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Malakoplakia is a rare granulomatous inflammatory disorder. Its diagnosis depends on histopathological findings; however, high-quality literature regarding proper medical/surgical treatment is lacking. A 38-year-old diabetic female patient was admitted to the emergency room with a history of lower gastrointestinal hemorrhage. Colonoscopy revealed a lesion in the descending colon, and abdominal computed tomography revealed a splenic flexure mass involving the lower pole of the spleen and upper pole of the left kidney. Biopsies confirmed the diagnosis of malakoplakia. After completing antibiotic treatment, a restaging computed tomography revealed a discrete mass increase; hence, the patient underwent laparoscopic en bloc colectomy and partial nephrectomy. Postoperatively, the patient developed a pancreatic fistula, which was successfully treated with percutaneous drainage and antibiotics. The presence of pathognomonic Michaelis-Gutmann inclusions on histopathology is frequently reported as the key to diagnosing malakoplakia. Herein, we present a successful, minimally invasive surgical treatment for colonic malakoplakia.

Genitourinary malakoplakia (GUM) is a rare and chronic form of bacterial soft tissue infection, which primarily affects the genitals and urinary organs. the diagnosis is histological. Symptoms may include itching, pain during sex, bleeding, abdominal pain and burning during urination. Although treatment usually includes antibiotics, corticosteroids or anti-inflammatory drugs, and a variety of other medications and procedures may be needed to treat the symptoms. In some cases, surgery is also indicated.

Malakoplakia is a rare chronic inflammatory condition, which primarily occurs in genitourinary tract, with prostatic malakoplakia being extremely rare. We present two cases of acute urinary retention, with clinically firm nodular prostate and a raised serum prostate-specific antigen. Transrectal ultrasound-guided prostatic biopsy showed features of malakoplakia. There was a significant reduction of size of prostate on transrectal ultrasonography after 4 weeks of antibiotics. However, one patient had failed trial without catheter and was subjected to transurethral resection of prostate. The biopsy of the prostatic chips also showed features of malakoplakia. Other patient improved symptomatically after antibiotics and was managed conservatively. Both the patients are on regular follow-up and are asymptomatic. Prostatic malakoplakia presenting as urinary retention is very uncommon with around 12 cases in the literature. Recognition of prostatic malakoplakia is important because clinically it can masquerade prostatic malignancy. Treatment with antibiotics is necessary before subjecting the patients for surgery in patients with obstructive symptoms.

Xanthogranulomatous Pyelonephritis (XGP) is a rare, chronic granulomatous inflammatory condition thought to arise secondary to a combination of obstruction, recurrent bacterial infection and an incomplete immune response resulting in focal or diffuse renal destruction. This destruction may be profound with the potential to infiltrate surrounding tissues and viscera. The imaging features of XGP can be ambiguous, mimicking malignancy, tuberculosis (TB) and malakoplakia earning the title of “the great imitator”. Computed tomography (CT) is the mainstay of XGP diagnosis and staging, accurately quantifying the stone burden and staging the renal destruction, including the extent of extra-renal spread. Although some cases in children have been successfully treated with antibiotics alone, nephrectomy remains the most common treatment for XGP in adults. The specific management strategy needs to be tailored to individual patients given the potential constellation of renal and extrarenal abnormalities. Although XGP has classically required open nephrectomy, laparoscopic nephrectomy has an increasing role to play arising from the advancement in laparoscopic skills, technique and instruments. Nephron-sparing partial nephrectomy may be considered in the focal form. Interventional radiology techniques most often play a supportive role, eg, in the initial drainage of associated abscesses, but have rarely achieved renal salvage. This narrative review seeks to synthesise the existing literature and summarise the radiological approach and interventional radiology management situated in a clinical context.

Malakoplakia is a rare chronic granulomatous disease found in the genitourinary tract, mainly. It is considered to be related to immunosuppression and/or infectious processes. We would like to present an operative case of cecal malakoplakia in a patient with a history of surgical resection and chemotherapy for cervical cancer. A 74-year-old female patient visited our hospital for 1-year follow-up after operation and chemo-radiotherapy for cervical cancer. An infiltrative mass of 6 cm, between the cecal base and the right psoas muscle, was observed on computed tomography. An ileocectomy was performed for diagnosis. Histopathologic examination revealed cecal malakoplakia. After surgery, based on previous reports, antibiotics therapy was added. Then the patient was discharged and treated in the outpatient clinic. To our knowledge, a rare case has been described of cecal malakoplakia during observation after surgery and chemo-radiotherapy for cervical cancer. Malakoplakia is known to be related to immunosuppressive condition. Therefore, our case suggests that close observation should be made in patients on immunosuppressive condition, such as chemotherapy.

Malakoplakia is a chronic granulomatous disease associated with incomplete clearance of bacterial pathogens. A multimodal approach to therapy includes antimicrobials with intracellular activity, reduction in immunosuppression, and debulking of lesions. Azithromycin has an intracellular mechanism of action and enhanced Gram-negative activity compared to other macrolides. Despite some in vitro data to support its use, there are no clinical breakpoints or epidemiological cut-off values for most Enterobacterales from the European Committee on Antimicrobial Susceptibility Testing (EUCAST) or the Clinical and Laboratory Standards Institute (CLSI). We present two cases, previously unreported, of Escherichia coli associated renal allograft malakoplakia successfully treated with azithromycin.

