Short reports

Angiolymphoid hyperplasia with eosinophilia inthe colon: a novel cause of rectal bleeding

D M Berney, M P Griffiths, C L Brown

AbstractAngiolymphoid hyperplasia with eosino-philia (epithelioid haemangioma) is anuncommon but distinctive lesion seenprincipally in the skin. A case of severegastrointestinal haemorrhage in a 63 yearold male is reported, which necessitated aright hemicolectomy. A lobulated nodulewas seen macroscopically that had thehistological appearance of angiolymphoidhyperplasia with eosinophilia, with sheetsoflymphocytes and eosinophils associatedwith many vessels showing plump andpleomorphic endothelial cells. This is thefirst reported case of this entity in thelarge intestine.( Clin Pathol 1997;50:611 -613)

Keywords: angiolymphoid hyperplasia; eosinophilia;colon

Angiolymphoid hyperplasia with eosinophilia(epithelioid haemangioma) is a familiar lesionthat presents as a skin lesion usually around thehead and neck. There are reports of this entityoccurring in the mouth' 2 but the lesion has notbeen reported elsewhere in the gastrointestinaltract. Angiolymphoid hyperplasia is often asso-ciated with an underlying vascular malforma-tion. A case is reported illustrating that thisreactive inflammatory condition may lead tosevere gastrointestinal haemorrhage, and dem-onstrates that this unusual lesion may occur atunexpected sites.

Case reportA previously well 63 year old African male wasadmitted as an emergency complaining of rec-tal bleeding. He had a six hour history of twobouts of profuse bloody motions associatedwith light headedness. He had no history ofprevious gastrointestinal problems and therewas no significant history of non-steroidal anti-inflammatory use.On examination the only positive finding of

note was mild left sided abdominal discomfort.Rectal examination was normal. Sigmoidos-copy revealed profuse bleeding but no bleedingsite was seen. All haematological and bio-chemical investigations were within normallimits. The patient had two further bouts ofrectal bleeding requiring resuscitation with twounits of colloid. Colonoscopy determined thesource ofbleeding as the right side of the colon,although the precise site was not identified.Immediate laparotomy and right hemicolec-tomy was performed; the patient made anunremarkable recovery.

PROCEDURESThe resected bowel was fixed in bufferedformalin. The caecum and ascending colonwas 35 cm in length. The terminal ileum was2 cm in length. Macroscopically a multilobu-lated nodule 1.6 cm in diameter was noted15 cm from the distal colonic excision margin.No other abnormality was seen.

Blocks of the nodule and the macroscopi-cally normal bowel were taken and embeddedin paraffin. Sections were stained with haema-toxylin and eosin, van Gieson, and periodicacid Schiff. The sections were also studiedimmunohistochemically using the avidin-biotin-peroxidase technique using antibodiesagainst CD34 (Q BEND 10, Bionostics,Bedfordshire, UK), factor VIII (Dako, HighWycombe, UK), and CAM 5.2 (Becton Dick-inson, Oxford, UK).

MICROSCOPIC FINDINGSMicroscopically, the nodule comprised inflam-matory cells infiltrating the submucosa in alobular distribution, extending through themuscularis mucosae into the lamina propriaand the muscularis propria (figs 1 and 2).The inflammatory cells principally com-

prised lymphocytes and eosinophils that sur-rounded many small capillaries, many of whichhad a plump "hobnail" endothelial lining (fig3). Focal ulceration was noted. Immunohisto-chemistry showed these vessels to be stronglypositive for CD34 and factor VIII but negative

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Figure 1 Low power view showing the lobular distributionof the inflammatory cell infiltrate and a large artery at thebase of the lesion.

Department ofHistopathology andMorbid Anatomy, TheMedipal and DentalSchool of StBartholomew's and theRoyal LondonHospital, The RoyalLondon Hospital,London El 1BB, UKD M BerneyC L Brown

Department ofSurgeryM P Griffiths

Correspondence to:Dr Berney.

Accepted for publication8 April 1997

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Figure 2 Dense infiltrate of inflammatory cells in thesubmucosa with an adjacent distorted artery.

for CAM 5.2. A prominent distorted mediumsized artery was seen beneath the maininfiltrate. An elastic van Gieson stain showeddisruption and reduplication of the internalelastic lamina and intimal hyperplasia of thisartery (fig 4). The regional lymph nodesshowed non-specific reactive changes.

