Adult-Onset IgA Vasculitis Associated With Pulmonary-Renal Syndrome Following COVID-19 Infection: A Case Report and Literature Review.

Immunoglobulin A (IgA) vasculitis, also known as Henoch-Schönlein purpura (HSP), is an immune complex-mediated inflammation of small blood vessels that leads to tissue destruction with or without organ damage. We described a case of a 41-year-old otherwise healthy female who presented with an ascending rash distributed on both lower extremities and arthralgia. Blood testing revealed high blood urea nitrogen (BUN), creatinine, and inflammatory markers, as well as a negative autoimmune panel. Urinalysis revealed proteinuria and hematuria. A kidney biopsy was performed, which revealed abnormalities. She was started on intravenous (IV) methylprednisolone pulse therapy. Suddenly, she complained of epistaxis and became desaturated. Computed tomography revealed bilateral pleural effusion, and she was transferred to the ICU. Bronchoalveolar lavage was performed and was consistent with an increasing bloodier return. Plasma exchange was performed. The rash and clinical symptoms improved dramatically. This study reports a case of IgA vasculitis based on The European Alliance of Associations for Rheumatology/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society (EULAR/PRINTO/PRES) criteria associated with pulmonary-renal syndrome following a case of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.

Embryonal tumor with multilayered rosettes; rare pediatric CNS tumor. A case report and review of literature.

Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered is a newly designated entity of the embryonal tumors of the central nervous system (CNS) according to the 2016 WHO classification system of CNS. Characteristically, these tumors are newly defined based on their specific molecular genetic amplification in chromosome 19q13.42 found at locus C19MC. To the best of our knowledge, we present the first reported case of ETMR in Saudi Arabian pediatric population. A 2-year-old boy presented to the hospital with generalized tonic-colonic seizure, vomiting, irritability, and inability to walk. Computed tomography (CT) scan showed a large left thalamic supratentorial brain tumor. The tumor measured 6.1 × 5.6 × 5.6 cm and was characterized by cystic changes, prominent vasculature, and calcifications. Histopathology, immunohistochemistry examination, and fluorescence in situ hybridization (FISH) analysis confirmed the diagnosis of ETMR. In addition to reporting this rare case, we provide a brief literature review, treatment options, patient outcome, and disease prognosis.

Cytospin performance when using Paris system for reporting urinary cytology.

Objectives: The Paris System (TPS) for Reporting Urine Cytology has significantly improved the approach to evaluating urine cytology. TPS criteria were defined mainly according to ThinPrep and SurePath preparations, as they are widely utilized. The objective of this study is to validate urine cytology interpretation according to the TPS classification using cytospin technique in relation to the gold slandered histology. Material and Methods: This retrospective study examined and analyzed 316 urine specimens from King Abdulaziz University Hospital between 2015 and 2020. Cytospin technique is performed for all cases. Slides were recategorized using TPS criteria, then compared with the original histology diagnosis. Results: According to the TPS, 108 cases were classified as 101 AUC (32%), 95 NEG (30%), 59 HGUC (18.7%), 31 SHGUC (9.8%), and 30 (9.5%) others. The computed sensitivity of cytospin in urine cytology was 94.7%, with 73.9% specificity, a positive predictive value of 85.6%, a negative predictive value of 89.5%, and overall accuracy of 86.8%. Conclusion: Urine cytology testing is considered to be a non-invasive and sensitive method to screen for urothelial carcinoma. TPS defined standards are reliable on cytospin prepared slides for reporting urine cytology.

Timing of ventriculoatrial shunt removal on renal function recovery of patients with shunt nephritis: Case report and systematic review.

BACKGROUND: Shunt nephritis is autoimmune complex-mediated glomerulonephritis rarely encountered complication following ventriculoatrial shunt (VAS). It's reported to occur after an average of 5.8 years of shunt insertion. We systemically analyzed the literature to know the time of VAS removal that is associated with good renal function recovery. METHODS: We report an unusual case of a 51-year-old female who presented with shunt nephritis 35 years after VAS implantation. This is the longest duration to be reported up to our literature review. A systematic literature review was conducted to assess the factors associated with renal function outcomes in patients with VAS who developed shunt nephritis. RESULTS: Our patient showed a full renal recovery after seven weeks of diagnosis; the atrial catheter was removed, and her shunt was converted to a ventriculopleural shunt (VPLS). Twenty-one articles met the inclusion criteria of our review. Age at shunt insertion of < 3 months is associated with a 66.7% incidence of poor renal outcome (P-value = 0.004). The time from shunt nephritis onset to shunt removal was positively associated with a higher risk of end-stage renal disease or death. A 3-month delay or longer is associated with an odds ratio of 22.4 of poor outcomes (95% confidence interval (CI)=2.2 - 228.7). The time from insertion to nephritis was not significantly associated with the outcome. CONCLUSION: The sooner the shunt is removed within a 3-month interval from the diagnosis of shunt nephritis, the better the outcome, regardless of the time interval from shunt insertion until the development of shunt nephritis.