Malakoplakia in humans most often affects the urinary bladder and is characterized by inflammation with von Hansemann–type macrophages, with or without Michaelis-Gutmann bodies, and is frequently associated with Escherichia coli infection. We describe the microscopic features of malakoplakia in the urinary bladder of 4 puppies. In all cases, the lamina propria of the urinary bladder was markedly expanded by sheets of large, round to polygonal macrophages with intracytoplasmic, periodic acid-Schiff-positive granules and granular inclusions, and rare Prussian blue–positive inclusions. Macrophages were positive for CD18 and Iba1. In 2 cases, Michaelis-Gutmann bodies were detected with hematoxylin and eosin stain and were best demonstrated with von Kossa stain. E. coli infection was confirmed in 2 cases with bacterial culture or polymerase chain reaction (PCR) and sequencing of the bacterial 16S ribosomal RNA gene. Transmission electron microscopy of one case demonstrated macrophages with abundant lysosomes, phagolysosomes, and rod-shaped bacteria. Microscopic features were similar to human cases of malakoplakia. In dogs, the light microscopic characteristics of malakoplakia closely resemble granular cell tumors and histiocytic ulcerative colitis.

Granulomatous interstitial nephritis (NIG) is a rare form of interstitial nephritis that can be related to acute or chronic clinical presentation. NIG is characterized by granulomas located to the renal interstitium and composed of either epithelioid histiocytes with giant cells and/or of foreign body reaction. The symptoms are unspecific and associate varying degrees of renal failure with abnormal urinanalysis. Extra-renal signs may point to systemic disease. Pathological examination from kidney percutaneous biopsy or surgical resection is required to assert NIG diagnosis and to guide the etiological research. The main causes of NIG are sarcoidosis, drug reactions, mycobacterial infections and crystalline nephropathies. Sarcoidosis is characterized by non-necrotic and well-formed giant cell epithelioid interstitial granulomas. Drug reactions have less well-defined granulomas with inconstant eosinophils. The presence of caseous necrosis within giant cell and epithelioid granulomas leads to infectious NIG diagnosis (tuberculosis and fungal infection). Identification of crystals within foreign body reaction can be improved by polarized light study. Xanthogranulomatous pyelonephritis and malakoplakia are rarer causes of NIG characterized by patches of histiocytes associated with inconstant giant cells. Differential diagnoses of NIG are represented by granulomatous reactions centered on glomeruli and vessels (vasculitis and emboli of cholesterol crystals). Less than 10% of NIG are idiopathic. The prognosis and the treatment vary according to the cause. The factors of poor renal prognosis are chronic irreversible tubulo-interstitial injury (tubular atrophy and interstitial fibrosis).

Malakoplakia is a rare chronic granulomatous inflammatory condition, commonly presenting in immunocompromised patients. It most commonly occurs in urinary tract. The cutaneous form is less frequent. Here, we report a case of cutaneous malakoplakia involving perianal skin in an immunocompetent 61-year old female diagnosed on histopathology utilizing special stains. To the best of authors’ knowledge, this is the 8case report of cutaneaous malakoplakia of perianal skin in the English literature and 3in the Indian literature. Keywords: Malakoplakia, Cutaneous, Perianal skin.

Background: Malakoplakia is a rare benign lesion, usually associated with deficient intralysosomal degradation of microorganisms, more commonly, Escherichia coli. Malakoplakia occurs in various organ systems, the most frequently affected site being the urinary bladder. We report a rare case of isolated extensive malakoplakia involving the prostate, diagnosed on transurethral resection performed for radiologically suspected prostatic abscesses. Case Presentation: A 61-year-old African American male presented with symptoms of urinary obstruction for the past 2 months. His medical history was significant for immunosuppression (liver transplantation 3 months prior and diabetes mellitus). He reported four episodes of E. coli-associated urinary tract infection after his liver transplantation. Serum prostate specific antigen was 1.83 ng/cc (normal inferior to 4 ng/cc), and urine culture was positive for E. coli sensitive to ceftriaxone. Pelvic magnetic resonance imaging was suggestive of prostatitis with prostatic abscesses; cystoscopy was unremarkable. The patient was started on intravenous ceftriaxone therapy. A standard bipolar transurethral resection of the prostate was performed, and purulent-like material was encountered in the resected tissue. Histologic examination demonstrated extensive infiltration and replacement of the prostatic tissue by sheets of pink histiocytes with targetoid inclusions consistent with Michaelis–Gutmann bodies, ultimately confirming malakoplakia of the prostate. Conclusion: Prostatic malakoplakia is an unexpected diagnosis in patients suspected of having malignancy or prostatitis. Its exact pathogenesis is unknown, but it involves defective bacterial degradation after phagocytosis. E. coli is often cultured from the patients' urine. Immunosuppression, present in our patient, is a well-known associated factor. Prostatic malakoplakia can radiologically masquerade as prostatic adenocarcinoma, despite the use of cutting-edge imaging technology. With the growing use of multiparametric 3T prostate magnetic resonance imaging to screen for prostate cancer, it is possible that urologists, radiologists, and pathologists will encounter prostatic malakoplakia more frequently in the future.

