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Malakoplakia of the Gastrointestinal Tract JOHN MCCLURE B.Sc., M.D., M.R.C.Path., D.M.J.Path

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Malakoplakia of the Gastrointestinal Tract JOHN MCCLURE B.Sc., M.D., M.R.C.Path., D.M.J.Path Postgrad Med J: first published as 10.1136/pgmj.57.664.95 on 1 February 1981. Downloaded from Postgraduate Medical Journal (February 1981) 57, 95-103 Malakoplakia of the gastrointestinal tract JOHN MCCLURE B.Sc., M.D., M.R.C.Path., D.M.J.Path. Division of Tissue Pathology, Institute ofMedical and Veterinary Science, Frome Road, Adelaide, South Australia, 5000 Summary sought medical aid for frequent loose bowel actions. The clinical and pathological features of 3 cases of There were no other symptoms and no significant colonic malakoplakia are documented thereby bringing past medical history. Sigmoidoscopy revealed an to 34 the total of recorded cases of malakoplakia ulcerated tumour 70 mm above the anus. A biopsy involving the gastrointestinal tract. This is therefore confirmed the presence of an adenocarcinoma of the most common site of involvement outside the colonic origin. Subsequently an abdomino-perineal urogenital tract. A comprehensive review of the world excision of the rectum was performed and the patient literature on gastrointestinal malakoplakia has been is alive and well 2 years after this operation. made and the characteristic features of the condition The resected specimen was a segment of rectum have been delineated. There was a bimodal age and anus 320 mm in length. There was a fungating Protected by copyright. incidence with a small cluster of cases occurring in tumour 60 mm in diameter and 30 mm in height and childhood and associated with significant additional situated 100 mm from the distal margin. The tumour systemic disease. In the adult cases the average age extended into the bowel wall and there were numer- was 57 years with a slight excess of males. The most ous soft yellow abscess-like areas in the adjacent fat commonly involved part of the gastrointestinal tract and also in a separately submitted portion of pre- was the colon and colonic carcinoma was the most sacral tissue. common associated disease. Histologically, these latter areas were composed of large numbers of macrophages containing round Introduction laminated basophilic inclusions (Michaelis-Gutmann Malakoplakia is characterized microscopically by bodies). Lymphocytes, plasma cells and occasional the accumulation of tissue macrophages containing polymorphonuclear granulocytes were also present. unique intracytoplasmic calcified inclusion bodies. The tumour was an adenocarcinoma of moderate These are called Michaelis-Gutmann (MG) bodies differentiation, primary to the site and had deeply after the authors who originally described the con- invaded the muscle coats. The tumour was sur- http://pmj.bmj.com/ dition (Michaelis and Gutmann, 1902). The Michaelis rounded by a heavy acute on chronic inflammatory Gutmann body is a rounded structure and often infiltrate with background fibrosis and large areas exhibits a concentric-ringed or targetoid reaction composed of macrophages containing typical MG with a basic dye such as haematoxylin. bodies (Fig. 1). Malakoplakia most commonly affects the mucosa Tumour permeation of lymphatic channels was of the urinary bladder (Voight, 1958) but involve- not noted and the drainage lymph nodes showed a ment of structures outside the urogenital tract is sinus histiocytosis. Some of the nodal macrophages on September 30, 2021 by guest. being reported with increasing frequency. Such sites contained MG bodies (Fig. 2). include colon (Terner and Lattes, 1965), skin (Arul The yellow nodules present in fat adjacent to the and Emmerson, 1977), breast (DiLeo and Anastasi, colon and in the presacral tissue were composed of 1969), lungs and skeleton (Gupta, Schuster and aggregates of malakoplakic cells. Christian, 1972) and brain (Mirra, 1971). The finding of malakoplakia in association with Case 2 3 cases of colonic carcinoma prompted a description The second case was that of a 73-year-old man of these cases