Phosphaturic Mesenchymal Tumor: A Case Report of a Rare Entity.

Phosphaturic mesenchymal tumor (PMT) is a rare entity that presents as a paraneoplastic syndrome that causes tumor-induced osteomalacia (TIO). Most of these cases were located in the extremities. We report a case of a phosphaturic mesenchymal tumor arising in the left upper thigh. The tumor was discovered during the clinical workup of a patient complaining of osteomalacia symptoms with multiple fractures.

Condyloma accuminatum of the male urethra: A case report.

Condyloma acuminatum is an anogenital lesion caused by the human papillomavirus infection. It is a common, sexually transmitted disease. It usually affects the external genitalia, while urethral and bladder involvement is uncommon. Human papillomavirus types are classified into three categories depending on their oncogenic potential: low risk (type 6, 11, 42, 43, 44, 59, 66, 68, and 70), intermediate-risk (type 30, 31, 33, 34, 35, 39, 40, 49, 51, 52, 53, 57, 58, 63, and 64) and high risk (type 16, 18, 45, and 56). High-risk and intermediate-risk human papillomavirus DNA types, together with other co-factors still to be defined, account for over 90% of anogenital pre-malignant and malignant tumours. Herein, we report a unique case of condyloma acuminatum positive for human papillomavirus -6 involving the penile urethra, presents as the primary and only site of the disease in a diabetic type-2 patient.

Pulmonary alveolar microlithiasis: A report of two unique cases.

Pulmonary alveolar microlithiasis (PAM) is an inherited autosomal recessive disease. PAM is classically characterized by calcium phosphate deposition within alveolar airspaces due to SLC34A2 (solute carrier family 34 member 2) gene mutation located on chromosome 4p15.2. Such cellular genetic mutation would lead to a defect in the sodium-phosphate transporter channel located in alveolar epithelial cells type-II. Ultimately, it would result in a malfunction of alveolar epithelial cells and the failure of these cells to clear-up the released phosphorous particles in the cellular surfactant recycling. PAM is usually diagnosed in adulthood, frequently notable in the third and fourth decades of life, occasionally can be associated with more severe clinical presentation and radiological findings. Nevertheless, the disease could manifest itself in the pediatric age group, which either shows non-specific signs and symptoms or be exclusively asymptomatic. Histopathological examination is the gold standard for the PAM diagnosis. Genetic counseling and testing might benefit the patient's family members. Herein, we present 2 cases of PAM in the pediatric age group, along with their clinical history, presentation, radiological studies, and histopathology findings, as well as a brief literature review.

Prevalence and 20-year epidemiological trends of glomerular diseases in the adult Saudi population: a multicenter study.

BACKGROUND: Recent international reports have shown significant changes in the incidence of different glomerular diseases. OBJECTIVE: Examine temporal and demographic trends of biopsy-diagnosed glomerular diseases in the adult population of Saudi Arabia over the last two decades. DESIGN: Medical record review. SETTINGS: Four tertiary medical centers in Saudi Arabia. PATIENTS AND METHODS: We identified all patients that underwent native kidney biopsy between 1998 and 2017. MAIN OUTCOME MEASURES: The frequency and the disease trends in four biopsy eras (1998-2002, 2003-2007, 2008-2011, and 2012-2017) for different glomerular diseases. SAMPLE SIZE AND CHARACTERISTICS: 1070 patients, 18-65 years of age; 54.1% female. RESULTS: Of 1760 patients who underwent native kidney biopsies, 1070 met inclusion criteria. Focal segmental glomerulosclerosis was the most common biopsy-diagnosed disease, with comparable frequencies over the four eras (23.6%, 19.8%, 24.1%, and 17.1, respectively [ P value for trend=.07]). The frequency of immunoglobulin A nephropathy increased progressively. The incidence of membranoproliferative glomerulonephritis declined significantly. Among the secondary types of glomerular diseases, systemic lupus erythematosus-associated lupus nephritis was the most common, followed by diabetic nephropathy. The prevalence of diabetic nephropathy increased from 1.4% in the first era to 10.2% in the last one. CONCLUSIONS: Trends in biopsy-diagnosed glomerular disease have changed. While focal segmental glomerulosclerosis remains the most common glomerular disease, there has been a significant rise in the prevalence of immunoglobulin A nephropathy and diabetic nephropathy. In contrast, membranoproliferative glomerulonephritis has declined. LIMITATIONS: Retrospective methodologies are vulnerable to lost data. CONFLICT OF INTEREST: None.

