Dr Ornella Leone.

Accepted for publication7 May 1996

Congestive gastropathy and mucosalvasculopathy' are the terms currently used todefine gastric mucosal abnormalities occurringin portal hypertension. The endoscopicappearance2 of this condition has been widelystudied and the macroscopic mucosal changeshave been categorised as fine pink speckling,superficial reddening, and, the most common,a mosaic or snake-skin pattern. The histologi-cal hallmark3 related to these endoscopicaspects is a prominent vascular ectasia in thelamina propria with an almost complete lack ofinflammatory cells. In addition, submucosalextensive oedema, thickened arterioles andveins showing features of arterialisation havebeen described.4To the best of our knowledge, reports of the

morphological patterns of vascular abnormali-ties in portal hypertension have been describedonly in gastric biopsies. Despite the originalstudy ofMoore et al 'in 1976, further modifiedby Lewi6 and Fowler,7 the classification of gas-trointestinal vascular malformations and therelated morphological findings remain unclear.Recent interest has focused on angiodysplasia,'a still undefined condition called vascular ecta-

Figure1Necrosis of thesupe"ficial and glandula

epteiu n hamrrhagc efuso in t''J-Ffhelamina .;

:ropria, related to ischaemic damage

Figure 1 eoi of th.uefca n lnua

epithelium and haemorrhagic effusion in the laminapropria, related to ischaemic damage.

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I NI.W

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Figure 2 Artery showing intimal hyperplasia and aspectsof vascular neoformation.

PATHOLOGICAL FINDINGSThe stomach measured 14.5 cm along thesmall curvature and 23 cm along the great cur-vature. The mucosa was brownish and widelyhaemorrhagic. On the posterior wall, in thedistal part of the gastric body, there were fourulcers, ranging in diameter from 1.9 cm to0.8 cm. One of the ulcers was sutured. Close tothe proximal resection margin, on the anteriorwall, there was an area (3.5 cm in diameter) ofgreyish mucosa and haemorrhagic speckles. Allof the ulcers along the above mentioned areawere sampled.The specimens were fixed in 10% buffered

formalin and paraffin embedded; 2 gm sectionswere stained with haematoxylin and eosin, vanGieson, and Masson's trichrome methods.

Microscopic examination confirmed thepresence of multiple acute ulcers; the sur-rounding mucosa showed features consistentwith ischaemic damage, such as necrosis of thesuperficial and glandular epithelium, andhaemorrhagic effusion in the lamina propria(fig 1). The remaining mucosa showed chronic,

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focally atrophic, gastritis with diffuse intestinalmetaplasia and foveolar hyperplasia.The submucosa showed marked oedema and

vascular anomalies involving both the arteriesand veins.The arteries were enlarged and showed

different stages of intimal hyperplasia up to acomplete obliteration ofthe lumen with aspectsof vascular neoformation (fig 2); the internallamina elastica was diffusely reduplicated andfocally fragmented. The parietal muscle layerwas hypertrophic and the fibres appearedintermingled with fibrous tissue (fig 3).The veins were also enlarged; some showed a

circumscribed thickening with non-concentricmuscularisation (fig 4), and others, in absenceof muscularisation, had a marked derangementof the structure, with fragmentation of theelastic fibres. The more the elastic fibres weredissociated, the less the muscularisation wasprominent. All of these vascular abnormalitieswere present throughout the stomach, butmore prominent in the ulcerated areas. Finally,the gastric muscle coat appeared somewhatreduced and focally interrupted by connectivetissue.

DiscussionTo date, the term "congestive gastropathy" hasbeen used to identify a clinicopathologicalentity related to portal hypertension. Onendoscopy, the features consistent with conges-tive gastropathy are quite well known and usu-ally described as "mosaic pattern" or "snake-skin pattern". The histopathological findings,such as vascular ectasia in the lamina propria,submucosal oedema, thickened arterial wall,and arterialisation of the veins, have also beendescribed, but only on bioptic fragments and ina relatively early stage of portal hypertension.

In our case, owing to the evaluation of a sur-gical specimen, we have been able to identifyand describe the prominent parietal wallvascular abnormalities occurring in a longstanding portal hypertension. Both veins andarteries were involved. The diffuse and con-spicuous venous muscularisation is in keepingwith a long standing portal hypertension. In thearteries, the prominent intimal hyperplasiawith scattered oedema could be the expression

_v -

Figure 3 Fibrous tissue within the parietal muscle layer.

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Figure 4 Vein showing non-concentric muscularisation.

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of the arterial involvement secondary to a longstanding venous congestion, actually repre-senting a more advanced stage of the disease.Indeed, the presence of fibrous tissue withinthe gastric muscle coat is likely to represent thedystrophic effect due to the vascular abnor-malities.The acute ulcers and the superficial mucosal

necrosis could be an expression of either alco-hol dependent toxicity or ischaemic damagesecondary to the prominent vascular altera-tions. Features in keeping with the latterpathogenesis are mucosal atrophy, submucosafibrosis, and the presence of fibrous tissuewithin the gastric muscle.