Background. Rhodococcus equi-related pulmonary malakoplakia is a rare condition with few reported cases; hereby, we present a case associated with advanced human immunodeficiency virus (HIV) infection, and thus far to our knowledge, the first report in Mexico. It is estimated that approximately 10% of the infections occur in immunocompetent patients, whereas the rest are immune deficient, targeting virtually any organ. Histologically, malakoplakia is characterized by the buildup of infiltrated inflammatory tissue as a consequence of the gathering of histiocytes embedded with concentric inclusions. The diagnosis relies on the cultures and the susceptibility testing as well as the pathologic findings compatible with the disease. Case Presentation. We present a 25-year-old male patient with persistent nonproductive cough for over a year and with weight loss, who comes to the emergency department with recent fever, swollen and tender lymph nodes, and hemoptysis. The patient gets diagnosed and treated for Rhodococcus equi-related pulmonary malakoplakia. Conclusion. Knowing the involvement of Rhodococcus in HIV is fundamental for the diagnosis and optimal treatment, which although unknown, a combination of antibiotics with intracellular penetration, on-time resection, and a proper immune reconstitution represents the best approach. Prognosis varies with mortality rates from 34% to 54%.

Renal malakoplakia is a rare form of chronic inflammatory granulomatous disease in the kidney. It occurs in adult patients with immunocompromised status or debilitating disease. In the present study, we reported a case of a 50-year-old woman with no underlying disease. This report describes the CT and pathological features of renal malakoplakia in a 50-year-old woman. Plain CT scan showed a large soft tissue mass at the middle and upper pole of the left kidney. Enhanced CT scan showed delayed enhancement in the solid part of the mass. Our results might provide some useful information for the diagnosis of renal parenchymal malakoplakia.Nierenmalakoplakie ist eine seltene Form der chronischen entzündlichen Granulomatose in der Niere. Es tritt bei erwachsenen Patienten mit immungeschwächtem Status oder schwächender Krankheit auf. In der vorliegenden Studie berichteten wir über einen Fall einer 50-jährigen Frau ohne zugrunde liegende Krankheit. Dieser Bericht beschreibt die CT und pathologischen Merkmale der Nierenmalakoplakie bei einer 50-jährigen Frau. Die CT zeigte eine große Weichteilmasse an der mittleren und oberen Pol der linken Niere. Ein verbesserter CT-Scan zeigte eine verzögerte Verbesserung am festen Teil der Masse. Unsere Ergebnisse könnten einige nützliche Informationen zur Diagnose der Nierenparenchymalen Malakoplakie liefern.

Xanthogranulomatous pyelonephritis is a rare chronic infectious process of the kidney, which has been described in three different forms: diffuse, segmental, and focal. It is also known as the great simulator since its clinical, radiological, and histopathological manifestations tend to be confused with other entities. We describe a case of a 55-year-old male patient with two months of clinical manifestations characterized by a 7x7-cm palpable mass in his right lumbar region, which was hot and painful upon touch and increasing in size. This article aims to present a case of xanthogranulomatous pyelonephritis and compare it with its primary differential diagnoses. It is evident that despite the condition being considered a simulating pathology, some key differences can be found to identify and distinguish it.

CASE A 66-year-old woman with polymyositis and recurrent urinary tract infections presented with lumbar pain and progressive lower extremity neuropathy. Imaging showed lytic destruction of the vertebral bone from L2 to L4 with extension into adjacent musculature. Histological examination demonstrated Michaelis-Gutmann bodies consistent with malakoplakia. The patient underwent revision T12-sacrum posterior fusion, L2-5 laminectomy, and anterior L3-4 corpectomy, followed by L2-4 anterior stabilization and a 6-week course of ceftriaxone. At 3 months postoperatively, she was asymptomatic without recurrence. CONCLUSION Malakoplakia of bone should be considered in the differential diagnosis of lytic bone lesions and can be effectively treated with surgical debulking with penetrative antibiotics.

Malakoplakia is a rare disease. Clinical presentation is non-specific, making its diagnosis an incidental finding on histopathological analysis. The aim of this case report is to describe a patient diagnosed with colon, renal and soft tissue malacoplakia mimicking a locally advanced colorectal cancer. A 75-year-old man was admitted due to intense abdominal pain. No relevant findings at the physical examination. Computed tomography showed parietal thickening of the descending colon with left kidney, iliopsoas muscle and retroperitoneum involvement. An elevated blood serum creatinine, elevated glycated hemoglobin and urinary infection were detected. Surgery was decided for suspicious symptomatic colonic neoplasm. Left segmental colectomy with left partial nephrectomy and retroperitoneal soft tissue resection was performed. Pathology report was compatible with malakoplakia. Malakoplakia is a rare disease and may affect multiple organs. Because there are no clinical-specific findings, diagnosis is usually made with histopathological study of the surgical specimen.