A Rare Benign Tumor in a 14-Year-Old Girl.

BACKGROUND: Oncocytomas are the second most common benign renal neoplasm but, unfortunately, they are difficult to differentiate from renal cell carcinoma. Renal oncocytomas are rare and have mostly been reported in adults. To our knowledge, this is only the sixth pediatric reported case of renal oncocytoma worldwide. CASE PRESENTATION: A 14-year-old Yemeni girl with a recurrent history of urinary tract infections came to our clinic complaining of left flank pain with a frontal headache. Ultrasound showed a 3 cm, well-defined echogenic lesion with mild vascularity. This lesion increased in size on her subsequent follow-ups. Computed tomography showed no intralesional fat, vessels invasion, or enlarged lymph nodes. The patient underwent laparoscopic radical nephrectomy, and a pathology report confirmed the diagnosis of renal oncocytoma. CONCLUSION AND RECOMMENDATIONS: We present the rare occurrence of renal oncocytoma in a pediatric patient and highlight the importance of considering oncocytomas in the diagnosis of a renal mass.

Chromophobe renal cell carcinoma of the kidney with neuroendocrine differentiation: A case report with review of literature.

Chromophobe renal cell carcinoma (chRCC) is a distinctive type of malignant kidney tumor characterized by large cells with defined cell membrane. Primary renal neuroendocrine tumors (NET) are rare with morphology similar to NET at other sites. There are few case reports describing the coexistence of these 2 neoplasms within the same tumor mass. We describe a case of chRCC with neuroendocrine features in a 70-year-old male patient who presented with hematuria and right flank pain. The histological and immunohistochemical features of both components were characteristic with no overlapping features. The neuroendocrine element was associated with nodal metastasis.

A clinicopathological study of C1q nephropathy at King Abdulaziz University.

INTRODUCTION: C1q nephropathy is a relatively rare idiopathic glomerulopathy characterized by mesangial immunoglobulin and complement deposits with dominance or co-dominance of C1q, with no evidence of systemic lupus erythematosus. We describe the incidence, clinical manifestation, histopathological features, and follow-up of patients with C1q nephropathy at our institute. MATERIALS AND METHODS: Of 750 kidney biopsy specimens obtained in the period of January 2000 to December 2011, all the cases that meet the criteria for the diagnosis of C1q nephropathy were retrieved.  The histological slides were examined and the clinical charts were reviewed by 2renal pathologists. RESULTS: We had 11 patients, all children, that met the criteria for the diagnosis of C1q nephropathy accounting for an incidence of 1.5%.  The mean age at the time of presentation was 3.7 years and all the patients were presented with nephrotic syndrome. Two patients had microhematuria and 2 had hypertension. Histological examination of these cases showed variable degrees of mesangial cells hypercellularity and matrix expansion with focal segmental glomerulosclerosis observed in 2 cases. Nine patients were steroid resistant (82%) and 2 were steroid dependent. Six patients required immunosuppressive therapy and 1 patient developed end-stage renal disease. CONCLUSIONS: In our series, C1q nephropathy affected predominantly young children. Mesangioproliferative pattern was the most frequent histopathological finding in these patients. Clinically, despite steroid resistance, the patients had a relatively good outcome; the worst prognostic outcome was associated with collapsing glomerulopathy.

Metaplastic carcinoma of the breast: an immunohistochemical study.

