Superior Oblique Muscle Extramedullary Plasmacytoma in a Patient with Multiple Myeloma and a Review of Literature.

Introduction: Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle. Case Presentation: A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation. Conclusion: Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.

Bilateral squamous cell carcinoma in horse shoe kidney.

To present a patient with horseshoe kidney and bilateral squamous cell carcinoma (SCC) which has not been reported so far. A 61-year-old woman presented with abdominal mass and recent episodes of gross hematuria. Imaging revealed malignant lesion of lower calyces of the right kidney and isthmus of horse-shoe kidney with midline crossing to the left side. Finally, the patient underwent bilateral enbloc radical nephroureterectomy and pathology evaluation was compatible with bilateral squamous cell carcinoma. This is the first report of bilateral SCC in horseshoe kidney which was managed via open enbloc radical nephroureterectomy.

Female Urethral Cavernous Hemangioma: Introducing Treatment and Follow-Up Course of Three Cases.

Cavernous hemangioma is a benign vascular tumor occurring in all parts of the urinary system, including the kidney, bladder, prostate, ureter, and rarely urethra. Urethral cavernous hemangiomas are mostly seen in male patients, and only a few cases of female urethral hemangiomas are reported. Herein, we present the management and follow-up course of 3 cases of female urethral cavernous hemangioma. All 3 cases were menopause women complaining of lower urinary tract symptoms. Definitive diagnosis is made by histopathologic evaluation. In case of large or pedunculated masses, initial surgical resection is highly recommended. Regular follow-up of patients in order to prevent any recurrence is suggested.

Achieving a one-year-tumor-free survival in a female with primary large cell neuroendocrine carcinoma of the urinary bladder and liver metastasis; a case report.

Primary neuroendocrine tumor (NET) of bladder is rare. It has four subtypes, and large cell neuroendocrine carcinoma (LCNEC) is the rarest. LCNEC affects mostly men over 60. Most common symptom is gross hematuria. It has no specific treatment. Metastasis is common and once occurred, average survival would be less than three months. Herein we present diagnostic and therapeutic management of a 65-year-old female with LCNEC of bladder and concurrent high-grade urothelial carcinoma. Despite developing early liver metastasis, she achieved a one-year tumor-free survival.

Primary Epidermoid Cyst of the Clitoris in Adult Female Population: Three Case Reports and Introducing a Safe Surgical Approach.

Epidermoid cyst (EPC) of the clitoris is a very rare cause of non-hormonal acquired clitoromegaly. Clitoral EPCs are extremely uncommon without prior history of genital surgery, trauma, circumcision, or piercing. Surgical removal with special care to avoid compromising neurovascular bundle of the clitoris is the preferred treatment. To our best knowledge, only three cases of adult female clitoral EPC without history of genital surgery, female circumcision, or medications including oral or implantable contraceptives have been reported. Herein, we describe three cases of primary EPC of the clitoris, their management, unique histopathology report, safe surgical approach, and their follow up course.

BCc1 Nanomedicine Therapeutic Effects in Streptozotocin and High-Fat Diet Induced Diabetic Kidney Disease.

BACKGROUND: One common feature of chronic diseases, such as cancer, diabetes and chronic kidney disease (CKD), is the disruption of iron metabolism and increase in labile iron pool, which can result in excessive production of harmful oxidative stress. The proper management of iron metabolism in this situation can be a valuable tool to ameliorate pathological events. MATERIALS AND METHODS: In the previous studies, the anti-neoplastic effects of BCc1, a nanochelating-based nanomedicine with iron-chelating property, were demonstrated in cell culture, animal models and clinical trials. In the present study, the therapeutic effects of BCc1 in animal model of diabetic kidney disease (DKD), induced by streptozotocin injection (35 mg/kg) and high-fat diet consumption, were evaluated. RESULTS: The results showed that BCc1 significantly decreased HOMA-IR index, uric acid, blood urea nitrogen, malondialdehyde and 8-isoprostane. In addition, it reduced urinary albumin excretion rate and albumin-to-creatinine ratio in comparison to DKD control rats. This nanomedicine had no negative impact on liver iron content, hemoglobin level, red blood cell count, hematocrit and mean corpuscular volume, while it significantly decreased aspartate aminotransferase and alanine aminotransferase compared to DKD control group. Moreover, the histopathological assessment indicated that lesser glomerular basement membrane and wrinkling, mesangial matrix expansion and pathological changes in proximal cortical tubules were seen in the kidney samples of BCc1-treated rats. CONCLUSION: In conclusion, BCc1 as an iron-chelating agent shows promising impacts in DKD animal model, which can ameliorate biochemical and pathological events of this disease.