Gross hematuria in adults with resultant anemia is highly concerning for genitourinary malignancy. However, in rare instances, malakoplakia can mimic such malignancy. Malakoplakia is a benign granulomatous condition with malfunction of the phagolysosomal activity of macrophages and monocytes.

Malakoplakia is a granulomatous inflammatory condition commonly involving the urogenital tract. Principally it involves the urinary bladder, prostate is a rare site. Clinical and radiological examination sometimes mimics malignancy. Histology remains confirmatory modality for the diagnosis. Hereby we report a case of prostatic malakoplakia in an elderly male who presented with lower urinary tract symptoms in the past fifteen days. Urine routine microscopy showed pus cells; culture report was positive for E. coli. Serum Prostate Specific antigen (PSA) was 4.6 ug/ml. Magnetic Resonance Imaging (MRI) showed a bulky prostate. A 12-core biopsy was done to rule out malignancy and the histopathology showed benign prostatic glands and the stroma with a predominant population of epithelioid histiocytes and the characteristic Michealis-Gutmann bodies. After a course of antibiotic Nitrofurantoin, transrectal ultrasound was repeated which showed reduction in the prostatic volume. Subsequently Trans-Urethral Resection of Prostate (TURP) was done to relieve the persistent lower urinary tract symptoms. Histology showed similar features. Special stains – Von kossa and Prussian Blue highlighted the Michealis-Gutmann bodies.

Malakoplakia of kidney (MOK) is a very uncommon disease. Various internet data bases were searched to review the literature on MOK. Results obtained from reported cases and discussions relating to MOK did reveal the ensuing: MOK is a rare chronic inflammatory disease; MOK can affect kidneys in adults and children including transplanted kidneys and the immunosuppressed. MOK often tends to be associated with chronic / long term urinary tract infections especially coliforms. The manifestation of MOK is non-specific including fever, lethargy, loin pain, haematuria and loss of weight plus other non-specific symptoms. The radiology imaging appearances tend to be non-specific (normal, mimicking abscess or renal carcinoma or other chronic inflammations of kidney. Diagnosis of MOK has been established through histological examination of biopsy specimen of the lesion, FNA, or nephrectomy specimens by presence of histiocytes with Michaelis-Gutmann bodies. PCR may also be used to identify E coli in kidney tissue. MOK can be successfully treated by utilizing antibiotics (quinolones) but nephrectomy has been undertaken because of the lesion mimicking renal carcinoma. MOK does tend to be associated with coliform infections, the immunosuppressed and may mimic malignancy of the kidney. If a lesion in the kidney is associated with recurrent coliform infection / immunosuppression, MOK should be suspected and radiology imaging biopsy of the suspected renal lesion should be undertaken for the diagnosis to avoid unnecessary nephrectomy. Nevertheless, some complicated cases of MOK do need to be effectively treated by means of neohrectomy.

A 4 mo old female Staffordshire bull terrier puppy was presented with chronic Escherichia coli cystitis. Ultrasound and cystoscopic examination revealed innumerable, intraluminal, finger-like proliferations arising from the dorsal urinary bladder (UB) wall. Histological examination of mucosal biopsies obtained by cystoscopy was suggestive of granulomatous cystitis. The proliferative lesions were removed surgically and submitted for histological examination. The UB submucosa was heavily infiltrated by macrophages with periodic acid-Schiff–positive cytoplasm exhibiting rare Michaelis-Gutmann bodies, leading to the diagnosis of malakoplakia. The puppy was prescribed with sulfamethoxazole-trimethoprim. The urinary signs disappeared despite the persistent UB wall thickening revealed by abdominal ultrasound. Urine culture performed during the ninth week of treatment showed a persistent infection by E coli resistant to sulfamethoxazole-trimethoprim. The dog was switched to doxycycline but was then lost to follow-up. Malakoplakia is a chronic granulomatous inflammation well documented in humans. Its pathophysiology is not fully understood, but bacterial infection, immunodepression, and a defective lysosomal function may lead to the intracytoplasmic accumulation of partially degraded bacteria that can subsequently mineralize to form the Michaelis-Gutmann bodies. Malakoplakia should be suspected when UB mass lesions are identified in a young dog with bacterial cystitis.

A 70-year-old black male presented with a 2-month history of obstructive lower urinary tract symptoms. He had no current or previous proven history of urinary tract infections and was not on any antibiotics, steroids or immunosuppressive drugs. A tender, firm and non-nodular prostate was noted on digital rectal examination and his prostate-specific antigen level was 32.4 ug/L. Histopathological analysis following transrectal ultrasound-guided prostate biopsy showed an unusual finding, malakoplakia associated with severe chronic active prostatitis. Only a limited number of cases of prostatic malakoplakia have ever been reported in the literature. We present the first ever case of malakoplakia without any evidence of urinary tract infection associated with severe chronic active prostatitis in a black African male. Level of evidence: Level 5.

