Ghadeer Mokhtar

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.

Histopathology of steroid-resistant nephrotic syndrome in children living in the Kingdom of Saudi Arabia

Sirs, Steroid-resistant nephrotic syndrome (SRNS) is believed to be associated with a high risk of developing chronic renal failure (CRF). The underlying histopathology usually affects the course of the disease and the response to treatment [1]. Focal segmental glomerulosclerosis (FSGS) was reported as the main cause of SRNS in Western countries [2] as well as in many other parts of the world [3]. However, in black South Africans, membranous nephropathy associated with hepatitis B infection was reported as a main cause of SRNS [4], and minimal change nephrotic syndrome [minimal change disease (MCD)] was reported as the main cause in Kuwaiti children [5]. In this study we report on the pattern of the histopathology of SRNS in children coming to King Abdul-Aziz University Hospital (KAUH). We reviewed the notes of all children with primary nephrotic syndrome that was steroid resistant who had come to the pediatric renal unit at KAUH between 2002 and 2007. Only patients who had undergone 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: 25 girls and 11 boys, with a female (F) to male (M) ratio of 2.3:1. Fifty percent (18 children) were Saudi and the other 50% were from various racial backgrounds (nine Asians, four Arabs, two Africans and three from the Far East). Their mean age at presentation ± standard deviation (SD) was 4.3±3.0 (range 1–12) years. Their mean serum albumin level at presentation was 15.6±7.1 g/l, and all had 4+ proteinuria. Five children had high creatinine concentrations at presentation, while the mean serum creatinine level was 50.4±45.6 μmol/l. Three children had low levels of complements at presentation, and none had positive test results for hepatitis surface antigen or antinuclear antibody (ANA). Renal histopathology was compatible with focal segmental glomerulosclerosis (FSGS) in 39% (14 children), immunoglobulin (Ig)M nephropathy in 28% (ten children), mesangio-proliferative glomerulonephritis (MesPGN) in 17% (six children), MCD in 8% (three children), complement C1q nephropathy (C1qNP) in 8% (three children) and IgA nephropathy in one patient (3%). The incidence and frequency of various histological subtypes of steroid-resistant nephrotic syndrome in children in the Kingdom of Saudi Arabia (KSA) do not seem to differ from those observed in Western and other countries. Therefore, similar diagnostic and therapeutic strategies for SRNS can be applied to our patients in KSA. Genetics studies have to be done in the future. Pediatr Nephrol (2009) 24:1429–1430 DOI 10.1007/s00467-008-1106-5

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 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.

Unusual Case of Post-Infection Nephritis Associated with Nephrin Mutation

NPHS1 gene is the gene responsible for production of Nephrin, which has role in the structure of glomerular filtration barrier. Nephrin located in slit diaphragm, which has a role in preventing passage of plasma proteins out of glomerular capillaries.

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.