Diagostic Accuracy of C4d-IHC in Diagnosis of MGN.

INTRODUCTION: Membranous glomerulonephritis (MGN) is the most common cause of nephrotic syndrome in adults. The gold standard techniques for diagnosis of MGN are based on a constellation of findings given by light microscope, electron microscope (EM), and immunofluorescence (IF). Occasionally, only formalin-fixed tissues are available for the analysis by light microscopy, which have limitations in differentiating minimal change diseases from MGN. Recently, the usage of C4d immunohistochemistry (IHC) has been proposed for the diagnosis of MGN. The aim of this study was to evaluate the accuracy of C4d-IHC in diagnosis of MGN. METHODS: The present investigation conducted on patients with nephrotic syndrome who underwent renal biopsy in Labbafinejad hospital, from 2016 to 2017. The entire specimens were examined by light microscope, immunofluorescence, and electron microscope as a gold standard method for diagnosis of MGN. The samples were then stained for C4d immunohistochemical analysis. Eventually, the sensitivity, specificity, positive, and negative predictive value for C4d-IHC was determined. RESULTS: The sensitivity and specificity of the C4d-IHC in order to differentiate MGN from other glomerulopathies were 95% and 87.5%, respectively. In addition, the negative and positive predictive values were 97.2% and 79.16%, respectively. CONCLUSION: It was ultimately attained that C4d-IHC has more accuracy in identification and diagnosis of MGN, in contrary to EM and IF, this method is more usable and cost effective, which requires a lower level of skill and advanced equipment. Indeed, this technique does not require fresh specimen.

Effects of the Human Amniotic Membrane on the Cartilage Graft: Prognosis and Absorption in White Rabbits.

BACKGROUND: Cartilage grafts are generally accepted for the restoration and reconstruction of nasal contours. The main concern that plastic surgeons may need to address after surgery pertains to the resorption and disfigurement of the grafted cartilage, especially in allogenic and heterogenic grafts. METHODS: A total of 12 white rabbits were divided into three groups according to the types of graft including autograft, allograft, and heterograft. We used three shapes of grafts, including block, crushed, and diced cartilage in the upper, middle, and lower rows. However, in each rabbit, these grafts were divided into two columns of wrapped and unwrapped grafts, with human amniotic membrane (HAM) grafted on each side of the rabbit's back. RESULTS: In total, 60 specimens underwent histopathological examination. No inflammation was observed in about 50% of the block-shaped conchal cartilages with HAM, and in 50%, less than 25 inflammatory cells per unit were seen. The prognosis and absorption of autograft specimens in block-shaped cartilages with HAM were significantly better compared with other shapes of cartilages with HAM and without HAM. The proliferation rate of fibroblasts in autograft and allograft specimens was more than that in heterograft specimens with HAM. CONCLUSION: Our findings have demonstrated the new role of HAM in clinical applications, indicating that HAM may be used as a low-cost, easily accessible alternative for wrapping in cartilage grafts instead of fascia or surgicel in early future. It is useful for improving the long-term outcomes and decreasing the resorption rate.

TLR-2, TLR-4 and MyD88 genes expression in renal transplant acute and chronic rejections.

