| Abstract|| |
Malakoplakia of the gastrointestinal tract is a rare chronic inflammatory disorder, usually affecting the descending colon, sigmoid colon and rectum. It is commonly seen in adults. Only few cases have been reported in children. We report a case of malakoplakia of colon and rectum in a 7-year-old child who presented with multiple polyposis coli.
Keywords: Children, colon, malakoplakia, polyposis, rectum
|How to cite this article:|
Jadhav MN, Patil RK, Kittur SK, yadav J. Colorectal malakoplakia in a child presenting as multiple polyposis coli. Indian J Pathol Microbiol 2015;58:86-8
|How to cite this URL:|
Jadhav MN, Patil RK, Kittur SK, yadav J. Colorectal malakoplakia in a child presenting as multiple polyposis coli. Indian J Pathol Microbiol [serial online] 2015 [cited 2020 Apr 9];58:86-8. Available from: http://www.ijpmonline.org/text.asp?2015/58/1/86/151197
| Introduction|| |
Malakoplakia is a rare chronic granulomatous disorder of uncertain etiology.  Though commonly seen in the urinary tract, it is also reported in other sites such as gastrointestinal tract, pancreas, liver, lymph node, skin, respiratory tract, vagina, and skin.  It was first described by Michaelis-Gutmann in 1902. One year later von Hansman coined the term "Malakoplakia" derived from the Greek word "malakos" (soft) and "plakos" (plaque). 
Gastrointestinal malakoplakia has been reported in people with wide age spectrum from 6 weeks to 88 years with an average age being 47.54 years.  It is rare in the pediatric age group. , Only few cases are reported in the literature presenting as multiple polyps involving colon and rectum.
| Case Report|| |
A 7-year-old male child was admitted to the hospital with a history of intermittent watery diarrhea, bleeding from the rectum and weight loss of 7 months duration. He also complained of mass protruding from the rectum while passing stools for the past 1-month. He had jaundice 1-year back. History revealed that the patient had a colonic biopsy done earlier in a private hospital for similar complaints, which was reported as an adenomatous polyp of the colon. On physical examination, the child was poorly built, cachectic with mild hepatomegaly. Five tiny polyps were felt on rectal examination.
Hematological investigations showed microcytic hypochromic anemia. Stool microscopy revealed plenty of red blood cells. Chest X-ray was unremarkable. Liver function tests were within the normal limits. Mild hepatomegaly was detected on abdominal ultrasound. Barium enema showed numerous polypoid filling defects in the colon.
Videocolonoscopy revealed multiple polyps scattered all over the large intestine [[Figure 1]a and b]. Proctocolectomy with ileoanal anastomosis was done.
|Figure 1: Barium enema showing multiple polypoid filling defects in the colon (a), Videocolonoscopy showing polyps in descending colon (b), Proctocolectomy specimen showing multiple polyps (c)|
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The resected specimen consisted of terminal ileum, appendix, cecum, colon and rectum measuring 38 cm in length. The serosa was unremarkable. The mucosal surface showed 35 pale brown sessile polyps varying from 0.5 cm to 3 cm in diameter unevenly distributed throughout the large intestine, with intervening thickened and thinned out areas [[Figure 1]c].
Microscopy from all the polyps and thickened wall of the colon and rectum showed ulcerated mucosa and submucosa densely infiltrated by sheets of histiocytes, lymphocytes, plasma cells and few eosinophils. The histiocytes had abundant granular eosinophilic cytoplasm. Numerous rounded densely basophilic, laminated, refractile, targetoid Michaelis-Gutmann bodies (M-G bodies) were seen both inside the histiocytes and extracellularly. These bodies were positive for periodic acid Schiff (diastase resistant), von Kossa and Perls' Prussian blue stains [Figure 2].The cytoplasmic granules were also highlighted by periodic acid Schiff stain. None of the polyps showed adenomatous features. The muscularis propria, serosa, mesocolon and the draining lymph nodes were unremarkable. Gram and Ziehl-Neelsen stains did not show any bacteria. A diagnosis of malakoplakia involving colon and rectum was made. The child was put on trimethoprim-sulfomethoxazole and ascorbic acid for 6 months, but he expired 8 months later due to postoperative complications.
|Figure 2: Microphotograph showing colonic mucosa (inset) (H and E, ×100) with histiocytes containing Michaelis – Gutmann bodies (H and E, ×1000) (a), PAS (b), Perls' Prussian blue (c) Von Kossa (×400) (d)|
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| Discussion|| |
Gastrointestinal tract is the second most common site of involvement by malakoplakia. ,, It usually involves the descending colon, sigmoid colon and rectum.  Endoscopically it appears in three different patterns:
- Unifocal mucosal nodules and plaques.
- Multifocal mucosal nodules and polyps.
- Large mass lesions. ,
Colonic malakoplakia was first described by Terner and Lattes in 1965.  McClure made a comprehensive review of the world literature on 34 cases of gastrointestinal malakoplakia.  In his study, the entire colon was involved only in two cases, and polyps were described in six cases. In the present case, the entire colon and rectum were involved by multiple polyps.
Gastrointestinal malakoplakia in childhood is often associated with immunocompromised states, tuberculosis, myeloid leukemia,  and celiac disease,  unlike in adults where it frequently coexists with colonic adenocarcinoma. , It is also reported in previously healthy children. , In our case, there were no associated lesions.
Malakoplakia is a histologic diagnosis characterized by aggregates of macrophages called 'von Hansman' cells with distinct M-G bodies, that are pathognomonic, but not necessary for diagnosis. , These bodies are positive for Perls' Prussian blue and von Kossa stains, as they are coated with calcium and iron salts. Periodic acid Schiff stains both M-G bodies and cytoplasmic granules.  Ultrastructural evidence suggests that these cells contain phagolysosomal inclusions comprising vesicles, electron dense bodies, membranous lamellae and the crystalline M-G bodies.  Disintegrated bacteria have been occasionally observed. 
Although the exact cause of malakoplakia is not clear, three possible mechanisms have been suggested:
- Tole of bacteria like Escherichia More Details coli, Mycobacterium tuberculosis, Proteus mirabilis and Staphylococcus aureus.
- Abnormal or altered immune response.
- Abnormal macrophage response because of defective lysosomal function. ,
This is due to guanosine monophosphate dehydrogenase and beta-glucuronidase deficiency which alters the microtubular and lysosomal function leading to incomplete elimination of bacteria from macrophages.  Current evidence points to macrophage killing activity. , In the present case the Gram and Ziehl-Neelsen stains failed to reveal any bacteria. However, complete workup of immunological status was not possible.
The early stages of malakoplakia should be differentiated from Whipple disease, Chediak-Higashi syndrome, tuberculosis, histiocyctic storage disorders, sarcoidosis and fungal infections.  In Whipple disease, the cytoplasmic granules are Sudan black positive whereas they are negative in malakoplakia. Chediak-Higashi syndrome can be differentiated by the presence of giant cytoplasmic granules. In sarcoidosis, Schaumann bodies in giant cells may give a clue to the diagnosis.  Special stains may help in excluding tuberculosis and fungal infections. In conclusion, malakoplakia must be considered in the differential diagnosis in children presenting with chronic diarrhea and bleeding from the rectum in appropriate clinical setting so that unnecessary radical surgery can be avoided.
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Dr. Meena N Jadhav
Flat No. T-34, Sharadha Residency, Sampige Road, V. V. Nagar, Belgaum - 590 001, Karnataka
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]