Sections from the remainder of the colonshowed no other abnormality. In particularthere was no vascular ectasia or evidence ofinflammatory bowel disease.

DiscussionThe histological findings in this case are iden-tical to those seen in the skin in angiolymphoidhyperplasia with eosinophilia (epithelioidhaem-angioma). This entity typically occurs duringearly to mid adult life and affects more womenthan men. Most lesions are situated around thehead and neck. Bleeding is a common second-ary feature. Occasionally there is local lymph

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node enlargement and peripheral eosinophilia,although an infectious agent has never beenisolated. One third of the lesions recur and onecase was reported in which metastasis to aregional lymph node occurred3; however, largeseries illustrate benign behaviour.4 Althoughsome authors consider these lesions to be neo-plastic, it has been shown that 60% of cases areassociated with a large vessel showing muraldamage or rupture.5 6 This is identical to thefindings in the present case and we believe thatthese lesions are reactive in nature, possiblyarising secondary to damage and repair of anartery or vein. For this reason we do not preferthe alternative name for this lesion, epithelioidhaemangioma, which implies a neoplasticentity.Angiolymphoid hyperplasia with eosino-

philia is frequently confused with Kimura'sdisease with which it has some superficial mor-phological similarities.7 However, deposits ofKimura's disease contain numerous lymphoidfollicles and have attenuated endothelial cellslining the vessels8 while large distorted vesselsare not seen. Kimura's disease is also invariablyassociated with a peripheral blood eosinophilia.To our knowledge, angiolymphoid hyperplasiawith eosinophilia has not been reported in thelarge bowel. However, cases are reported withlesions within the oral cavity2 and specificallyon the tongue.' Vascular ectasias and angiodys-plasia are well recognised in the large bowel asa cause of rectal bleeding and Dieulafoy's vas-cular malformation has similar large vessels toour case running in the submucosa. However,there are no reports of an associated inflamma-tory cell infiltrate of this nature in thesemalformations and Dieulafoy's vascular mal-formation is seen primarily in the stomach.The alternative diagnoses of inflammatoryfibroid polyp and eosinophilic colitis were alsoconsidered. While inflammatory fibroid polypsfrequently possess a marked infiltrate ofeosinophils, they have not been reported asshowing the marked arterial changes that wereseen at the base of this lesion, nor theprominent endothelial changes. Also, there wasno oedema or spindle cell stroma in our case,features that are well described in inflamma-tory fibroid polyp. The possibility of eosi-nophilic colitis is ruled out because of the focalnature of the lesion. Histopathological recogni-tion of angiodysplasia is frequently difficult inresected specimens because of the collapse of

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Figure 3 High power view showing sheets oflymphocytesand eosinophils including vessels lined with "hobnail"endothelial cells.

Figure 4 Artery at base of lesion showing severe distortionand reduplication of the internal elastic lamina.

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vessels. In contrast, this lesion was easily iden-tified by the nodule seen macroscopically. Thepropensity of angiolymphoid hyperplasia witheosinophilia to bleed was seen in this case,highlighting a potentially fatal outcome of anunusual lesion previously unreported at thissite.

1 Razquin S. Mayayo E, Citores MA, Alvira R. Angiolym-phoid hyperplasia with eosinophilia of the tongue: report ofa case and review of the literature. Hum Pathol1991;22:837-9.

2 Toeg A, Kermish M, Grishkan A, Temkin D. Histiocytoidhemangioma of the oral cavity: a report of two cases. J OralMaxillofac Surg 1993;51:812-14.

3 Reed RJ, Terazakis N. Subcutaneous angioblastic lymphoidhyperplasia with eosinophilia (Kimura's disease). Cancer1972;29:489-97.

4 Olsen TJ, Helwig EB. Angiolymphoid hyperplasia witheosinophilia: a clinicopathologic study of 116 patients. JAm Acad Dermatol 1985;12:781 -96.

5 Fetsch JF, Weiss SW. Observations concerning the patho-genesis of epithelioid hemangioma (angiolymphoid hyper-plasia). Modern Pathology 199 1;4:449-55.

