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Malacoplakia of liver: A case report
HUMAN PATHOLOGY
MALACOPLAKIA
OF LIVER:
A CASE
Volume 22, No. 12 (December
1991)
REPORT
S. J. ROBEKTSON, MD, R. B. HIGGINS, MD, ANI) C. POWELL, MSc
This is theJirst reported co..sein the English literature ~J‘mala~oplukia In common with other cuses qf malacoplakia, the patient was immunosuppressed seco&ary to steroid therapy jtifbrunderlying systemic lupus erythematosus. The patient died 18 months after presentation; at autopsy, the histologic feutures included those of the late or jbrous stage of malacoplakia. HUM PATHOL 22:12941295. Copyright 0 1991 by W.B. Saunders Comparly
ofthe liver.
Malacoplakia is an unusual inflammatory condition with a characteristic histologic appearance. It was first described by Michaelis and Gutmann in 1902l and further characterized by Von Hansemann in 1903.’ Originally described in the bladder, it was most commonly found in the urinary tract. Other locations included the testis, prostate, epididymis, gastrointestinal tract, retroperitoneum, bone, adrenal glands, brain, pancreas, tonsils, conjunctiva, joints, spleen, lungs, larynx, vagina, and skin.smg This is, however, the first case reported in the English literature with involvement of the liver. One previous case has been reported in the Russian literature.‘” However, rather than being spontaneous, the latter case was associated with an abdominal wound with liver damage. REPORT
FIGURE 2. Electron microscopy of a biopsy specimen showing Michaelis-Gutmann bodies. (Magnification X6,000.)
OF A CASE
A 54year-old woman developed systemic lupus erythematosus with symptoms of arthritis, skin lesions, and thrombocytopenia. She was treated first with Imuran (azathioprine; Burroughs Wellcome Inc, Kirklond, Canada) and later, with steroids. Two years afterward, the liver was noted to be enlarged and a liver scan identified a lesion in the right lobe. The clinical impression was that of abscess or metastatic tumor.
FIGURE 1. Biopsy specimens of the liver showing sheets of histiocytes. Many show characteristic Michaelis-Gutmann bodies. (Hematoxylin-eosin stain: magnification x40.)
From the Department of Pathology, Ottawa General Hospital. Ottawa, Ontario, Canada; and the Department of Surgery, StJoseph’s Hospital, St John, New Brunswick, Canada. Accepted for publication February 5, 1991. Keyword.~: malacoplakia. liver, ultrastructure. irnmunohistochetu-istry. Address correspondence and reprint requests to S. J. Robertson, MD, Department of Pathology, Ottawa General Hospital, 501 Smyth Rd, Ottawa. Ontario. Canada KlH 8L6. Copyright 0 1991 by W.B. Saunders Company 0046~8177/91/2212-0017$5.00/0
1294
The patient underwent laparotomy ture. Bacteriologic culture yielded
with liver biopsy and culE.tcherichia coli and Acinr-
tobacter calcoaceticus.
Histologically, an inflammatory process was identified consisting mainly of histiocytes with scattered plasma cells and lymphocytes. Other areas showed pockets of polymorphonuclear leukocytes. Numerous cytoplasmic inclusion bodies were noted within the histiocytes. These had the characteristic features of Michaelis-Cutmann bodies and they stained positively with periodic acid-Schiff stain and VonKossa stain for calcium. The histiocytes showed positive staining with immunoperoxidase for cu-l-antitrypsin and lysozyme. Electron microscopy showed Michaelis-Gutmann bodies, but intracellular bacteria were not identified. The diagnosis of tnalacoplakia was made (Figs 1 and 2). Treatment included discontinuing steroids and instituting antibiotic therapy. A drainage tube was left in place. The patient gradually recovered, but 1 year later developed an autoimmune hemolytic anemia that necessitated reinstituting steroid therapy. A splenectomy was also done. One month after the splenectomy, the patient developed a liver abscess with a retroperitoneal tract over the right iliac crest to the right buttock. This was incised and drained. Bacteriologic culture yielded a growth of B roli and Enterobacter cloacae. The patient was discharged from the hospital, but returned 6 months later with abdominal pain. A laparotomy was performed, but no evidence of abscess formation was present. A cholccystectomy was done at that time. Postoperatively. the patient developed septic shock and died 18 months after the initial diagnosis of malacoplakia. Autopsy showed intra-abdominal hemorrhage from the gallbladder bed, small abscesses of liver, and a fibrotic area in the right lobe of the liver. Microscopically. the liver showed fibroblasts and abundant collagen with scattered inflammatory cells and small pockets of histiocytes. Michaelis-Gutmann bodies were identified, but most were intensely basophilic and larger than on the original biopsy. Many of these were found in an extracellular location. These features correspond to those described as the late or fibrosed stage of malacoplakia” (Fig 3).