Introduction Malakoplakia is a rare chronic inflammatory disorder, predominantly affecting the urinary tract. In the head and neck region, it is very rare and may confuse the clinicians during investigations, as features may mimic malignancy. Materials and methods We report a case of malakoplakia involving the parotid gland and review of the reported cases of malakoplakia in head and neck region. Results Histologically, this is the first classic case report of malakoplakia involving the parotid gland in the world literature. A total of 49 cases have been reported in the head and neck region; 38.7% of these are cutaneous. In soft tissue, the tongue is the most common site. Salivary gland involvement is very rare. Previously, submandibular salivary gland involvement has been reported. Conclusion A possibility of malakoplakia should be considered as a differential diagnosis in patients with enlarged head and neck masses. Histology is essential to diagnose this benign inflammatory disorder and to differentiate from a malignant process.

Summary Antibodies targeting uroplakin II (UPII) are highly specific for urothelial cells and are frequently used to determine if a primary bladder lesion or a metastatic lesion originates from the urothelium. However, to date, no studies have tested the expression of UPII in histological mimickers of bladder cancer that are nonurothelial in origin. Given the potential risk of misdiagnosis, immunohistochemical markers are often used to better characterize these lesions. In the present study, we analyzed the immunohistochemical expression of UPII in a set of urothelial carcinoma mimickers that included conventional nephrogenic adenoma (n = 8), papillary nephrogenic adenoma (n = 6), endometriosis/endosalpingiosis (n = 5), inflammatory myofibroblastic tumor (n = 4), ectopic prostate tissue (n = 2), and malakoplakia (n = 2). We also examined the expression of GATA-3, another commonly used immunohistochemical marker in bladder cancer diagnosis, in the same lesions. Weak immunoreactivity for UPII was identified in 6 of 27 mimickers (22%), and GATA-3 was expressed in 16 of 27 mimickers (59%). Strong immunoreactivity for UPII appeared to be a specific marker for urothelial cell of origin, although weak staining was seen in a significant proportion of mimickers. GATA-3 immunostaining was present in a greater number and broader spectrum of mimickers; however, only one case of papillary nephrogenic adenoma showed dual positivity for UPII and GATA-3. These findings support the immunohistochemical panel-based approach in the diagnosis of bladder lesions, especially if nonurothelial bladder cancer mimickers are in the differential diagnosis. Additional larger studies would be of value to expand on these findings.

Introduction/Objective Malakoplakia is a chronic inflammatory disease, usually associated with bacterial infection. The urinary system is the most commonly involved site. Involvement of the female genital tract is extremely rare. Histopathologically, the disease is manifested by solid sheets of histiocytes with abundant granular eosionophilic cytoplasm, and intra- and extracellular basophilic bodies. It should be differentiated from other types of granulomatous inflammation and from signet ring cell carcinoma. Treatment consists of antibiotics, but surgery is sometimes necessary. Methods/Case Report A 74-year-old woman experienced postmenopausal bleeding for several years. A previous endometrial biopsy showed a mucinous glandular proliferation of uncertain etiology. She also had coronary heart disease, hypertension, diabetes mellitus and hyperlipidemia. A prior urinalysis showed abundant bacteria, and she had been hospitalized recently for urinary tract infection (UTI). She underwent laparoscopic total hysterectomy. Grossly, the endometrium was mostly uniform, 0.1 cm in thickness, with one 0.4-cm polyp. Microscopically, the endometrium showed simple hyperplasia without atypia, benign endometrial polyp, and multifocal malakoplakia, manifested by sheets of histiocytes with granular eosinophilic cytoplasm, many of them containing variably-sized basophilic spherules imparting to them a targetoid, or bull’s eye, appearance. Multiple endometrial plasma cells were also seen. Results (if a Case Study enter NA) NA Conclusion Very few cases of endometrial malakoplakia have been reported in the medical literature. Here we present unexpected malakoplakia coexisting with endometrial simple hyperplasia without atypia. Reports from the electron microscopy literature suggest that the basophilic spherules, named Michaelis-Gutmann inclusion bodies, are electron-dense granules that result from coalescence of phagolysosomes, reflecting failed digestion of bacteria. In our patient, longstanding chronic endometritis, confirmed by the presence of endometrial plasma cells, possibly coexisting with chronic UTI, culminated in malakoplakia, which clinically and pathologically mimicked endometrial hyperplasia or even carcinoma, given her old age, chronic bleeding, and the mucinous epithelium in the endometrial biopsy. The finding of endometrial malakoplakia should remind us of the bacterial etiology of chronic endometritis and the need for timely treatment of urogenital infections with antibiotics.