6 Henry PG, Burnett JW. Angiolymphoid hyperplasia witheosinophilia. Arch Dermatol 1978;114:1168-72.

7 Kung ITM, Gibson JB, Bannatyne PM. Kimura's disease: aclinicopathological study of 21 cases and its distinctionfrom angiolymphoid hyperplasia with eosinophilia. Pathol-ogy 1984;16:39-44.

8 Googe PB, Harris NL, Mihim MC. Kimura's disease andangiolymphoid hyperplasia with eosinophils. Two distincthistopathological entities. J Cutan Pathol 1987;14:263-71.

Mucinous cystadenoma of the appendix withraised serum carcinoembryonic antigenconcentration: clinical and pathological features

T Shimizu, M Shimizu, K Kawaguchi,W Yomura, Y Ihara, T Matsumoto

Shimizu Women'sClinic, Takarazuka,JapanT ShimizuW Yomura

Department ofPathology, KawasakiMedical School,Kurashili, JapanM Shimizu

Division ofGastroenterology,Department ofMedicineT Matsumoto

Department ofObstetrics andGynecology, TazukeKofukai MedicalResearch Institute,Kitano Hospital,Osaka, JapanK Kawaguchi

Department ofObstetrics andGynecology, KobeCity General Hospital,Kobe, JapanY Ihara

Correspondence to:Dr Shimizu, 2-2-4Minamiguchi, Takarazuka,Hyogo, Japan 665.

Accepted for publication13 May 1997

AbstractA case ofmucinous cystadenoma mimick-ing ovarian cancer is reported. Serumcarcinoembryonic antigen (CEA) concen-tration was raised, and computed tomo-graphy ofthe abdomen and pelvis demon-strated a long oval shaped cystic massmeasuring 9 cm in length on the rightanterior side of the uterus. Because ofpossible right ovarian cancer, laparotomywas performed and the mass was found tobe a mucinous cystadenoma ofthe appen-dix. This case indicates that mucinouscystadenoma ofthe appendix may show anunusual presentation including its locationas well as the high serum CEA, mimickingovarian cancer. Therefore, gynaecologistsas well as gastroenterologists should con-sider its possibility as a differential diag-nosis ofthe right adnexal mass in a patientwithout previous appendectomy.(3 Clin Pathol 1997;50:613-614)

Keywords: mucinous cystadenoma; mucocele; appen-dix; carcinoembryonic antigen; ovarian cancer

An enlarged appendix with luminal dilatationby mucus has generally been called mucocele.'Higa et al,2 in 1973, investigated cases ofmucocele of the appendix and they classifiedtheir lesions into three groups: mucosal hyper-plasia, mucinous cystadenoma, and mucinouscystadenocarcinoma. These investigators alsorecommended avoiding the term mucocele.However, mucocele is still used for grossdescription, rather than histological diagnosis.'In addition, the term retention mucocele, alsocalled simple mucocele, is still applied to apathological description of the mucinous

dilatation of the appendiceal lumen resultingfrom any cause other than epithelialproliferation.3 On the other hand, when anappendiceal adenoma secretes large amountsofmucus resulting in a clinically palpable cysticlesion, the term cystadenoma of the appendixpreferably is used.We report a case of mucinous cystadenoma

of the appendix accompanied by raised serumcarcinoembryonic antigen (CEA) concentra-tions. The case was unique because of the loca-tion of the tumour, which led to an initial diag-nosis of ovarian cancer.

Case reportA 75 year old Japanese woman (gravida 6, para6) underwent a medical check up that identi-fied raised serum CEA. Two months later, shevisited a women's clinic and was referred to ourhospital with a putative diagnosis of right ovar-ian cancer. Laboratory data including CA125and CA19-9 were all within normal limits,except for a raised value for CEA (17.7 ng/ml;normal range 0-2.5). While there was no masspalpable on the abdomen, physical examin-ation of the pelvis revealed a sausage shapedmass in the right adnexal region measuring8 cm in length. There was no lymphadenopa-thy and cytological examination of the cervixand vagina showed no malignancy. Computedtomography (CT) and ultrasonography of theabdomen and pelvis demonstrated an ellipticalcystic mass measuring 9 cm in length on theright anterior side of the uterus (fig 1). Bariumenema examination showed no abnormalitieswithin the colorectum.Laparotomy was performed because of pos-

sible right ovarian cancer. During the opera-tion, no ascites was noted. Operative findings

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