Ovarian tumour-cutaneous fistula as a primary presentation of pelvic malakoplakia coexisting with a benign ovarian tumour mimicking advanced ovarian cancer.

Malakoplakia is a rare granulomatous, chronic inflammatory disease generally affecting the urogenital organs, though it can arise in other organs. The clinical manifestations of malakoplakia vary depending on the affected organ. The final diagnosis is confirmed by the presence of Michaelis-Gutmann bodies on pathology. This report describes a case of pelvic malakoplakia accompanied by an ovarian tumour-cutaneous fistula, initially misdiagnosed as advanced ovarian cancer invading the anterior abdominal wall with left pleural effusion based on imaging studies and increased serum carbohydrate antigen 19-9. The patient underwent left thoracentesis and fluid collection from the fistula tract for cytology, which showed no malignancy. She underwent primary debulking surgery, including removal of the fistula tract from anterior abdominal wall. Histopathological examination revealed malakoplakia coexisting with mucinous cystadenoma of the left ovary. For postoperative management, she received prolonged oral antibiotics for 6 months. There was no evidence of disease recurrence at the 24-month follow-up.

Clinical Evaluation and Surgical Management of Ocular Malakoplakia of the Caruncle and Periorbital Region: A Report of Two Case and Literature Review.

Ocular malakoplakia, a rare inflammatory disorder characterized by Michaelis-Gutmann bodies, is presented in 2 unique cases involving uncommon anatomical sites-the orbit and caruncle. The first case describes a 10-year-old girl with localized subconjunctival swelling near the caruncle, prompting surgical excision, and revealing characteristic malakoplakia features. Despite medical management, surgical intervention provided symptom relief. The second case involves a painless swelling below the lower lid in a 23-year-old female, initially suggestive of lymphoma. Excision biopsy confirmed malakoplakia, emphasizing the potential for clinical misdiagnosis. Histopathological examination showcased Michaelis-Gutmann bodies, von Hansemann cells, and chronic inflammation, confirming the ocular malakoplakia diagnosis. These cases underscore the rarity of ocular malakoplakia, particularly in pediatric patients, and highlight the importance of accurate diagnosis and appropriate management.

A Rare Case of Prostatic Malakoplakia Associated with Prostate Adenocarcinoma: A Case Report and Review of Literature.

Malakoplakia is a chronic granulomatous condition that has been rarely seen affecting the prostate. Isolated malakoplakia of the prostate occurring together with prostatic adenocarcinoma is rarer still with only 9 previously recorded cases. We present a case diagnosed through needle biopsy with prostatic adenocarcinoma and then on subsequent prostatectomy was diagnosed with extensive malakoplakia occurring with the carcinoma. Patient was noted to have a urinary tract infection (UTI) 2 weeks after needle biopsy and notably, 4 of the 9 previously reported cases also presented with UTI following their biopsies. The theory that prostatic malakoplakia may be a complication of the prostate needle biopsy is logically possible, but due to the paucity in cases, it is difficult to infer causality.

Malakoplakia with aberrant ALK expression by immunohistochemistry: a case report.

BACKGROUND: Malakoplakia is a rare inflammatory disease of the urogenital tract. There have been no reports of malakoplakia expressing anaplastic lymphoma kinase (ALK) to date. Here, we present one case of malakoplakia with aberrant ALK expression by immunohistochemistry and discuss the clinical significance. CASE PRESENTATION: A 65-year-old Chinese woman with a history of diabetes presented with solid masses in the liver and kidney and elevated lesions on the mucosal surface of the colon. Right nephrectomy and partial liver resection were performed. Microscopically, sheets of histiocytes with poor intercellular adhesion were seen, with Michaelis-Gutmann bodies present in both the intracellular and extracellular interstitium. CD10-, CD68-, and CD163-positive cells were present, with Michaelis-Gutmann bodies confirmed by staining with Alcian blue, periodic acid-Schiff (PAS), periodic acid-Schiff with diastase, Von Kossa, and Prussian blue. Aberrant ALK1 and ALK (D5F3) expression was observed in the cytoplasm and nucleus of cells. However, ALK gene mutation was not detected by fluorescence in situ hybridization or whole exome next-generation sequencing. NGS revealed nine individual somatic gene mutations: GOT1L1, GLIS2, SPOUT1, TMEM97, MUC3A, NSD2, SFXN5, ADAD1 and RAD50. The significance of the somatic gene mutations detected in this study is not clear, and the relationship between them and malakoplakia cannot be clarified by existing scientific studies. The pathological diagnosis was malakoplakia with aberrant ALK expression by immunohistochemistry. The antibiotics imipenem and vancomycin were started based on the results of drug sensitivity analysis and the patient was subsequently discharged. She experienced no discomfort during 30 months of follow-up. CONCLUSION: This is the first reported case of malakoplakia with aberrant ALK expression, it should be differentiated from ALK-positive histiocytosis to avoid misdiagnosis.