Introduction/Objective Malakoplakia is a granulomatous disease of infectious etiology that affects immunosuppressed individuals. It commonly involves the genitourinary tract, but the involvement of other systems and organs has been reported. Gram-negative bacterial infections are mostly the responsible agent, with Escherichia coli found in approximately 80% of cases. Malakoplakia can present clinically and radiologically as a malignancy. Methods/Case Report We present a case of a 16-year-old male with a history of acute liver failure requiring liver transplantation due to autoimmune hepatitis. He was admitted with concerns for rejection, and he was found to have an abdominal wall mass. MRI studies showed a lobulated, solid, and cystic mass measuring 11.0 x 4.9 x 1.9 cm in the right abdominal wall. The mass was extending through all the layers of the abdominal wall and protruding into the abdominal cavity with mass effect on the liver, encasing the common bile duct, hepatic artery and main portal vein, concerning for malignancy, versus a possible infectious process. Ultrasound-guided FNA and biopsy were performed. The Diff-Quik and Papanicolaou-stained smears showed abundant foamy macrophages with numerous granular intra- and extracellular slightly refractile inclusions (4-10 um), rare multinucleated giant cells, and fibroblasts. A Von Kossa stain highlighted the targetoid inclusions consistent with Michaelis-Gutman bodies. Additional stains were performed on the core needle biopsy, and the histiocytes were positive for CD68 and CD163. Stains for keratins, CD45, HSA, CD1A, and Langerin were negative. No acid-fast or fungal microorganisms were identified on AFB and GMS stains, respectively. The final diagnosis was malakoplakia, with no evidence of malignancy. Results (if a Case Study enter NA) NA Conclusion Fine-needle aspiration cytology is useful in establishing a fast diagnosis of malakoplakia and excluding malignancies.

Attention of the pathologist to basic urinary bladder anatomy and histology is essential to accurately diagnose bladder pathology, as well as determine TNM staging for proper prognostic evaluation. In this chapter, the normal anatomy, histology, and benign pathology of the urinary bladder are reviewed, with emphasis on key points that the surgical pathologist should know. Topics covered herein include bladder cancer staging according to the most recent AJCC Staging Manual, differentiating muscularis propria from hypertrophic muscularis mucosa, gross evaluation and handling of bladder tumors, functional anatomy, urachal remnants, diverticula, exstrophy, ectopic prostate tissue, intestinal and squamous metaplasia, von Brunn nests/cystitis cystica and glandularis, nephrogenic adenoma, polypoid cystitis, types of inflammatory cystitis, malakoplakia, fibroepithelial polyp, and Mullerian lesions. Potential mimickers of malignancy are noted, and characteristics used to differentiate mimickers from malignant lesions are discussed. This chapter is therefore a useful reading for genitourinary pathologists, general surgical pathologists who encounter bladder cases, and pathology trainees.

A broad spectrum of lesions, including hyperplastic, metaplastic, inflammatory, infectious, and reactive, may mimic cancer all along the urinary tract. This narrative collects most of them from a clinical and pathologic perspective, offering urologists and general pathologists their most salient definitory features. Together with classical, well-known, entities such as urothelial papillomas (conventional (UP) and inverted (IUP)), nephrogenic adenoma (NA), polypoid cystitis (PC), fibroepithelial polyp (FP), prostatic-type polyp (PP), verumontanum cyst (VC), xanthogranulomatous inflammation (XI), reactive changes secondary to BCG instillations (BCGitis), schistosomiasis (SC), keratinizing desquamative squamous metaplasia (KSM), post-radiation changes (PRC), vaginal-type metaplasia (VM), endocervicosis (EC)/endometriosis (EM) (müllerianosis), malakoplakia (MK), florid von Brunn nest proliferation (VB), cystitis/ureteritis cystica (CC), and glandularis (CG), among others, still other cellular proliferations with concerning histological features and poorly understood etiopathogenesis like IgG4-related disease (IGG4), PEComa (PEC), and pseudosarcomatous myofibroblastic proliferations (post-operative spindle cell nodule (POS), inflammatory myofibroblastic tumor (IMT)), are reviewed. Some of these diagnoses are problematic for urologists, other for pathologists, and still others for both. Interestingly, the right identification of their definitory features will allow their correct diagnoses, thus, avoiding overtreatment. The literature selected for this review also focuses on the immunohistochemical and/or molecular data useful to delineate prognosis. Sin financiación No data 2021 UEM

Primary eosinophilic, mastocytic, and histiocytic disorders of the gastrointestinal (GI) tract comprise a spectrum of poorly understood and rare entities. Despite their extremely low prevalence in the general population, these diagnoses are commonly considered in the differential diagnosis when evaluating GI biopsies in daily practice. Surgical pathologists will often encounter biopsies with a prominence of one or more of these cell types. The question that then arises is whether the patient actually has one of these rare disorders or the findings are a secondary manifestation of mucosal injury due to an unrelated etiology. The aim of this chapter is to provide a pragmatic approach to interpretation of GI biopsies when faced with an inflammatory process with prominence of these cell types and to highlight our evolving understanding of these disorders that affects clinical practice.

Recurrent bladder perforation due to xanthogranulomatous cystitis with malakoplakia is rare entity and can lead to spontaneous bladder perforation. A 15 years girl presented with sudden pain abdomen with reduced urine output. Her exploratory laparotomy revealed, perforation of 2 cm at the dome of bladder with unhealthy margins. Excisional bladder biopsy and repair of bladder perforation by 3-0 polyglactin suture was done. The histopathology showed xanthogranulomatous cystitis with malakoplakia. Her records revealed the same histopathology in bladder perforation at age of 9 with lost follow-up till age of 15. Exploratory laparotomy and bladder repair should be done to save the patient.