and a review of the available world who presented with a history of rectal bleeding literature on malakoplakic disease of the gastro- accompanied by some loss of appetite and a little intestinal tract. weight loss over a 3-month period. There was noth- ing of relevance in the past medical history. Rectal Case reports and pathological findings examination revealed a tumour on the left side and Case 1. This was a 79-year-old man who initially therefore an anterior resection with defunctioning 0032-5473/81/0200-0095 $02.00 © 1981 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.57.664.95 on 1 February 1981. Downloaded from 96 J. McClure ss zB. ;· t..3 . ··....w.~*.Wo....~....., , .'.,, .~-.jA.. .A.·o 4 *.j i.r3F '*·.'e' :. ·E..· s , ; .S,.'i. ...A', ....a.,9 .~i·:.. .,.. ...... Nl· AP~~~~~~~·:Ifi% Obsi· .pF~+ r 'r ~~~~^tN-*·r* N,P 4~~~~~I~" ~P~ I~ok--If Ng AI Am ··~*ri j·r, rja Protected by copyright. FIG. 1. Malakoplakic macrophages and acute inflammatory cells in proximity to infiltrating adenocarcinoma (top right) (HE, x 100). colostomy was performed. The postoperative course abdominal quadrant pain of 2 weeks' duration. Ad- was complicated by pulmonary embolism. The ditionally there was a 10- to 12-month history of patient survived this but died after one year as a vague malaise, anorexia and moderate weight loss. consequence of disseminated malignancy and an In the course of extensive investigation, a liver scan Escherichia coli bacillaemia (proved by blood revealed multiple areas of reduced isotope uptake culture). and a follow-up liver needle biopsy showed the The resected specimen was a portion of rectum presence of a moderately differentiated mucin- http://pmj.bmj.com/ and sigmoid colon 360 mm in length. Arising 30 mm secreting adenocarcinoma. A suggestion was made from the distal limit was a 40 mm in diameter that, inter alia, a likely primary site was the gastro- ulcerating tumour and 90 mm proximal to this there intestinal tract. Investigation of this failed to reveal was another polypoid tumour also 40 mm in dia- a primary tumour. meter. Microscopically both lesions were moderately Post-mortem examination revealed a small poly- differentiated adenocarcinomas of bowel origin. poid tumour of the rectum not associated with Both tumours had invaded deeply into the bowel stenosis. There were massive secondary deposits wall reaching the serosal surface. There was a in the liver and smaller metastases in the right lung. on September 30, 2021 by guest. marked stromal inflammatory reaction to each tum- There was also a small adenomatous polyp of the our with infiltrations of polymorphs, lymphocytes, sigmoid colon. Microscopically the rectal tumour plasma cells and striking numbers of macrophages was a moderately well differentiated mucin-secreting many of which contained typical MG bodies. The adenocarcinoma. It has penetrated the full thickness drainage lymph nodes in this case also showed a of the bowel wall to reach the serosal surface. In the sinus histiocytosis but the nodal macrophages did stroma of the tumour, there was an exuberant in- not contain MG bodies. flammatory infiltration (Fig. 3) by polymorphs, lymphocytes, plasma cells and numerous macro- Case 3 phages again containing MG bodies. The third case was that of a 59-year-old woman In the 3 cases, the MG bodies were uniformly who died 3 months after her initial admission to positive in reaction to the von Kossa (Fig. 4) and hospital. At that time she gave a history ofright upper alizarin red S technique. Many, but not all, gave Postgrad Med J: first published as 10.1136/pgmj.57.664.95 on 1 February 1981. 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N".# http://pmj.bmj.com/ 9..`6~ 3 on September 30, 2021 by guest. 40~~~~ ~~~~~~~~~~0 F ·1-·~iJCIA .9~~~~~0 FIG. 3. Malakoplakic macrophages admixed with acute inflammatory cells against a background of vascular granulation tissue (HE, x 200). Postgrad Med J: first published as 10.1136/pgmj.57.664.95 on 1 February 1981. Downloaded from 98 J. McClure .:,~ ,,,:: :!~:..:~, ,.,..~,~,, ,,:.~ ... .A...,,.~. ,.... -..,: .. .:...~?jisiii. 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