A Case of Urinary Bladder Malakoplakia in a Young French Bulldog: Diagnostic and Therapeutic Issues.

A 3-month-old female French Bulldog presented with hematuria, severe pollakiuria, and urinary incontinence lasting for 1.5 months. Broad-spectrum empirical antibiotic therapy and nonsteroidal anti-inflammatory drugs were initiated by the referring veterinarian. Due to a lack of improvement, the dog was referred. At referral examination, urinary clinical signs persisted (hematuria, severe pollakiuria) and a firm bladder was noted. Abdominal ultrasonography revealed severe, diffuse bladder wall thickening with a significant reduction in the bladder lumen. Urinary tract endoscopy showed whitish exophytic proliferations throughout the entire bladder wall. Histological bladder wall analysis led to a diagnosis of bladder malakoplakia. Prolonged antibiotic therapy with fluoroquinolones was prescribed and resulted in clinical remission despite persistent bacteria in the bladder wall. This report describes a case of successfully medically managed bladder malakoplakia, a very rare condition in veterinary medicine, well documented in humans.

[Colonic malakoplakia: Rare disease that can mimic a consuntive syndrome]. / Malacoplaquia del colon: Rara enfermedad que puede presentarse como un sindrome consuntivo.

Colonic malacoplakia is an unusual cause of chronic diarrhea, and it may present as a consumptive disease. At the colon, it can induce ulcerative and erosive nodular lesions, that mimic other common granulomatous or infectious diseases. Diagnosis is support in biopsies showing groups of histiocytes, with typical Michaelis-Gutmann inclusions, which are positive with the Von Kossa stain. We present the case of a 55-year-old male, without associated diseases, who presented with diarrhea, weight loss and anemia, showing a very good clinical response to antibiotics.

Presacral malakoplakia presenting as foot drop: a case report.

BACKGROUND: Malakoplakia is a rare condition characterized by inflammatory masses with specific histological characteristics. These soft tissue masses can mimic tumors and tend to develop in association with chronic or recurrent infections, typically of the urinary tract. A specific defect in innate immunity has been described. In the absence of randomized controlled trials, management is based on an understanding of the biology and on case reports. CASE PRESENTATION: Here we describe a case of presacral malakoplakia in a British Indian woman in her late 30s, presenting with complex unilateral foot drop. Four years earlier, she had suffered a protracted episode of intrapelvic sepsis following a caesarean delivery. Resection of her presacral soft tissue mass was not possible. She received empiric antibiotics, a cholinergic agonist, and ascorbic acid. She responded well to medical management both when first treated and following a recurrence of symptoms after completing an initial 8 months of therapy. Whole exome sequencing of the patient and her parents was undertaken but no clear causal variant was identified. CONCLUSIONS: Malakoplakia is uncommon but the diagnosis should be considered where soft tissue masses develop at the site of chronic or recurrent infections. Obtaining tissue for histological examination is key to making the diagnosis. This case suggests that surgical resection is not always needed to achieve a good clinical and radiological outcome.

A Surgical Challenge Generated by Colonic Malakoplakia in Disguise as a Locally Advanced Colonic Malignancy-A Case Report.