CASE STUDIES
FIGURE 3. Autopsy specimen showing abundant collagen with scattered histiocytes and Michaelis-Guimann bodies seen frequently In an extracellular location. (Hematoxylin-eosin stain; magnification X400.)
l)Is(:I’ssil(
)N
III SIIIII~LII-1. Ihih is lhc host case of ndacoplakia of lhc Ilvrr wpo13rd in (hr Euglish literature. 1_1istolols\ al IIres~n~,ition was tliaguostic. As in previously rrportrti c;ws. thrrc was an elcmen~ 01’ imrrlunosuppl-ession in rhe host. and batleriologic culture vickled 1:‘coli ;ts well as other- gram-negative Imcilli. Llnfor-runairly. the patient’s underlying sysrmiic lupus crvrhematosus nrcessk~ted I-einstitution of‘ steroids and thr Iklienl suhsrqurntly died. At the time of autopsy. I8 months ,tftrr initid Dresentation, the histolopic features in the liver
FATAL PULMONARY ARTERIAL 9-MONTH-OLD INFANT 1.bi H~.NII’R, INI)
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CHEMOTHERAPY
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MALACOPLAKIA
OF LIVER:
A CASE
Volume 22, No. 12 (December
1991)
REPORT
S. J. ROBEKTSON, MD, R. B. HIGGINS, MD, ANI) C. POWELL, MSc
This is theJirst reported co..sein the English literature ~J‘mala~oplukia In common with other cuses qf malacoplakia, the patient was immunosuppressed seco&ary to steroid therapy jtifbrunderlying systemic lupus erythematosus. The patient died 18 months after presentation; at autopsy, the histologic feutures included those of the late or jbrous stage of malacoplakia. HUM PATHOL 22:12941295. Copyright 0 1991 by W.B. Saunders Comparly
ofthe liver.
Malacoplakia is an unusual inflammatory condition with a characteristic histologic appearance. It was first described by Michaelis and Gutmann in 1902l and further characterized by Von Hansemann in 1903.’ Originally described in the bladder, it was most commonly found in the urinary tract. Other locations included the testis, prostate, epididymis, gastrointestinal tract, retroperitoneum, bone, adrenal glands, brain, pancreas, tonsils, conjunctiva, joints, spleen, lungs, larynx, vagina, and skin.smg This is, however, the first case reported in the English literature with involvement of the liver. One previous case has been reported in the Russian literature.‘” However, rather than being spontaneous, the latter case was associated with an abdominal wound with liver damage. REPORT
FIGURE 2. Electron microscopy of a biopsy specimen showing Michaelis-Gutmann bodies. (Magnification X6,000.)
OF A CASE
A 54year-old woman developed systemic lupus erythematosus with symptoms of arthritis, skin lesions, and thrombocytopenia. She was treated first with Imuran (azathioprine; Burroughs Wellcome Inc, Kirklond, Canada) and later, with steroids. Two years afterward, the liver was noted to be enlarged and a liver scan identified a lesion in the right lobe. The clinical impression was that of abscess or metastatic tumor.
FIGURE 1. Biopsy specimens of the liver showing sheets of histiocytes. Many show characteristic Michaelis-Gutmann bodies. (Hematoxylin-eosin stain: magnification x40.)