BACKGROUND: Metaplastic breast carcinoma is a rare entity of breast cancer expressing epithelial and/or mesenchymal tissue within the same tumor. The aim of this study is to evaluate the clinicopathological features of metaplastic breast carcinoma and to confirm the triple negative, basal-like and/or luminal phenotype of this type of tumor by using immunohistochemical staining. METHODS: Seven cases of MBC were evaluated for clinico-pathological features including follow up data. Cases were studied immunohistochemically by CK-Pan, Vimentin, ER, PR, HER2, basal markers (CK5/6, p63, EGFR, SMA and S-100), luminal cytokeratins (CK8, CK18 and CK19), markers for syncytial cells (ß-HCG and PLAP), as well as prognostic markers (p53, ki-67 and calretinin). RESULTS: The mean age of the patients was 36 years. Three cases showed choriocarcinomatous features. All of our cases were negative for ER, PR and HER2. Six out of the 7 cases showed basal-like differentiation by demonstrating positivity with at least one of the basal/myoepithelial markers. Also 6 out of the 7 cases expressed luminal type cytokeratins (CK8, CK18 and/or CK19). P53 was positive in 3 cases, ki-67 was strongly expressed in only one case, while calretinin was expressed in 6 cases. CONCLUSION: Metaplastic breast carcinoma presents in our population at a younger age group than other international studies. All cases are categorized immunohistochemically under the triple negative group of breast cancer and 86% of them exhibited basal-like and luminal phenotype. Majority of cases developed local recurrence and distant metastasis in a relatively short period of time. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1101289295115804.

Pathological patterns of mesangioproliferative glomerulonephritis seen at a tertiary care center.

Background: Mesangioproliferative glomerulonephritis (MesPGN) is a common morphological pattern that encompasses several groups of renal diseases including IgA nephropathy (IgAN), IgM nephropathy (IgMN), lupus nephritis (LN), C1q nephropathy (C1qN) and other entities. Objectives: The aim of this study was to analyze the pathological findings and the clinical features of cases of MesPGN seen at the king Abdulaziz University, in Saudi Arabia. Patients and Methods: A total of 750 percutaneous native renal biopsies were seen at our institution from January 2000 to December 2011. All the cases diagnosed as MesPGN on light microscopy (LM) were retrieved from the archives of pathology. The pathological features and the clinical data of these cases were reviewed. The clinical data was available for 80 cases only. Results: A total of 103 cases (14%) met the inclusion criteria for the diagnosis of MesPGN. The most common diagnostic entity was IgMN (46.6%) followed by IgAN (30%) along with few cases of class II LN, C1qN, minimal change disease (MCD), Alport's syndrome, focal segmental glomerulosclerosis (FSGS), thin basement membrane disease (TBMD), and fibrillary glomerulonephritis. The most common clinical presentation was nephrotic syndrome seen in 71% of 80 cases, followed by hematuria (14%). Histologically, focal mesangial proliferation was seen in 62% while diffuse proliferation was seen in 38% of the cases. Conclusion: Mesangioproliferative glomerulonephritis is an important cause of nephrotic syndrome in young adults in the western region of Saudi Arabia. Future studies from the region are needed to elucidate the clinical relevance of mesangial cell proliferation to the end stage kidney diseases.

IgM nephropathy: clinical picture and pathological findings in 36 patients.

Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis characterized by a variable degree of mesangial hyper- cellularity and diffuse IgM deposits. This study describes the clinical presentation and the morphological findings in 36 patients, five adults and 31 children, with IgMN. The initial manifestations of the disease were the nephrotic syndrome in 32 patients, proteinuria in two, hematuria associated with proteinuria in 16 and isolated recurrent gross hematuria in two patients. The nephrotic syndrome was steroid responsive in 9% of the cases, steroid dependent in 25% and steroid resistant in 66%. Five patients were hypertensive at the onset of the disease. The mean follow- up period was 3.4 years (range, 1-7 years). One patient developed end- stage kidney disease five years after the diagnosis. On light microscopy, 24 specimens showed mild focal and segmental mesangial hyper- cellularity, 10 cases were normal in cellularity and two cases showed diffuse global mesangial hyper- cellularity. Four cases showed focal and segmental glomerulosclerosis. Mild interstitial inflammation and fibrosis were observed in 11 cases and focal tubular atrophy and hypertensive hyaline arteriosclerosis were noted in three cases. In addition to IgM, the immunofluorescence study showed C3 deposits in 12 cases, IgG in nine cases and IgA in one case. Electron dense- deposits in the mesangium and para- mesangial areas were demonstrated on electron microscopy in 22 cases. In view of its different clinical presentations and the constant findings of diffuse mesangial IgM deposition in all the cases, it is concluded that this form of nephropathy constitutes a distinct entity separate from minimal change disease or focal and segmental glomerulosclerosis. Patients with IgMN had a higher prevalence of steroid resistance and dependence in the proteinuric group.

Role of electron microscopy in evaluation of native kidney biopsy: a retrospective study of 273 cases.