Colonic malakoplakia is an uncommon granulomatous development of cells resulting from the impaired capacity of the mononuclear cells to eliminate the phagocytosed bacteria, and in rare cases it can also affect the gastrointestinal tract. We report the case of a 78-year-old female patient that was admitted to hospital by The Emergency Department with the diagnosis of bowel obstruction, confirmed by the clinical and paraclinical investigations. We decided to surgically manage the case for suspicious symptomatic colonic neoplasm. The histological examination of the surgical specimens revealed colonic malakoplakia, characterized by the presence of the aggregated granular histiocytes and Michaelis-Gutmann bodies. Through this paper, we want to raise awareness for Malakoplakia, which remains an extremely rare disease that may affect multiple organs, and because it does not present specific symptoms or clinical manifestations, the final diagnosis remains the histopathological study. The clinical conduct should be decided after taking into consideration all the aspects of this pathology along with the benefits and risks for the patient.

Renal Allograft Malakoplakia Presenting as a Pseudotumoral Lesion.

Malakoplakia is an uncommon inflammatory disease that can involve many organ systems but is often encountered in the urogenital tract. Kidney allograft malakoplakia is even rarer and can have a diffuse parenchymal or a pseudotumoral presentation. We describe a case of grafi malakoplakia in an adult female, who presented with dull aching pain in the right loin, fever, and vomiting. Ultrasonography of the kidney graft showed a heterogeneous lesion (2.6 cm × 2.9 cm), raising suspicion of primary or metastatic renal tumors. The diagnosis was established after a histopathological examination of the kidney biopsy. This pseudotumoral presentation of malakoplakia can mimic renal cell carcinoma, lymphoma, fungal infections, or tuberculosis. It is essential to perform a biopsy for establishing the diagnosis.

Malakoplakia of the urinary bladder: A review of the literature.

OBJECTIVE: The aim of the study is to make a review of the literature about bladder malakoplakia. MATERIAL AND METHODS: We searched articles on the PUBMED web-literature database with the following keywords: "vesical malakoplakia" and "bladder malakoplakia". In the literature we found 254 articles. At final we have excluded 219 articles, including in our study only 35 articles. RESULTS: The overall average age found was 50.85 years. The average age of men was 43.22 years, while that of women was 53.37 years. 75% of the patient cases were women and 25% were men. Regarding comorbidities, in 5.55% of the cases were missing whereas 47.22% of the patients suffered from recurrent urinary tract infection (UTI) and 19.44% from immune system disorders. Urine culture was positive in 69.44% with E.coli being isolated in 92% of cases. Hydroureteronephrosis was present in 44.44% of the cases: left in 6.25% of cases, right in 18.75% and bilateral in 75%. The mean serum creatinine of patients with hydroureteronephrosis was 5.11 (1-21) mg/dl. The most frequent site of the lesion was the vesicoureteral junction (VUJ) (42.31%), followed by the trigone (38.46%). 30.56% of patients were treated with antibiotic and surgery (transurethral resection of bladder, partial or radical cystectomy), less frequent options were antibiotics alone and surgery alone. The recurrence rate was 15%. CONCLUSIONS: Malakoplakia is a disorder usually related to other affections, like UTI and immunodepression, and it seem to be caused by an abnormal macrophage function. In almost half of the described cases of isolated bladder malakoplakia, hydroureteronephrosis and renal failure were present.Treatment is not standardized, but both medical and surgical therapies are effective to avoid recurrence.

Renal malakoplakia with invasion of the liver and diaphragm: a patient case and literature review.

Renal malakoplakia, a seldom seen chronic inflammatory condition, continues to elude medical, surgical, radiological and pathological specialists due to its mimicry of other renal pathologies and low incidence. The variable clinical manifestations and non-specific radiological findings of malakoplakia can be misleading, and ultimately require a pathological diagnosis. A literature review reveals an extremely low prevalence of renal malakoplakia, a handful of invasive renal malakoplakia cases and no reports of liver and diaphragmatic invasion. We present a case of a renal mass with liver and diaphragmatic invasion in a 59-year-old woman that deceived clinicians and radiologists until a pathological diagnosis of renal malakoplakia was performed. This case highlights the need of awareness for malakoplakia in the differential diagnosis for renal invasive and non-invasive masses. The need to await a surgical biopsy and pathological diagnosis is critical to ensure a correct diagnosis and avoid unnecessary surgery of the kidney.

