Extensive Urinary Malakoplakia with Lymph Node Involvement: A Case Report

Case Report

Austin J Clin Pathol. 2014;1(3): 1011.

Extensive Urinary Malakoplakia with Lymph Node Involvement: A Case Report

Yihong Ma1, Jeffrey W Nix2, Ryan C Telford3, Gene Siegal1 and Dejun Shen1*

1Department of Pathology, University of Alabama, USA

2Department of Urology, University of Alabama, USA

3Department of Radiology, University of Alabama, USA

*Corresponding author: Dejun Shen, Department of Pathology, University of Alabama at Birmingham, 619, 19th Street South, NP 3546, Birmingham, AL 35249, USA

Received: May 25, 2014; Accepted: June 24, 2014; Published: June 27, 2014


Malakoplakia is an unusual chronic inflammatory disease found mostly in the urinary system, although involvement of other organ systems has been occasionally reported. Masses, ulcers, plaques or papules are formed along the urinary tract and may represent a diagnostic challenge on cystoscopic or imaging studies. Extensive malakoplakia could be systemic and is often associated with immune suppression. We herein report a48-year-old woman with extensive malakoplakia involving the left kidney and renal pelvis, the left ureter, the urinary bladder and the perirenal lymph nodes. The patient was a heavy smoker with a recent history of recurrent urinary tract infections, but had no other significant medical history including immune suppression. She initially presented with abdominal pain, nausea, emesis and unintended weight loss. A CT scan demonstrated significant left hydronephrosis caused by mass lesions in her bladder and left distal ureter. Marked retroperitoneal lymphadenopathy was also noted. Transurethral resection of the bladder tumor and a left laparoscopic ureteronephrectomy revealed extensive malakoplakia upon pathological examination. The patient's retroperitoneal lymphadenopathy was improved as assessed by imaging studies after surgery and antibiotics therapy. Malakoplakia should be considered in the differential diagnosis for patients with a history of urinary tract infections presenting with a mass lesion. Early histological diagnosis and prompt antibiotic treatment may be helpful in avoiding disease progression and potential complications.

Keywords: Malakoplakia; Urinary system; Lymph node involvement; Ureteronephrectomy; Transurethral resection of bladder tumor; Immune suppression


CT: Computed Tomography; H&E: Hematoxylin and Eosin; TURBT: Transurethral Resection of Bladder Tumor


Malakoplakia is an unusual chronic inflammatory disease typically found in the urinary system although involvement of other organ systems has been reported [1]. These lesions usually present as masses, ulcers, plaques or papules along the urinary tract, and are frequently mistaken for a neoplasm on cystoscopic imaging studies [2,3]. Extensive malakoplakia may be associated with a history of immune suppression due to concomitant lymphoma, diabetes mellitus, renal transplantation, or long-term therapy with systemic corticosteroids [4]. Alcoholism has also been reported as a possible risk factor [5]. The pathophysiology of malakoplakiais thought to be associated with insufficient killing of bacteria by macrophages where the partially digested bacteria lead to a deposition of iron and calcium [6]. The deposition forms characteristic intra cytoplasmic Michaelis- Gutmann bodies that are typically 1-10μm in diameter, which can be identified by hematoxylin and eosin (H&E) staining of representative tissue sections. In addition, a calcium stain (von Kossa) is much more sensitive in highlighting their presence [1]. The insulting bacteria are Rhodococcusequior Escherichia coli in most cases [1,6]. Treatment options include antibiotics such as quinolone and trimethoprim-sulfamethoxazole against commonly involved pathogens, ascorbic acid and bethanechol to facilitate intracellular digestion of bacteria, and surgical resection in extreme cases [1]. Discontinuation of immunosuppressive medications and prolonged systemic antibiotics therapy are usually needed to effectively treat malakoplakia. While most of the cases may be controlled by the above therapies, the prognosis may not be ideal in some immune suppressed patients [4].

Case Presentation

The patient was a 48-year-old African-American woman with a long history of cigarette smoking and a more recent history of recurrent urinary tract infections. No other significant medical history including immune suppression was recorded. She initially presented to an outside hospital with abdominal pain, nausea, emesis, fever and chills. She was diagnosed with and treated for pneumonia at the time. However, she failed to recover from the disease with persistent fever, chills and weight loss. A CT scan showed an enhancing mass along the left bladder base measuring approximately 3.6 cm in greatest dimension (Figure 1A). A synchronous 2.0cm enhancing lesion was also noted within the left distal ureter (Figure 1B) which caused dilatation of the proximal ureter and severe hydro ureteronephrosis (Figure 1A-C), leading to a non-functional left kidney. Marked retroperitoneal lymphadenopathy was also identified, predominantly along the left periaortic space with an index node measuring 2.3cm in greatest diameter (Figure 1C). At the time, a multifocal transitional cell carcinoma with lymph node metastasis was suspected based on the imaging study. A transurethral resection of the bladder tumor (TURBT) was performed and a diagnosis of malakoplakia was established. Meanwhile, a left retrograde pyelogram showed a significant filling defect in the middle to distal ureter. No contrast was seen to advance past this lesion.