From the Department of Pathology, Ottawa General Hospital. Ottawa, Ontario, Canada; and the Department of Surgery, StJoseph’s Hospital, St John, New Brunswick, Canada. Accepted for publication February 5, 1991. Keyword.~: malacoplakia. liver, ultrastructure. irnmunohistochetu-istry. Address correspondence and reprint requests to S. J. Robertson, MD, Department of Pathology, Ottawa General Hospital, 501 Smyth Rd, Ottawa. Ontario. Canada KlH 8L6. Copyright 0 1991 by W.B. Saunders Company 0046~8177/91/2212-0017$5.00/0
1294
The patient underwent laparotomy ture. Bacteriologic culture yielded
with liver biopsy and culE.tcherichia coli and Acinr-
tobacter calcoaceticus.
Histologically, an inflammatory process was identified consisting mainly of histiocytes with scattered plasma cells and lymphocytes. Other areas showed pockets of polymorphonuclear leukocytes. Numerous cytoplasmic inclusion bodies were noted within the histiocytes. These had the characteristic features of Michaelis-Cutmann bodies and they stained positively with periodic acid-Schiff stain and VonKossa stain for calcium. The histiocytes showed positive staining with immunoperoxidase for cu-l-antitrypsin and lysozyme. Electron microscopy showed Michaelis-Gutmann bodies, but intracellular bacteria were not identified. The diagnosis of tnalacoplakia was made (Figs 1 and 2). Treatment included discontinuing steroids and instituting antibiotic therapy. A drainage tube was left in place. The patient gradually recovered, but 1 year later developed an autoimmune hemolytic anemia that necessitated reinstituting steroid therapy. A splenectomy was also done. One month after the splenectomy, the patient developed a liver abscess with a retroperitoneal tract over the right iliac crest to the right buttock. This was incised and drained. Bacteriologic culture yielded a growth of B roli and Enterobacter cloacae. The patient was discharged from the hospital, but returned 6 months later with abdominal pain. A laparotomy was performed, but no evidence of abscess formation was present. A cholccystectomy was done at that time. Postoperatively. the patient developed septic shock and died 18 months after the initial diagnosis of malacoplakia. Autopsy showed intra-abdominal hemorrhage from the gallbladder bed, small abscesses of liver, and a fibrotic area in the right lobe of the liver. Microscopically. the liver showed fibroblasts and abundant collagen with scattered inflammatory cells and small pockets of histiocytes. Michaelis-Gutmann bodies were identified, but most were intensely basophilic and larger than on the original biopsy. Many of these were found in an extracellular location. These features correspond to those described as the late or fibrosed stage of malacoplakia” (Fig 3).
CASE STUDIES
FIGURE 3. Autopsy specimen showing abundant collagen with scattered histiocytes and Michaelis-Guimann bodies seen frequently In an extracellular location. (Hematoxylin-eosin stain; magnification X400.)
l)Is(:I’ssil(
)N
III SIIIII~LII-1. Ihih is lhc host case of ndacoplakia of lhc Ilvrr wpo13rd in (hr Euglish literature. 1_1istolols\ al IIres~n~,ition was tliaguostic. As in previously rrportrti c;ws. thrrc was an elcmen~ 01’ imrrlunosuppl-ession in rhe host. and batleriologic culture vickled 1:‘coli ;ts well as other- gram-negative Imcilli. Llnfor-runairly. the patient’s underlying sysrmiic lupus crvrhematosus nrcessk~ted I-einstitution of‘ steroids and thr Iklienl suhsrqurntly died. At the time of autopsy. I8 months ,tftrr initid Dresentation, the histolopic features in the liver
FATAL PULMONARY ARTERIAL 9-MONTH-OLD INFANT 1.bi H~.NII’R, INI)
bfb:wl)Ir~l
Ml),
(:AIHEKINI’
M. SII.\XR,
OCCLUSIVE
(:~:l.l.lhi\NE‘.
MB,
VASCULAR
19.1~ WII.SON,
DISEASE
VBKS,
MBBS, MS{:
1295
MAKh
FOLLOWING
(;K~F~R~.K(;,
CHEMOTHERAPY
RID. Hrlt.tl
O’~~~:OI~O\~I~.II.
IN A Ml).