| Abstract|| |
Diagnosis of malakoplakia presenting as gall bladder mass is a diagnostic dilemma faced by pathologists, radiologists, and surgeons. Malakoplakia is a rare inflammatory disorder and tumor mimicker usually occurring in the urinary tract, may occasionally be found in gall bladder. Here, we present a rare case, presenting as gall bladder mass in a known case of gallstone disease, clinically suspected as carcinoma and later turned out to be malakoplakia in gall bladder.
Keywords: Carcinoma gall bladder, gall bladder mass, Malakoplakia, Michaelis–Gutmann bodies, Perls' stain, xanthogranulomatous cholecystitis
|How to cite this article:|
Singh K, Chatterjee T. A case of gallbladder mass: Malakoplakia (The tumor mimicker). Indian J Pathol Microbiol 2017;60:122-4
| Introduction|| |
The histopathological examination (HPE) of gall bladder sometimes reveal unique and interesting findings, thereby leading to change in entire treatment profile and thus making HPE must for all specimens. Here we present a rare case, presenting as gall bladder mass in a known case of gallstone disease, clinically suspected as carcinoma and later turned out to be malakoplakia in gall bladder.
| Case Report|| |
A 65 year-old nondiabetic male patient, known case of gallstone disease presented to the surgery outpatient department (OPD), Command Hospital, Udhampur, Jammu and Kashmir (India) with complaints of right-sided abdominal pain and vomiting. Hemogram, liver function tests, and renal function tests were within normal limits. Serum amylase and serum lipase were within normal limits. Signs and symptoms of acute pancreatitis were negative. Ultrasonography whole abdomen revealed multiple stones and contracted gall bladder with wall thickening predominantly in body and absence of pancreatic and peripancreatic tissue edema. Magnetic resonance cholangiopancreatography abdomen showed contracted gall bladder with focal thickening in body of gall bladder. Considering the possibility of gall bladder mass with differential diagnosis as carcinoma gall bladder and xanthogranulomatous cholecystitis, diagnostic laparoscopy was performed which revealed thickened and contracted gall bladder, impacted calculi, and densely adherent common bile duct.
With clinical diagnosis of carcinoma gall bladder, open cholecystectomy was undertaken, and specimens were received in the form of gall bladder with liver wedge, common bile duct margin, hepatoduodenal ligament and retro portal tissue, in the histopathology laboratory. Grossly, gall bladder specimen revealed thickened wall, obliteration of the cavity by diffuse nodules with yellowish discoloration predominantly in body of gall bladder and impacted black colored stones [Figure 1]a.
|Figure 1: (a) Gross picture of gall bladder with liver wedge showing deposition of yellowish nodules. (b) Histopathological examination showing diffuse sheets of foamy histiocytes and giant cells in gallbladder wall (H and E, ×20); inset showing cytoplasmic inclusions in few histiocytes|
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HPE was done after staining the paraffin blocks with hematoxylin and eosin, and then special stain in the form of Perls' stain was done. HPE showed diffuse sheets of foamy histiocytes with few histiocytes containing inclusions [Figure 1]b. Foci of giant cells and chronic mononuclear inflammatory infiltrate noted in wall of the gall bladder. Perls' stain (for iron deposits) revealed Michaelis–Gutmann bodies in cytoplasm of histiocytes, which are pathognomic of malakoplakia [Figure 2]a. Wall of liver was free from this pathological process as evident by the absence of foamy histiocytes and Michaelis–Gutmann bodies on microscopic examination [Figure 2]b. There was the absence of transmural lymphoplasmacytic inflammatory infiltrates, extramural inflammatory nodules and tissue eosinophilia. No evidence of dysplasia/malignancy and no caseating epithelioid granulomatous pathology were noted in all the specimens. Within 2 weeks of hospitalization, the patient recovered well and was discharged to home. The patient is on regular follow-up since the last 6 months in surgery OPD and has no fresh complaints.
|Figure 2: (a) Michaelis–Gutmann bodies in cytoplasm of histiocytes (Perls', ×40). (b) Wall of liver was free from this pathological process as evident by absence of foamy histiocytes (H and E, ×20)|
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| Discussion|| |
Malakoplakia is not only a rare chronic granulomatous disorder first defined in 1902 by Michaelis and Gutman  most commonly affecting the urinary system  but also affects multiple body organs for example gastrointestinal tract, genital tract, central nervous system, skin, and hepato-biliary tract. Malakoplakia is often an incidental finding in routine specimens outside the genitor-urinary system  for example in cholecystectomy specimens, but being a great tumor mimicker, it can present as mass lesion, as was in our case. The diagnostic laparoscopic findings in our case were so suspicious of malignancy that the surgical procedure was changed to open cholecystectomy. In other reported cases also, the suspicion of malignancy led to subsequent open cholecystectomy. Etiopathogenesis has not been fully elucidated, but the possible mechanisms that have been suggested include role of microorganisms (particularly Escherichia More Details coli, Mycobacterium tuberculosis and Staphylococcus aureus), an abnormal altered immune response and an abnormal macrophage response because of defective lysosomal function. It is suggested that macrophages in the malakoplakia are capable of phagocytosis but unable to digest and clear the bacteria. Partially digested bacteria accumulates in histiocytes or macrophages and lead to the deposition of iron and calcium, resulting in accumulation of inclusion-like structures in cytoplasm of histiocytes, the Michaelis–Gutmann bodies which are considered to be pathognomic of malakoplakia. Malakoplakia is reported commonly in diabetics, as this condition is associated with defective phagocytic activity, however, in our case patient, was nondiabetic male which explains the possibility of multifactorial pathogenesis of this disorder. Xanthogranulomatous cholecystitis and malakoplakia can present in a similar fashion and are thought to be part of spectrum of chronic inflammatory pathology, with difference being malakoplakia being more aggressive and shows the presence of both intracellular and extracellular Michaelis–Gutmann bodies as in our case. Although malakoplakia is considered benign disease requiring local excision but many cases showed aggressive course with local recurrence and fistulization and failure to respond to antibiotics., Gallbladder involvement in autoimmune pancreatitis as a part of IgG4-associated systemic disease is also common. In our case, this was excluded by lack of characteristic laboratory investigations and by the absence of characteristic histopathological findings of this disease.
It is to be noted that malakoplakia is known to coexist with other lesions such as carcinoma and tuberculosis, however in our case, tissue was adequately sampled and examined histopathologically to rule out other lesions, especially carcinoma gall bladder and xanthogranulomatous cholecystitis.
| Conclusion|| |
The conclusion of this case report is to stress upon differentiating malakoplakia-the tumor mimicker from carcinoma gall bladder, possibility of coexistence of malakoplakia with malignancy and tuberculosis and to distinguish it from more common and less aggressive xanthogranulomatous cholecystitis, as this will change the treatment modalities, prognosis and follow-up of the patient.
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Conflicts of interest
There are no conflicts of interest.
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Dr. Kanwaljeet Singh
Department of Pathology, Command Hospital, Udhampur, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]