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Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 443-445
Isolated malakoplakia of inguinal lymph node: A rare case report

Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

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Date of Web Publication18-Jan-2014


Malakoplakia is rare chronic inflammatory disorder which commonly affects urinary tract. Though it has been reported in several sites outside the urinary tract, isolated lympnode involvement is extremely uncommon. Herein we present a case of 20 year old male with right inguinal lymphnodal mass. Histological findings including special stains and immunohistochemistry findings were characteristic of malakoplakia. This case is being presented to create awareness for inclusion of this entity in the differential diagnosis of lymphadenopathy.

Keywords: Lymph node, malakoplakia, M-G bodies

How to cite this article:
Khera R, Narla S, Uppin SG, Uppin MS, Paul RT. Isolated malakoplakia of inguinal lymph node: A rare case report. Indian J Pathol Microbiol 2013;56:443-5

How to cite this URL:
Khera R, Narla S, Uppin SG, Uppin MS, Paul RT. Isolated malakoplakia of inguinal lymph node: A rare case report. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Sep 23];56:443-5. Available from: http://www.ijpmonline.org/text.asp?2013/56/4/443/125365

   Introduction Top

Malakoplakia is a rare chronic inflammatory disorder first described by Michaelis and Gutmann in 1902. [1] Most common site affected is the urinary tract. The other reported sites include gastrointestinal tract, central nervous system, female genital tract and the tongue. [2] Isolated lymph node involvement by malakoplakia is extremely uncommon. We here report a case of malakoplakia of inguinal lymph node.

   Case Report Top

A 20 year-old male presented with right inguinal swelling of 4 months duration. On examination, it was soft, mobile, non-tender mass measuring 5 × 5 cm. He did not have any other complaints. There was no evidence of any other lymphadenopathy, hepatomegaly or splenomegaly. Routine hematological and biochemical investigations were within normal limits. In the past he had suffered from multiple abscesses in the perineal and gluteal region, for which he was treated with antibiotics. The excision biopsy of the inguinal mass was sent for histopathological examination.

   Pathologic Findings Top

The mass measured 4 × 4 × 2 cm. Cut surface was solid, grey white with brownish areas. Histopathological examination of the mass revealed an enlarged lymph node with partially effaced architecture. Majority of the lymph node was replaced by sheets of histiocytes with peripheral areas showing residual normal lymphoid tissue [Figure 1]a . These histiocytes had abundant eosinophilic granular cytoplasm, round vesicular nuclei and micro nucleoli [Figure 1]b. There were admixed inflammatory cells, including lymphocytes, plasma cells, neutrophils and few eosinophils. In addition there were numerous extracellular and intracellular faintly basophilic round bodies [Figure 1]c. Some of these had targetoid appearance. These were highlighted by Von Kossa, PAS and Perl's stain [Figure 2]a,b,c and were identified as Michaelis Guttmann bodies (MG bodies). Stains for fungal organisms and acid-fast bacilli were negative. Immunohistochemistry with CD68 was used to confirm histiocytic nature of the lesion [Figure 3]. A histopathological diagnosis of Malakoplakia was made. He was started on antibiotics and is on follow-up.

   Discussion Top

Malakoplakia is an inflammatory disease with disputed etiology and pathogenesis. [3] The urinary tract is the most commonly involved site. Wielenberg et al.[4] in a review of 153 cases, found 89 cases (58%) that involved the urinary tract, out of which 63 (40%) occurred in the urinary bladder. The first description of malakoplakia outside the urinary tract was in 1958. [5] There are now accumulating reports of malakoplakia occurring in a wide variety of organs other than the urinary tract, most commonly in the gastrointestinal system, retroperitoneum and less common in lungs, bones, mesenteric lymphatic nodules, medium ear, larynx, palatine tonsil, parotid gland, temporal bone, neck and tongue. The age of presentation varies between 6 weeks of life and 85 years, being more frequent in adults than in children. Lymph node involvement in malakoplakia has been reported as a regional spread from malakoplakia of bladder [6] and pancreas. [7] Concurrent involvement of lymph node by malakoplakia has also been described in case of colonic [8] and prostate [9] adenocarcinoma. Our patient presented with isolated involvement of inguinal lymph node mass at 20 years of age without any lesion in genitourinary or gastrointestinal tract. Malakoplakia of lymph node as the primary site has rarely been reported in the literature before. The pathogenesis of lymph nodal involvement in malakoplakia remains to be elucidated. There has been a substantial body of evidence implicating the role of an infectious agent in the pathogenesis of malakoplakia. It is hypothesized that there is a defect in the host intracellular bactericidal function, leading to an accumulation of incompetent macrophages along with intracellular and extracellular bacterial residues. The most commonly associated organisms are gram-negative bacilli including, most frequently, E. coli, Proteus mirabilis, and Klebsiella pneumoniae. It may be suggested that micro-organisms phagocytosed by the macrophages may either remain viable or partially digested in these cells, because of defective phagocytic digestion. These bacteria-laden histiocytes may remain at the site of implantation or enter into the lymphatic system, eventually localizing to regional lymph nodes. Therefore, the mechanism may be similar to lymph-node involvement in other infective processes such as tuberculosis and Whipple's disease.
Figure 1: a) Histological section of lymph node showing partial eff acement of architecture due to infi ltrate of histi ocytes (H and E stain; ×40). b) Sheets of histi ocytes with abundant granular eosinophilic cytoplasm (H and E stain; ×100). c) Numerous extracellular and intracellular faintly basophilic round MG bodies (H and E stain; ×400).

