Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2002  |  Volume : 8  |  Issue : 1  |  Page : 28-30
Malakoplakia in the appendix: Unusual association with mucinous cystadenoma: Case report and review of literature

Department of Pathology and Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

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Date of Submission30-Jan-2001
Date of Acceptance07-Jul-2001

How to cite this article:
Barayan SS, Al-Anazi AH. Malakoplakia in the appendix: Unusual association with mucinous cystadenoma: Case report and review of literature. Saudi J Gastroenterol 2002;8:28-30

How to cite this URL:
Barayan SS, Al-Anazi AH. Malakoplakia in the appendix: Unusual association with mucinous cystadenoma: Case report and review of literature. Saudi J Gastroenterol [serial online] 2002 [cited 2021 Feb 28];8:28-30. Available from:

Malakoplakia is a rare chronic inflammatory disorder affecting commonly the genitourinary tract, but also reported in many different organs. The classical diagnostic histopathological features are aggregates of foamy macrophages with granular eosinophilic cystoplasm containing Michaelis­Gutmann bodies. Malakoplakia of the gastrointestinal tract is rare and in the appendix is extremely rare. To our knowledge, its occurrence in association with mucinous cystadenoma of the appendix has not been reported in the literature.

   Case Report Top

A 67-year-old Saudi female P8 + 0 presented with a long history of vague right lower abdominal pain for many years with no associated constitutional symptoms. She was thought to have a right ovarian mass and was referred to King Faisal Specialist Hospital and Research Center in Riyadh for further evaluation and management. Physical examination was unremarkable apart from generalized abdominal tenderness with guarding. Pelvic ultrasound and CT scan showed a right iliac fossa predominantly cystic mass related to the terminal ileum with no connection to right adnexa measuring 10cm in its long axis. The patient underwent laparotomy and right hemicolectomy was done.

   Pathological Findings Top

The hemicolectomy gross specimen contained an ovoid firm cystic mass attached to the cecum measuring 8cm long and 4cm in diameter with a wall thickness of 0.8cm. It appeared to be independent of caecal mucosa. The mucosa of the rest of the specimen was unremarkable.

The appendix could not be identified. The cyst wall was composed of dense fibrous tissue with a few preserved smooth muscle fibers, focally, it was lined partly by a single layer of mucin producing columnar epithelial cells and partly by foamy macrophages containing Michaelis-Gutmann bodies [Figure - 1],[Figure - 2]. Michaelis-Gutmann bodies were positive with periodic acid schiff and Von Kossa stain. No demonstrable organisms were seen in Gram or Geimsa stained sections.

   Discussion Top

Malakoplakia is a chronic inflammatory disorder. It was first described by Michaelis-Gutmann in 1902. In 1903, it was named 'malakoplakia' by Von Hansemann. The term malakoplakia originates from the Greek 'malakos' (soft) and plakion (plaque) [1],[2] . It affects commonly the genitourinary tract. Malakoplakia has also been reported to occur outside the genitourinary tract [1],[3],[9] . It has been reported to have a bimodel age distribution, one occurs in children less than 13 years and the other is in the middle age group [3] . It is commonly associated with immunocompromised status, systemic disorders (example: sarcoidosis, tuberculosis, leukemia etc.), carcinoma and autoimmune diseases [1],[10] . But it was also reported in previously healthy persons. El­Mouzan MI et al proposed a possible genetic predisposition [4] . Malakoplakia of gastrointestinal tract is rare with prediliction to the large bowel and in particular to the rectosigmoid region. Most of the cases were associated with either systemic disease or gastrointestinal pathology. Colonic malakoplakia was particularly associated with neoplasms and immunosuppression. In the gastrointestinal tract the common gross presentation is a polypoid mass although a multinodular or stenotic presentation are reported. Endoscopically three types are known: (1) large mass, (2) focal lesion and (3) ubiquitious mucosal [1],[4],],[5],[6],[7] . To date, four cases of malakoplakia of the appendix have been described in the literature. Blackshear reported the first in 1970 [8] . None of these were associated with mucinous cystadenoma.

