Complete treatment with partial cystectomy in giant xanthogranulomatous cystitis case imitating bladder tumor.

Xanthogranulomatous cystitis (XC) is a very rare chronic benign inflammatory disease of the bladder. It may cause local invasion although it is not a malign lesion and may occur together with malign lesions. It has a clinical importance as the distinction from malign lesions is difficult clinically and pathologically. Sharing a 37-year-old female case with giant XC imitating bladder tumor referring to the hospital with hematuria and stomach ache, together with current literature, we wanted to present that the disease can be treated with bladder-preserving approaches instead of radical approaches even though the mass is big in these cases. Application of basic excision and partial resection for small masses and radical cystectomy for large masses was reported in literature. We think that our case may provide a contribution to literature in treatment approach since we provided surgical cure with partial resection in a big mass with dimensions of 9 cm × 8 cm which is different from the present literature. Even though XC is a rare disease, it should be considered in prediagnosis for especially big dimensioned masses, and treatment should be planned according to the pathology result after together with cystoscopy in suitable patients.

INTRODUCTION

Xanthogranulomatous cystitis (XC) is a very rare chronic benign inflammatory disease of the bladder. Colon, pancreas, stomach, lung, appendix, gall bladder, over, vagina and endometrium cases were also reported apart from the bladder. Function disorder occurring in macrophages due to immunological defect is held responsible. It is considered that chronic irritation plays an important role in etiology. It may cause local invasion although it is not a malign lesion and may be seen together with malign lesions. It has a clinical significance as its distinction from malign lesions is difficult in clinical and pathological terms.[12]

In the light of current literature, we wanted to share XC case imitating bladder cancer radiologically and macroscopically in the patient referring with stomach ache and hematuria.

CASE REPORT

A 37-year-old female was presented to the urology clinic with low abdominal pain throughout the couple last months, yet she was painless for the last half a month. Accompanying low abdominal pain, there was also a 1 week hematuria history. During the physical examination, a hard suprapubic mass, bimanually palpable, was determined. It can mimic clinically and macroscopically malignancy.[3]

Laboratory analyses showed a normal hematological and biochemical profile. However, patient's urinalysis revealed plenty of red blood cells per high-power field. The urine culture of the patient was sterile. Patient's ultrasonography showed a heterogeneous mass lesion at the anterior wall of the bladder. Patient's magnetic resonance image (MRI) demonstrated a cyst of 90 mm × 80 mm on the anterior wall of the bladder. The MRI revealed no invasion on the surrounding organs [Figure 1].

Figure 1

Magnetic resonance image demonstrated a cyst on the anterior wall of the bladder

A tumoral fistula opening to the superior bladder wall was observed during patient's cystoscopy. Thereupon, the patient underwent surgical exploration. A solid mass of 9 cm reaching from the bladder into the abdominal cavity was seen. It was removed via mass partial cystectomy and simple excision [Figure 2]. The histological analysis of the obtained specimen revealed chronic inflammatory XC. Xanthogranulatous macrophages were negative for periodic acid-Schiff-positive material, and calcospherules (Michaelis–Guttman bodies) [Figure 3], CD68-positive epithelioid and foamy macrophages were detected [Figure 4]. Acid-fast bacilli staining was also negative. Likewise was the immunohistochemical staining for cytokeratin. No malignancy or urachal remnant abnormalities were observed. No complications occurred during the postoperative period. Moreover, during the 1-year follow-up, the patient has not reported any complaints.

Figure 2

Macroscopic partial cystectomy sample image

Figure 3

Microscopy of simple excision samples reveals foamy histiocytes and absence of Michaelis–Gutmann bodies (×100)

Figure 4

CD68-positive epithelioid and foamy macrophages were detected (×200)

DISCUSSION

Xanthogranuloma is a rare granulomatous inflammatory condition characterized by the presence of multinucleated giant cells, lipid-laden macrophages (xanthoma cells), and cholesterol crystals.[4] Histologically, xanthogranulomatous lesions can be confused with malakoplakia and can be differentiated from it, by the absence of Michaelis–Guttmann bodies (basophilic lamellar inclusion bodies) and the presence of lare no of monocytes.[2] It was first reported in 1932,[5] and according to the best of our knowledge, there are less than thirty cases (up to now, more than twenty cases have been reported in PubMed literatures) with the following clinical characteristics: Median age of 45 years; no obvious sexual preference; majority of lesions located in the bladder dome (85.7%), and associated mostly with an urachal remnant (70.8%).[6] Yang et al. reported an unusual mass located on the posterobasal wall of the bladder without a relation to the urachus.[7] However, a few were associated with urothelial carcinoma or urachal carcinoma.[8] In XC urinary symptoms, abdominal pain or umbilical discharge is present.[9] Although hematuria is a common symptom, it may not be seen in every case.[2] In the present case, a 37-year-old woman, there were low abdominal pain and hematuria complaints. During initial imaging and surgical procedure, lesion, stemming from the superior bladder wall, was explicit.

Although the etiology of XC is still unclear, some explanations proposed are as follows: A chronic inflammatory process caused by mechanisms such as (a) immunological defect of the macrophage, (b) Gram-negative or anerobic bacteria presence in urinary tract infections or infections after tubal ligation,[10] (c) chronic infection or cystitis of the urachal diverticulum, (d) foreign body such as suture material retained,[11] (e) local response to bladder tumor,[812] (f) abnormal lipid metabolism and accumulation, and (g) inflammatory bowel disease.[13] In the present case, the underlying reason of XC could be a response to chronic infection without obvious symptoms.

As medical treatment is considered not to yield the expected outcomes, conservative management is rarely preferred.[614] In the treatments, surgical resection is preferred as there are no reports about postoperative recurrence.[15] Many times, a simple excision may suffice for a localized xanthogranuloma; however, if combined with adenoma or an urachal remnant, then partial cystectomy can be considered. If urachal carcinomas are suspected, an extensive surgery including urachal tract excision can be necessary. Additional antibiotic therapy may be considered in cases with positive microbial cultures.[10] Yet, as XC is associated with urachal remnant as a source of chronic bacterial infection, conservative treatment is not preferred.[9]

Application of basic excision and partial resection for small masses[1] and radical cystectomy for large masses[2] was reported in literature. We think that our case may provide a contribution to literature in treatment approach since we provided surgical cure with partial resection in a big mass with dimensions of 9 cm × 8 cm, which is different from the present literature.

To summarize, in the present case, histological features were similar to other XC cases reported. Treatment preferred was simple excision of the lesion and a close follow-up.

CONCLUSION

Even though XC is a rare disease, it should be considered in prediagnosis for especially big dimensioned masses and treatment should be planned according to the pathology result after together with cystoscopy in suitable patients. Hence, there may be a treatment chance with partial resection for the patient without applying radical treatments as in our case.

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Conflicts of interest

There are no conflicts of interest.