Appendiceal malakoplakia masquerading as a cecal mass is uniquely rare. The presence of an infiltrate of granular eosinophilic macrophages containing Michaelis–Gutmann bodies on histopathology is pathognomonic of malakoplakia. Cutaneous, gastrointestinal and most commonly urogenital malakoplakia is reported in association with an immunocompromised state, infectious, inflammatory and neoplastic processes. Presentation varies from microscopic disease to plaques, nodules, polypoid lesions and small masses. However, a cecal mass postea proven appendiceal malakoplakia deserves special attention. We could not find similar case reports in the English literature. The pathogenesis of malakoplakia is poorly understood, and it is unclear if it is a harbinger of malignancy, a precursor lesion or an inflammatory marker. In the setting of a dominant appendiceal mass, post-treatment endoscopic and tumor marker surveillance is paramount but, however, undefined in contemporary literature.

Malakoplakia is an inflammatory process related to defective macrophage response to bacterial infection. To further characterize the clinicopathologic manifestations of gastrointestinal malakoplakia, 26 cases were identified from 6 institutions. Hematoxylin and eosin-stained slides and available stains were reviewed, and pertinent clinicopathologic features analyzed. Sixteen patients were women (62%). Mean patient age was 64 (range: 24 to 83). Sites included the colorectum (n=23), appendix (n=1), and stomach (n=2). Clinical indications for tissue procurement included screening (n=14), tumor resection (n=5), diarrhea (n=1), adenoma surveillance (n=1), ulcerative colitis flare (n=1), abdominal pain (n=1), and appendicitis (1). All cases featured histiocytes with abundant, pale, eosinophilic cytoplasm focally containing Michaelis-Gutmann bodies. The process frequently involved the mucosa (n=19), with architectural distortion in 13 cases. Lymphoid aggregates were present in 18 cases, which were prominent or obscuring in 11 (all colon biopsies) and provoked concern for lymphoma in 2. Associated findings included adenocarcinoma (n=5), adenoma (n=2), gastric hyperplastic polyps (n=1), chemical gastritis (n=1), collagenous colitis (n=1), and active chronic colitis (n=2). In cases with available stains, Michaelis-Gutman bodies were highlighted by Periodic Acid-Schiff with diastase, Von Kossa, and iron stains. Although 2 cases were positive for Tropheryma whipplei antibody, no T. whipplei transcripts were detected on real-time polymerase chain reaction. All patients with available follow-up are alive and well with no additional instances of malakoplakia. Malakoplakia of the gastrointestinal tract is a benign, incidental finding. Although histologic features in the stomach and colon resections are similar to those at other sites, exuberant lymphocytic response in colon biopsies and immunoreactivity with T. whippleii antibody may provoke initial confusion and lead to unnecessary time and resource investment.

Malakoplakia is a rare, granulomatous disease that affects a wide variety of organs and can have a clinical and radiographic presentation resembling that of malignancy. The genitourinary tract is the most commonly involved site. There are scant reported cases presenting as a locally advanced renal mass and even rarer, diagnosed by fine-needle aspiration (FNA) cytology. We report clinical, imaging, cytologic, and histological findings of an interesting case of malakoplakia initially diagnosed by FNA cytology. We also briefly review the literature and emphasize the importance of recognizing this entity when encountered in an aspirate material, which can help mitigate the diagnostic confusion of malakoplakia for clinicians.

Context. Malakoplakia can be caused by incomplete digestion of Escherichia coli by lysosomes, leading to recurrent urinary tract infections and consequential mass-forming events that mimic tumors. Objectives. By using ultrastructural findings, we aimed to specify the process of phagolysosome to evoke malakoplakia. Design. We observed a series of processes to form a peculiar Michaelis-Gutmann (MG) body in three patients with malakoplakia and compared with xanthogranulomatous pyelonephritis. Results. The ultrastructural findings were realigned according to the sequence of events as pre-phagosomal, phagosomal, and post-phagosomal stages. For the mature MG body, numerous lysosomal aggregates targeting pathogens and subsequent incomplete digestion are prerequisite factors for the pre-phagosomal stage. Scattered lamellated residue is late evidence of the pre- phagosomal stage. Phagosomes can be formed by the fusion of multiple pathogens and multiple lysosomes. We utilized transmission and scanning electron microscopy to speculate on the process of phagolysosomal formation. Conclusion. The recognition of E. coli captured by phagosomes or partially damaged by lysosomal attack within the cell was recorded for the first time. Furthermore, SEM observation was performed on human tissue

The alimentary tract serves as host to a large number of diseases. In the non-neoplastic group of disorders, conventional histochemistry continues to play an important diagnostic role. It is particularly important in recognizing specific infectious diseases, such as Helicobacter gastritis, Whipple disease, intestinal tuberculosis and other forms of mycobacteriosis, malakoplakia, intestinal spirochetosis, fungal enteritides, amebiasis, cryptosporidiosis, isosporiasis, and microsporidiosis. Those conditions and their histochemical properties are discussed in this review, along with the use of histochemistry in the characterization of structural gastrointestinal disorders. The latter include mucosal metaplasias, amyloidosis, glycogenic acanthosis of the esophagus, lymphocytic-collagenous colitis, gastric neuroendocrine hyperplasia, and pill gastritis.