Mucinous tumors of the appendix are group of appendiceal diseases characterized by local or diffuse dilatation of the appendiceal lumen by mucinous secretions with different morphological features and pathogenesis. It was assumed in the past that these changes were secondary to proximal obstruction of the lumen by a pathological lesion with atrophic epithelium but no neoplastic features. In other cases focal areas of mucinous hyperplasia of the lining epithelium may be seen, which resembles the histological feature of colorectal hyperplastic polyps. In this instance there is no evidence of appendiceal rupture or peritoneal implants. The majority of cases, where the term 'mucocele' is used are true mucinous tumors. The majority are benign (mucinous cystadenoma). In 20% of cases, however, it is associated with appendiceal perforation and localized collection of mucus in the peritoneal cavity. Histologically, there is again no cell atypia and appendectomy is usually curative. On the other hand, mucinous cystadenocarcinoma is less common, associated with invasion of the appendiceal wall and peritonium (pseudomyxoma peritoni). These tumors resemble the appearance and carry the same prognosis as the ovarian mucinous cystadenocarcinoma.

The pathogenesis of malakoplakia remains obscure. It is strongly suggested that it results from altered immune response to an infectious agent. Impaired killing and phagocytic function of mononuclear cells, which is expressed by abnormal low levels of cyclic guanosine monophosphate or high adenosine monophosphate to cyclic guanosine monophosphate ratio, which are responsible for effective lysosomal activity and therefore bacteriocidal functions is the prime factor in its pathogenesis. This leads to partial digestion of the microbial agent which acts like a focus for deposition of organic and inorganic substances commonly calcium, phophorous and iron [1],[4],[11] . In our case, the existence of malakoplakia with mucinous cystadenoma lead to the confusion in clinical and radiological diagnosis. It seems likely that malakoplakia has developed as a result of altered tissue immune response against normal bowel flora. Secondary to the underlying mucinous cystadenoma, several bacteria are cultured from malakoplakia, the most common on gram-negative bacilli ( E.coli Scientific Name Search , klebsiella, enterobacter species) and gram-positive cocci (staphyloccus aureus, streptococcus and enterococcus species). In addition, in immunosuppressed patients, the immune response is impaired due to abnormal leukocytic function. In these patients, the disease process is usually progressive and refractory to treatment until underlying immunosuppresion status is corrected [1],[10] .

The classical diagnostic histopathological findings include aggregates of large foamy macrophages with abundant granular eosinophilic cytoplasm, which are called Von Hansemann cells. This granularity is PAS positive and it is due to giant phagolyosomes.

Some of the macrophages contain round laminated calospherules call Michaelis-Gutmann (MG) bodies. These are basophilic concentrically laminated structures, which are present intra as well as extracellularly. MG bodies are positive with PAS, von-Kossa and stain for calcium, Prussian blue stain for iron, which confirm the diagnosis of malakoplakia [1] . Electron microscope examination usually deferred to atypically cases or cases presenting in unusual site. In the early states of MG body evaluation, giant phagolysosomes are evident and these usually contain myeloid figures with a finger-print like configuration, which may represent digestion of bacterial cell wall. Small electron dense calcification can be also seen. In the initial states of calcifications, the dense particles are arranged usually haphazardly then they aggregate to form a dense core body and then more of calcified specules will arrange at the periphery in a radial fashion. Demonstration of bacteria elements is sometimes seen [6],[12],[13] . X-ray microanalysis of MG bodies demonstrated that it is composed of variable constituent. But it is well known that it contains hydroxyapatite, which develops within the phagolysosomes and cytosegrosomes. Before mineralization, they are only composed of laminated myelinoid figure-like membrane structure, which act as a nidus for deposition of organic and inorganic substances. In general, biochemical and radiographic microanalysis vary and they don't contribute much to the diagnosis [9],[12]. Many different therapeutic modalities have been described in the literature. They are mainly focused on two major targets. First, to improve the killing capability of macrophages by trying to elevate the level of cyclic guanisine monophophate (C-GMP). Second, to eliminate any underlying infectious cause. In addition, the control of any underlying immunosuppressing agent is mandatory especially in widespread disease status. Surgical excision of localized lesions is usually curative as demonstrated in our case. Many antimicrobial agents are tried including ciprofloxicin, clofamine, ampicillin, cefazolin, gentamicin and trimetheprim­sulfamethoxazole. Ascorbic acid and bethanechol chloride were used to improve macrophages lyosomal activity by elevating C-GMP levels [1],[4],[16]. In patients presenting with right-sided abdominal pain and mass, the possibility of this being due to a mutinous cystadenoma with or without malakoplakia should be suspected and investigated. Both clinician and pathologist should be aware that the clinical presentation and macroscopic findings can simulate a malignant neoplasm.