Case study: Malakoplakia of the bladder.

OBJECTIVES: Malakoplakia is a rare chronic inflammatory disease thought to be the result of defective bacterial phagocytosis and lysosome function, and there is difficulty in accurate diagnosis as a result of non-specific symptoms that mimic other diseases and cancers. This study presents a case of bladder malakoplakia associated with renal failure presenting as a tumor. METHODS: A 55-year-old woman with history of kidney disease who presented with general malaise and worsening renal failure was found to have a bladder mass and underwent transurethral resection of bladder tumor (TURBT), and subsequent histological examination. RESULTS: The bladder mass consisted of basophilic structures known as Michaelis-Gutmann bodies within clusters of macrophages on histological examination, and stained positive for CD68. Von Kossa stain highlights Michaelis-Gutmann bodies, consistent with the diagnosis of malakoplakia. CONCLUSIONS: Conservative treatment via antibiotics has been effective. Proper diagnosis of bladder malakoplakia is important, as the conditions it mimics often require surgery and resection. Additionally, it is important to recognize the implications bladder malakoplakia has on renal functioning, particularly regarding urinary obstruction.

Outgrowing skin involvement in malakoplakia after kidney transplantation: A case report.

INTRODUCTION: Malakoplakia is a rare pseudotumor that arises in the context of recurrent infections, particularly in immunocompromised states. We report a case of renal allograft parenchymal malakoplakia. CASE REPORT: A 59-year-old woman successfully received a cadaveric renal transplant in June 2018. Two months after transplantation, she was treated for a urinary tract infection (UTI). In March 2019, she underwent allograft biopsy for increasing creatinine. The biopsy identified T cell mediated rejection and steroid pulse therapy was performed. In December 2019, she was hospitalized for right flank pain and pyuria, and her creatinine level was 1.9 mg/dL. Radiographic findings were suggestive of a hematoma or abscess in the perirenal area, and septated fluid collection was suspected. Biopsy results suggested malakoplakia, and von Kossa stain was positive for Michaelis- Gutmann bodies. Tissue culture demonstrated Escherichia coli, and this was treated with antibiotics. The dose of tacrolimus was reduced. The patient was discharged after 1 month of hospitalization and was maintained on oral antibiotics. Follow-up imaging revealed an increase in the extent of lesion into the adjacent abdominal wall. Assuming the case to be refractory, we performed surgical resection and abscess drainage. Although the renal parenchymal involvement persisted, the size showed a decreasing trend over 2 months of serial observation with ultrasonography. CONCLUSIONS: Malakoplakia should be considered as a differential diagnosis for recurrent UTI with graft dysfunction. Malakoplakia can be successfully treated with reduction in immunosuppression and medical therapy using long-term antibiotic treatment in most cases. However, early surgical treatment must be considered for refractory cases.

Cutaneous malakoplakia with verruciform xanthoma in the same lesion.

Cutaneous malakoplakia (CM) is a rare, chronic, granulomatous disease characterized histopathologically by Michaelis-Gutmann bodies (MGB). Verruciform xanthoma (VX) is a rare, benign lesion characterized histopathologically by epithelial papillomatous hyperplasia, local hyperkeratosis with incomplete keratosis, infiltration of foam cells and inflammatory cells in the papillary dermis. We present an elderly Chinese man with CM and coexisting VX with histological confirmation of MGB.

[Cutaneous malakoplakia: A rare pseudo-tumor to know in immunocompromised patients]. / Malakoplakie cutanée : une pseudo-tumeur rare à ne pas méconnaître chez l'immunodéprimé.

Cutaneous malakoplakia is a rare pseudo-tumor that occurs in immunocompromised patients. It is a reaction to an infection caused by Gram negative bacteria. The clinical presentation is nonspecific and the diagnosis is histological. The evolution is recurrent and the combination of a surgical treatment, antibiotics and adaptation of immunosuppressive therapy is necessary to cure the disease. The emergence of antibiotic resistance in bacteria responsible for the pathology can complicate the treatment and require additional microbial sampling. We report a case that occurred in a renal transplant patient with a complex diagnostic and therapeutic management.