INTRODUCTION: Electron microscopy (EM) has been widely utilized in the evaluation of kidney biopsies. However, few recent reports have critically assessed its diagnostic value. The aim of this study is to assess the role and value of EM in the evaluation of native kidney biopsies at our institution. MATERIALS AND METHODS: A retrospective evaluation of 273 native kidney biopsies performed at our institution over 7 years was done by 2 renal pathologists in order to assess the contribution of EM to the final diagnosis in the knowledge of the light microscopy and immunofluorescence findings. RESULTS: Electron microscopy had an important diagnostic contribution in 39% of cases, in 17% of which EM was essential for diagnosis. Electron microscopy was essential in the diagnosis of minimal change disease, hereditary nephritis, fibrillary glomerulonephritis, and certain classes of lupus nephritis. CONCLUSIONS: In a great percentage of kidney biopsies, it was possible to make the diagnosis with certainty based on light microscopy and immunofluorescence findings alone. However, still there are numbers of cases in which EM is essentially needed to reach definitive diagnosis. Therefore, at least a piece of tissue should be kept for EM in appropriate fixative in each case, which could then be performed at the discretion of the pathologist.

Diagnostic significance of atypical category in the voided urine samples: A retrospective study in a tertiary care center.

BACKGROUND: Voided urine samples continue to play an important role in the surveillance of urothelial malignancy and also as a screening mode for high risk patients. In some cases, it is difficult to reliably distinguish changes induced by inflammation, stone or other reactive condition from neoplasm, and these cases are categorized as atypical. The aim of our study is to evaluate the prevalence and the significance of atypical diagnosis in the voided urine samples and also to identify the cytomorphologic features that are seen more frequently in the atypical malignant urine samples. MATERIALS AND METHODS: All voided urine cytology samples with a diagnosis of atypical urothelial cells, between the period of 2000 and 2009, were obtained from the cytology database. Only those cases with histologic follow-up were included in the study. The cytology and the histology slides were retrieved and reviewed. The following parameters were evaluated: cellularity, cell clusters, nuclear membrane irregularities, hyperchromasia and India-ink type nuclei, the presence of spindle cells and the cytoplasmic characteristics. RESULTS: Out of 72 voided urine samples included in the study, 49 cases (68%) had a positive histologic diagnosis of urothelial malignancy in the follow-up histology; of these (55%) were high-grade urothelial carcinoma. Increased cellularity, papillary cell clusters, nuclear membrane irregularity, hyperchromasia and India-ink type nuclei were observed more frequently in the atypical malignant urine samples, while cytoplasmic vacuolization were seen more in the negative reactive urine samples. CONCLUSION: The atypical category diagnosis is associated with a significant proportion of urothelial carcinoma. It should be used by the pathologist to convey concern to the clinician in difficult cases that may require close follow-up.

Simultaneous onset of steroid resistant nephrotic syndrome and IDDM in two young children.

The cases are reported of two young children who developed insulin-dependent diabetes mellitus (IDDM) within 2 weeks of receiving a diagnosis of nephrotic syndrome. Neither patient responded to 8 weeks of daily prednisolone. The first patient presented at 2 years and 9 months of age. Her renal biopsy showed mesangial proliferation. The second child presented with steroid resistant nephrotic syndrome at 18 months of age and developed IDDM 2 weeks later. He achieved partial remission with cyclosporine therapy. His initial renal biopsy at 3 years of age showed minimal change disease and follow-up renal biopsy at 5 years of age showed early diabetic glomerulosclerosis. Tests for NPHS2 and WT1 genetic mutations were negative in both patients. To our knowledge this is the first report of steroid resistant nephrotic syndrome with almost simultaneous onset of IDDM in young children.

Squamous cell carcinoma of the breast.

Pure primary squamous cell carcinoma of the breast is a rare disease, thus strict criteria should be applied when making such a diagnosis. Breast abscesses are a common presentation. We report a case of primary SCC of the breast in a 69-year-old woman in which the initial clinical impression was a breast abscess. She had an incision and drainage, and cytological and histological examination revealed squamous cell carcinoma. A secondary source was excluded and the patient underwent mastectomy, which was followed by a local recurrence in 4 months.

Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: a single center study.

Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH), Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty-six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18) were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East). Their mean age at presentation was 4.3 +/- 3.0 years (range 1-12 years). The mean serum albumin at presentation was 15.6 +/- 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 +/- 45.6 micromol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA). The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS) in 39% (n=14), IgM nephro-pathy in 28% (n=10), mesengioproliferative glomerulonephritis (MesPGN) in 17% (n=6), mini-mal change disease (MCD) and C1q nephropathy (C1qNP) in 8% each (n=3 + 3) and IgA nephro-pathy in 3% (n=1). Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some children.