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Figure 2: MG bodies showing positi ve staining with a) von Kossa, b) PAS and C) Perl's stain (×400)

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Figure 3: Histi ocytes showing diff use strong cytoplasmic positi vity with CD68 (HRP-Polymer; ×100)

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The diagnostic feature of malakoplakia is the microscopic appearance of MG bodies, which are round to oval in form and can be seen both intra and extracellularly. [10],[11] They form from the fusion of persistent phagolysosomes in macrophages and their associated calcification. Their composition is 94.6% organic and 5.4% inorganic; the inorganic components include calcium, phosphorus and iron. MG bodies stain positive with periodic acid-schiff reagent, von Kossa's reaction for calcium and Perl's ferrocyanide reaction to ferric iron. The abnormal function of macrophages in digesting bacteria is attributed to low levels of intracellular cyclic guanosine monophosphate and diminished release of β-glucuronidase. [12] It is interesting to note that such conditions are often associated with a generalized disease capable of altering the immune mechanism. Since our patient had past history of multiple perineal and gluteal abscesses, a possible etiologic relation to these may be considered.

Malakoplakia is generally considered a chronic, self-limiting inflammatory disease that may undergo spontaneous regression but, depending on the underlying process and organ involved, may be associated with significant risk of mortality. [7] Management of malakoplakia requires a combination of surgical and medical therapies. Antimicrobials such as fluoroquinolones are particularly effective because of their intramacrophagic penetration, and ciprofloxacin is the drug of choice. [13] Macrophage function can also be improved by the use of a cholinergic agent, such as bethanechol chloride. Surgical management is curative in almost all these cases as there is no reported evidence of malignant potential. Our patient was treated with antibiotics following surgical excision of mass and is on follow-up.

Although rare, malakoplakia can be a cause of lymphadenopathy. Awareness of such an occurrence is essential for diagnosis and proper management.

   References Top

1.Michaelis L, Gutmann C. Einschlusse in Blastentumoren. Z Klin Med 1902;47:208-15.  Back to cited text no. 1
2.George MY, Bibi N. Malakoplakia outside the urinary tract. Arch Pathol Lab Med 2007;131:297-300.  Back to cited text no. 2
3.Chaudhry AP, Satchidanand SK, Anthone R, Baumler RA, Gaeta JF. An unusual case of supraclavicular and colonic malakoplakia - A light and ultrastructural study. J Pathol 1980;131:193-208.  Back to cited text no. 3
4.Wielenberg AJ, Demos TC, Rangachari B, Turk T. Malacoplakia presenting as a solitary renal mass. AJR Am J Roentgenol 2004;183:1703-5.  Back to cited text no. 4
5.Scott EV, Scott WF Jr. A fatal case of malakoplakia of the urinary tract. J Urol 1958;79:52-6.  Back to cited text no. 5
6.PozoMengual B, Burgos Revilla FJ, BrionesMardones G, Linares Quevedo A, García-Cosio Piqueras M. Bladder malacoplakia with lymphatic involvement and an aggressive course. Actas Urol Esp 2003;27:159-63.  Back to cited text no. 6
7.Nuciforo PG, Moneghini L, Braidotti P, Castoldi L, De Rai P, Bosari S. Malakoplakia of the pancreas with diffuse lymph-node involvement. Virchows Arch 2003;442:82-5.  Back to cited text no. 7
8.Gidwani AL, Gidwani SA, Khan A, Carson JG. Concurrent malakoplakia of cervical lymph nodes and prostatic adenocarcinoma with bony metastasis: Case report. Ghana Med J 2006;40:151-3.  Back to cited text no. 8
9.McClure J. Malakoplakia of the gastrointestinal tract. Postgrad Med J 1981;57:95-103.  Back to cited text no. 9
10.Kumar V, Coady MS. Malakoplakia of the neck in an immunocompromised patient. Plast Reconstr Surg 2005;116:125-7e.  Back to cited text no. 10
11.McClure J. Malakoplakia. J Pathol 1983;140:275-330.  Back to cited text no. 11
12.Govender D, Ahmed SE. Malakoplakia and tuberculosis. Pathology 1999;31:280-3.  Back to cited text no. 12
13.Schmerber S, Lantuejoul S, Lavieille JP, Reyt E. Malakoplakia of the neck. Arch Otolarngol Head Neck Surg 2003;129:1240-2.  Back to cited text no. 13

Correspondence Address:
Shantveer G Uppin
Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad - 500 082, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.125365

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  [Figure 1], [Figure 2], [Figure 3]

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