tion but continued to deteriorate clinically due to her long-term disabilities , which precluded her from receiving chemotherapy . She was discharged to inpatient hospice and passed away two months later .
DISCUSSION
Malakoplakia is a rare granulomatous disease in the urogenital tract as well as the gastrointestinal and nervous systems . It is a histiocytic inflammatory disease first reported in 1902 by Michaelis and Gutmann . 1 , 2 Etiology and treatment of malakoplakia have not been fully defined . Multiple cases have been associated with immunosuppression as a factor in the development of the disease . 3 , 4 , 5 Other researchers have reported its presence alongside irritable bowel disease 1 , 6 and in otherwise healthy patients . 3
Three mechanisms of the pathogenesis of malakoplakia are suggested . The first possible mechanism involves bacterial colonization with organisms such as Escherichia coli ( E . coli ) and Rhodococcus equi in patients given chemotherapy and those with acquired immunodeficiency virus , respectively . 6 The second possible mechanism involves an altered immune response in the setting of innate or acquired immune deficiency , which would be present in patients on chronic immune suppression , either for inflammatory diseases or post-transplant . The third possible mechanism involves a lysosomal deficiency causing an increase in abnormal macrophages , which cannot break down bacteria . Partially digested bacteria build up with calcium and iron to form the Michaelis-Gutmann bodies that are characteristic of malakoplakia . 6
Clinical presentation is highly variable and depends on the bodily system involved . In the gastrointestinal tract , it can be asymptomatic , or the patient can have abdominal pain , rectal bleeding , fever , constipation , or bowel obstruction . CT scans of malakoplakia reveal that it may have appearances likened to inflammatory processes , allowing for endoscopic evaluation in a stable patient . Diagnosis of malakoplakia , however , is often confirmed post-operatively from histopathologic examination of specimens with suspected adenocarcinomas . 2 Because there is no clear consensus on managing this condition , the treating surgeon is posed with several challenges .
Current treatments vary depending on the individual case . For example , antibiotics have been effective in healthy patients or those with E . coli infestation . There has also been evidence for discontinuing immunosuppression medications and treating with a choline agonist to correct the lysosomal defect . Ultimately , surgery can be a therapeutic option .
Malakoplakia associated with colon cancer is a rare entity , and its prognostic effect in a patient with colonic adenocarcinoma is unknown . When the disease is present in the gastrointestinal system , it is most seen in the descending colon , sigmoid , and rectum . To our knowledge , 19 cases of colonic malakoplakia have been reported in the literature over the last 10 years ( Table 1 ), with the total number of gastrointestinal-related malakoplakia cases at 28 in the same period . As with our case , association with immunosuppression is common . Our case presents unique challenges due to the presence of multiple factors implicated in the development of malakoplakia , such as immunosuppression , Crohn ’ s disease , and colonic adenocarcinoma . Our patient was immunosuppressed by mycophenolate and tacrolimus , and in keeping with the altered immune response theory for the pathogenesis of malakoplakia , she had a history of chronic steroid use for her liver transplant . 2 , 6 Having both Crohn ’ s disease and cancer could have increased the proliferation of bacteria , impaired the ability to digest it , and altered gut flora , thereby contributing to the development of malakoplakia . The patient ’ s pathology showed wide dispersion of the histiocytic cells in the resected tumor . Rather than being a peri-tumor occurrence , the cells made up the bulk of the mass resected from the ileum .
Historically , malakoplakia has been found along the borders of resected tumor specimens . 7 , 8 , 9 Matter et al suggested that the presence of malakoplakia along the border of a tumor can lead to a more advanced disease with a worsened prognosis . 7 This finding can affect treatment decisions , especially in elderly patients who may have compounding diseases . Surgical resection can also be offered , especially if complications arise related to the condition . 1
CONCLUSION
Malakoplakia is a rare granulomatous disease associated with immune deficiencies or immunosuppressive medications . No typical clinical presentation has been described , and treatment can be challenging . While other sites of malakoplakia presentation such as skin , peri-anal / perineal , site of traumatic injury , head , and neck may facilitate diagnostic tissue sampling , intra-abdominal malakoplakia remains a diagnostic challenge and is usually encountered in the final surgical specimen obtained . The combination of carcinoma , Crohn ’ s disease , and a liver transplant further complicates this case as the malakoplakia cannot be concretely attributed to any of the three factors . Thus , although colonic malakoplakia is a rare condition , it can pose a diagnostic and therapeutic dilemma of which the modern-day surgeon should be aware .
REFERENCES
1 . Yared RA , Badran HA , Kamareddine MH , et al . Colonic Malakoplakia : A Rare Finding in a Healthy Male . Case Rep Gastroenterol . 2018 ; 12 ( 2 ): 453 – 456 . Published 2018 Aug 21 . doi : 10.1159 / 000492208
2 . Bates AW , Dev S , Baithun SI . Malakoplakia and colorectal adenocarcinoma . Postgrad Med J . 1997 ; 73 : 171-3 .
3 . Govender D , Essa AS . Malakoplakia and tuberculosis . Pathology . 1999 ; 31 : 280-283 .
4 . Shin MS , Cooper JA Jr , Ho KJ . Pulmonary malacoplakia associated with infection in a patient with AIDS . Chest . 1999 ; 115 : 889-892 .
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