BACKGROUND: Graft rejection due to alloreactivity is still the main obstacle to successful renal transplantation. Toll-like receptors (TLRs), which are significantly involved in initiating inflammation, triggering innate immunity, occurrence of ischaemia reperfusion injury (IRI) and subsequent deterioration of allograft function, are of interest in molecular diagnosis of graft rejection. METHODS: In present research, we have evaluated the mRNA expressions of TLR-4, TLR-2 and myeloid differentiation primary response gene 88 (MyD88) in peripheral blood mononuclear cells (PBMCs) and biopsy samples of 26 stable graft function (SGF), 14 acute T-cell-mediated rejection (ACMR), six acute antibody-mediated rejection (AAMR), 10 chronic T-cell-mediated rejection (CCMR) and four chronic antibody-mediated rejection (CAMR) cases of renal transplant recipients, using TaqMan detector real-time polymerase chain reaction (RT-PCR). RESULTS: It was found that TLR4 mRNA level was significantly elevated in PBMCs of both ACMR (P.v: 0.025) and CCMR (P.v: 0.007) cases, while TLR2 gene was upregulated only in PBMCs of ACMR (P.v: 0.024). Moreover, MyD88 expression was increased in biopsy samples of all rejection groups AAMR (P.v: 0.032), ACMR (P.v: 0.002), CAMR (P.v: 0.038) and CCMR (P.v: 0.013) and could distinguish them from stable grafts with AUC (area under curve) of 0.81, 0.80, 0.83 and 0.77, respectively. CONCLUSION: These data showed that MyD88 gene upregulation in renal tissue could have diagnostic value and increased level of TLR4 mRNA in PBMCs could be suggestive of cell-mediated rejections. Therefore, monitoring the expression level of inflammatory signalling genes might be useful in predicting allograft rejection.

Circulating miR-20a and miR-26a as Biomarkers in Prostate Cancer

Objective: Circulating microRNAs (miRNAs), present in body fluids, have been considering importance as cancer biomarkers. The primary aim of this study was to assess whether circulatory miR-20a and miR-26a can be used as diagnostic biomarkers in prostate cancer (PCa). Methods: Relative expression miR-20a and miR-26a has been assessed in 40 patients with PCa and 40 non-cancerous volunteer. Sample Collection of patients was performed before and one week after prostatectomy. Total RNA was extracted from serum and miR-20a and miR-26a expressions were quantified by using Real-Time PCR method. Results: miR-20a was significantly up-regulated in pre-operation serum samples of PCa patients compared to the serum samples of non-cancerous controls, however, in post-operation samples no significant differences was showed. miR-26a level was not significantly decreased in pre and post-operation serum samples compared to the serum samples of controls. However, the expression level ratios of both miR-20a and miR-26a were insignificantly decreased when post-operation serum samples compared to pre-operation ones. Conclusion: Decrement of circulating miR-20a and miR-26a in patients after surgery may reflect the tumoral origin of those microRNAs and the results may use for tumor remnant monitoring after prostatectomy.

Novel Mutations in TACSTD2 Gene in Families with Gelatinous Drop-like Corneal Dystrophy (GDLD).

In the current study, we conducted a mutation screening of tumor-associated calcium signal transducer 2 (TACSTD2) gene in six consanguineous Iranian families with gelatinous drop-like corneal dystrophy (GDLD), in order to find the causative mutations. Detailed eye examination was performed by ophthalmologist to confirm GDLD in patients. To detect the possible mutations, direct Sanger sequencing was performed for the only exon of TACSTD2 gene, and its boundary regions in all patients. In the patients with GDLD, the corneal surface showed lesions with different shapes from mild to severe forms depending on the progress of the disease. The patients showed grayish corneal deposits as a typical mulberry form, corneal dystrophy along with corneal lipid deposition, and vascularization. Targeted Sanger sequencing in TACSTD2 gene revealed the causative mutations in this gene in all studied families. Our study expanded the mutational spectrum of TACSTD2 which along with the related symptoms could help with the diagnosis, and management of the disease.

Evaluating the Prevalence of the Epidermal Growth Factor Receptor in Transitional Cell Carcinoma of Bladder and its Relationship With Other Prognostic Factors.