Xanthogranulomatous pyelonephritis (XGP) is a rare chronic destructive granulomatous inflammatory process that affects sporadically the parenchyma of the kidney and the disease tends to be associated in many cases with long-term urinary tract obstruction, urinary tract infection and also in cases with calculi within the kidney. XGP does affect females more commonly in comparison with males, it does affect all age groups including newly born up to the elderly. Majority of the patients tend to be symptomatic and some of the symptoms could include: pyrexia, flank / loin / abdominal pain, palpable mass in loin/abdomen/costovertebral region, weight loss, visible haematuria. There may be associated diabetes mellitus, obesity, or immune deficiency. There may be anaemia and leucocytosis upon blood tests. Urine cultures may grow Escherichia coli, or proteus but in other situations the urine culture could be negative. Ultrasound scan of renal tract, CT and MRI scan of abdomen and pelvis tend to be undertaken in the assessments of patients. CT scan has globally been the establishment of radiology imaging diagnosis. Radiology imaging may demonstrate focal or diffuse XGP but the diffuse form is more common. It may reveal multiple low-attenuated cystic masses in the kidney, calculi in the kidney, hydronephrosis / dilated calyces / bear’s paw appearance in some cases. Macroscopy examination of the specimen of the kidney would tend to show multiple yellow nodules around calyces, and the lesion may form a mass or be infiltrative. Pathology examination of specimens of XGP would tend to show granulomatous inflammatory infiltrate that consists of neutrophils, lymphocytes, plasma cells, xanthomatous histiocytes, as well as multi-nucleated giant cells. Immunohistochemistry staining may show positive staining for CD68. Some of the differential diagnoses include: clear cell carcinoma of the kidney, papillary renal cell carcinoma, sarcomatoid renal cell carcinoma, leiomyosarcoma, malakoplakia of kidney, megalocystic interstitial nephritis, and renal replacement lipomatosis. Majority of diffuse cases have been treated by nephrectomy and antibiotics and some focal cases have been treated by partial nephrectomy and antibiotics but on very rare occasions cases have been treated by drainage of the abscess with biopsy and in conjunction with antibiotics. Percutaneous nephrostomy has also been undertaken pre-operatively to decompress the kidney by draining the abscess in the occasional case.

Malakoplakia is a rare granulomatous, inflammatory disease generally manifesting as ulcers of the urogenital tract, especially in the bladder, but it can occur in any part of the body. Because of its varied clinical presentations, malakoplakia is considered for differential diagnosis upon suspicion. The final diagnosis is confirmed by the presence of Michaelis-Gutmann bodies. We report a case of pelvic malakoplakia accompanied by left lower quadrant pain that was misdiagnosed as endometrial cancer with pelvic mass based on imaging studies. The patient underwent dilatation and curettage, and the pathology report revealed no malignancy. Because of persistent pain and septic shock, she underwent a debulking operation to remove the mass. Histopathologic examination revealed malakoplakia. For postoperative management, she received broad-spectrum antibiotics, but abdominal pelvic computerized tomography performed on postoperative day 9 revealed pelvic mass recurrence. To the best of our knowledge, this is the only rare case report of pelvic malakoplakia mimicking endometrial cancer.

Malakoplakia is a rare condition in which histiocytic cells accumulate within different organs and tissues, sometimes mimicking neoplasia. Gynecologic involvement is extremely rare and therefore may cause relevant diagnostic confusion for both clinicians and pathologists. In this paper, we described the seventh case of ovarian malakoplakia, and we reviewed the literature to compare it with the previously reported ones. Moreover, we investigated the histologic and molecular differential diagnosis of malakoplakia, with special attention to other histiocytic disorders of gynecologic interest. Finally, we discussed the most relevant points with regard to possible pathogenesis and management. Malakoplakia often represents a forgotten entity that should be remembered preoperatively, when approaching a possible gynecologic neoplasia. Moreover, it is of remarkable importance to differentiate malakoplakia from multisystem histiocytosis involving gynecologic organs. All this would prevent misdiagnosis and overtreatment of such a rare but benign condition.

Malakoplakia is an unusual characteristic inflammatory condition, it is distinctive feature is the presence of histiocytic cells with eosinophilic granular cytoplasm, often known as “von Hansemann’s histiocytes”, along with the pathognomonic siderocalcific Michaelis-Gutmann bodies (MGBs). We present a case of malakoplakia within a colonic adenoma seen against a background of Enterococcus infection. To our knowledge, this is a unique presentation which has not previous been described.

Malakoplakia is an unusual acquired granulomatous disease that can affect many systems including urogenital tract. It presents a huge diagnostic challenge as it can mimic malignancy. We report a 55-year diabetic woman who presented with history of macroscopic hematuria and right flank pain. On investigations, ultrasound of kidney, ureter and bladder (KUB) showed right hydronephroureter, and CT KUB showed right moderate hydronephroureter and right ureteric stone. Endoscopic examination revealed multiple white plaques involving urinary bladder and right ureter. The diagnosis of malakoplakia was based on microscopic findings that are specific for its diagnosis.