   References Top

1.Lowitt MH, Kariniemi AL, Niemi KM, Kao GF. Cutaneous malacoplakia: A report of two cases and review of 'the literature. Dermatology 1996; 34:325-32.  Back to cited text no. 1    
2.Von Hansemann D. Uber malakoplakia der hamblase virchows. Arch Path Anat 1903, 173:302-18.  Back to cited text no. 2    
3.Moran CA, West B, Schwartz IS. Malacoplakia of the colon in association with colonic adenocarcinoma. American Journal of Gastroenterolog 1989; 84:1580-2.  Back to cited text no. 3    
4.EI-Mouzan MI, Satti MB, AI-Quorain, El-Ageb A. Colonic malacoplakia - occurrence in a family. Report of cases. Dis Col & Rec 1988; 31:145-50.  Back to cited text no. 4    
5.Boucher LD, Aoki M, Lee EY, Cibull ML. Malakoplakia of liver -associated with a perforated colonic diverticulum. J. Clin Gastroenterol 1994, 19:318-20.  Back to cited text no. 5    
6.Price HM Hanrakan JB, Florida RG. Morphogenesis of calcium laden cytoplsmic bodies in malakoplakia of the skin; an electron microscopic study. Hum Pathol 1973:­4:381-94.  Back to cited text no. 6    
7.Sanusi ID, Tio FO. Gastrointestinal malacoplacia: report of a case and a review of the literature. Am J Gastroenterol 1974; 62:356-66.  Back to cited text no. 7  [PUBMED]  
8.Blackshear WM, Malakoplakia of the Appendix: A case report. Am J Clin Path 1970; 53.281-7.  Back to cited text no. 8    
9.Price HM, Hanrahan JB, Florida RG. Morphogenesis of Sencer O, Sencer H, Uluoglo O, et al. Malakoplakia of the skin: ultrastructure and quantitative x-ray microanalysis of Michaelis-Gutmann bodies. Arch Pathol Lab Med 1979, 103:446-50.  Back to cited text no. 9    
10.Shabti M, Anaise D, Frei L, et al. Malakoplakia in renal transplantation: an expression of altered tissue reactivity under immunosuppression. Transplant Proc 1989; 21:3725-­7.  Back to cited text no. 10    
11.Abdou NI, NaPombejara C, Sagawa A et al. Malakoplakia: evidence for monocyte lysosomal abnormality correctable by cholinergic agonists in vitro and in vivo. N Engl J Med 1997; 297:1413-9.  Back to cited text no. 11    
12.Papadimitriou JM, Henderson DW, Spagnolo DV. Diagnostic ultrastructure of non-neoplastic diseases. (Book)  Back to cited text no. 12    
13.Porrazzi LC, Degregorio A, Ferrarccio F, et al. A case of fistulizated pelvic malakoplacia: cytohistologic, immunohistochemical and electron-microscopic study. Appl Pathol 1989, 7249-55.  Back to cited text no. 13    
14.Chen CS, Lai MK, Hsueh S, Hwang TL, Chuang CK. Renal malakoplakia with secondary hepato-duodenal involvement. Journal of Urology 1994; 151:982-5.  Back to cited text no. 14  [PUBMED]  
15.Herrero C, Torras H, Palou J, et al. Successful treatment of a patient with cutneous malacoplakia with clofazimine and trimethoprim-sulfamethoxazole. J Am Acad Dermatol 1990; 23:947-8.  Back to cited text no. 15  [PUBMED]  
16.Van Furth R, Van't Wout JW, Wertheimer RP, et al. Ciprofloxacin for treatment of malakoplakia. Lancet 1992;339:148-9.  Back to cited text no. 16    

Correspondence Address:
Abdul Rahman H Al-Anazi
King Fahad Hospital of the University, P.O Box 40010, AL Khobar 31952
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

PMID: 19861789

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