In addition, we noted the similarity of somearterial abnormalities-intimal hyperplasia upto total lumen obliteration, diffuse duplication,and focal fragmentation of the internal laminaelastica-with the arterial anomalies describedin primary angiodysplasia such as Dieulafoy'sdisease.'" Nevertheless, in the present case theclinical presentation in a cirrhotic patient withportal hypertension was not in keeping with thehypothesis of a Dieulafoy-like angiodysplasiathat usually occurs in middle aged and elderlymen without any relevant family history, exces-sive alcohol or aspirin intake. In addition, inDieulafoy's disease the mucosal ulcer, localisedin the upper stomach, is usually single, small,and shallow, whereas in our case the ulcerswere multiple and deep penetrating. Finally,the prominent muscularisation of the veins was

the main histological feature supporting a latestage congestive gastropathy rather than anangiodysplasia.

In conclusion, our study focuses on the largespectrum of microscopical vascular alterationspresent in a late stage congestive gastropathy,of which, to the best of our knowledge, this isthe first description. The similarity withDieulafoy-like angiodysplasia emphasises thatclear cut criteria to define gastric vascularlesions do not exist yet.

1 Sarfeth IJ, Tarnawski A, Hajduczeck J, Stachura J, Bui HX.Gastric mucosal vasculopathy in portal hypertension. Gas-troenterology 1987;92:1129-31.

2 Sarin SK, Misra SP, Singal A, Thorat V, Broor SL. Conges-tive gastropathy in portal hypertension: variations in preva-lence. Am _r Gastroenterol 1988;83:1235-9.

3 Quitero E, Pique JM, Bombi JA, Bordas JM, Sentis J,Montserrat E, et al. Gastric mucosal vascular ectasias caus-ing bleeding in cirrhosis. Gastroenterology 1987;93:1054-61.

4 Triger DR, Hosking SW The gastric mucosa in portalhypertension. J Hepathol 1989;8:267-72.

5 Moore JD, Thompson NW, Appelman HD, Foley D. Arte-riovenous malformations of the gastrointestinal tract. ArchSurg 1976;11:381-9.

6 Lewi HJE, Gledhill T, Gilmour HM, Buist TAS. Arterio-venous malformations of the intestine. Surg Gynecol Obstet1979;149:712-16.

7 Fowler DL, Fortin D, Wood WG, Pinkerton JAJr, KoontzPG Jr. Intestinal malformations. Surgery 1979;86:377-85.

8 Scully R, Mark EJ, Mc Neely WF. Case records of the Mas-sachussets General Hospital. N Engl7Med 1991;10: 1086-96.

9 Tivadar L M, Vilmos AT. The caliber persistent artery of thestomach: a unifying approach to gastric aneurysm, Dieula-foy's lesion, and submucosal arterial malformation. HumPathol 1988;19:914-21.

10 Leone 0, Zanelli M, Santini D, Minni F, Marrano D. Dieu-lafoy's disease associated with early gastric cancer. J ClinPathol 1995;48:267-70.

Inflammatory pseudotumour of the liver

G Kafeel, P U Telesinghe

Department ofPathology, StateLaboratory, RIPASHospital, BSB,Darussalam, BruneiG KafeelP U Telesinghe

Correspondence to:Dr G Kafeel.

Accepted for publication10 December 1996

AbstractInflammatory pseudotumour is not acommon lesion. The first series of 12 caseswas described in 1986, to which 37 morecases have now been added. The histology,differential diagnosis, and prognosis ofthis lesion have been described in detail,but the aetiology is unknown and the modeoftreatment remains controversial. A newcase is presented and compared with thepreviously reported cases. Fine needleaspirate yielded a growth of klebsiellaorganisms. The possibility ofthis infectionas an aetiological agent is considered.(7 Clin Pathol 1997;50:352-353)

Keywords: inflammatory pseudotumour; liver; kleb-siella

Case reportA 70 year old female was referred to RIPASHospital, Brunei with a history of intermittentlow grade fever, progressive weight loss andweakness, and vague abdominal pain on the

right side. She looked sick and weighed only 32kg. Her body temperature was raised at38.4°C. A tender mass was felt in right iliacfossa. Ultrasonography of the abdomenshowed a necrotic mass of 4.0 cm diameter onthe inferolateral aspect of the right lobe of theliver displacing the hepatic flexure downwards.Except for a raised erythrocyte sedimentationrate (ESR) of 30 mm in the first hour the restof her haematological and biochemical profile,including the liver function tests, was withinthe normal range. a-Fetoprotein (AFP) andcarcinoembryonic antigen (CEA) were notraised.

Ultrasound guided fine needle aspiration ofthe mass yielded about 2 ml of turbid fluidwhich showed abundant neutrophils, fibrin,and few degenerating liver cells on a necroticbackground. No malignant cells, fungal ele-ments, parasites, or acid-fast bacilli could beseen. Kiebsiella spp were cultured from theaspirated material. The lesion was consideredto be inflammatory. After six weeks of treat-

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