BACKGROUND: The most common malignancy in the urinary system has been bladder cancer and the most predominant histologic subtype has been transitional cell carcinoma (TCC). There were many molecular risk factors, related with poor prognosis. One of these factors was expression of epidermal growth factor receptor (EGFR). OBJECTIVES: The aim of this study was to evaluate the prevalence of the epidermal growth factor receptor in transitional cell carcinoma of bladder and its relationship with other prognostic factors. PATIENTS AND METHODS: This analytic descriptive study has performed with 61 patients with TCC of bladder after radical cystectomy whom have been hospitalized in Labbafinejad hospital in Tehran, Iran between 2007 and 2010. We have used Chi-square and t-test to analyze our data samples. RESULTS: Records of 61 patients have studied. Fifty three of the total samples were positive for EGFR expression (86.9%). Fifty samples of these fifty-three belonged to men and three others were women's samples (P = 0.46). Among the group with EGFR expression the results were as follows: 25 patients (47.2%) were 60 years old or less and 28 patients (52.8%) were older than 60 (P = 0.023), 16 patients (30.2%) had invasion to lamina properia, and the rest of them had invasion to deeper layers (P = 0.56). For most patients we could not determine the invasion of tumoral cells into the lymph nodes (Nx) (P = 0.067). Thirty four patients (64.2%) had not lymphovascular invasion (P = 0.44) and in forty three of patients (81.1%), perineural invasion have not seen (P = 0.23). Finally, 36 patients (67.9%) were grade 3 (P = 0.27). CONCLUSIONS: In this study we have concluded that most patients had EGFR positive expression. Also, except for the age, there was not any significant relation between expression of EGFR and the other prognostic factors such as, gender, invasion of the tumor into the layers, involving the lymph nodes, lymphovascular or perineural invasion, and grading.

Polyomavirus Hominis 1(BK virus) Infection in Prostatic Tissues: Cancer versus Hyperplasia.

PURPOSE: Polyomavirus hominis 1, better known as BK virus (BKV) infection might be a predisposing factor for prostate cancer (PCa). The aim of this study was to compare the frequency of BK virus infection in pathological specimens of patients with PCa compared to patients with benign prostatic hyperplasia. MATERIALS AND METHODS: From July 2011 to June 2012, paraffin-embedded tissue blocks of patients with PCa (60 specimens) and also with benign prostatic hyperplasia (60 specimens) were investigated. After DNA purification, existence of virus nucleic acid was assessed by polymerase chain reaction. RESULTS: Viral DNA was identified in 9 patients (15%) with benign prostatic hyperplasia (BPH) and 17 patients (28%) with PCa (P = .076). In patients with PCa, viral DNA was observed more often in those with lower total Gleason scores (P = .045). CONCLUSION: The frequency of BK virus infection in PCa patients was higher than BPH patients. BK virus was more often observed in patients with lower Gleason scores. Less detection of BK virus DNA in overt cancer may prove the activity of the virus which paves the way for tumorigenic transformation at early stages of PCa.

Soluble major histocompatibility complex class I chain-related antigen A level in chronic allograft dysfunction.

INTRODUCTION: Soluble major histocompatibility complex class I chain-related antigen A (soluble MICA) has recently been considered as an inhibitory molecule which is shed from tumors and protects them against natural killers and some subgroups of T cells' cytolysis. In transplantation, soluble MICA is also a foreign antigenic molecule that can induce allospecific responses. This study aimed to clarify its possible role in long-term kidney allograft outcome. MATERIALS AND METHODS: Thirty patients with biopsy-proven chronic allograft dysfunction (CAD) were pair-matched with kidney allograft recipients with 30 stable graft function. Fifteen healthy individuals were enrolled as controls. Soluble MICA antigen and anti-HLA antibodies were measured in their serum. RESULTS: There was no significant difference between CAD patients, stable recipients, and healthy volunteers in frequency or titer of soluble MICA; however, soluble MICA-positive patients were more frequent in the stable group was than the CAD group (43.4% versus 33.3%). In addition, a high level of soluble MICA was accompanied by enhanced humoral responses. No significant difference was found in anti-HLA antibodies production between the CAD and stable groups. CONCLUSIONS: Our data suggest that soluble MICA, at least in a defined range, can protect the allograft against natural killers and T cell cytolysis; nonetheless, its excessive amounts might stimulate immune system to exert enhanced humoral response. In order to confirm the protective or detrimental role of soluble MICA in kidney transplantation, conducting larger studies is necessary.

Analysis of relation between C677T genotype in MTHFR gene and prostatic cancer in Iranian males.

Methylenetetrahydrofolate reductase (MTHFR) enzyme is one of the most important enzymes with a pivotal role in the folate metabolism and DNA synthesis pathways. Single nucleotide polymorphism (SNPs) in the coding gene has been related to many medical diseases as well as diverse malignancies including the prostate cancer which is the leading cause of the cancer deaths in men and one of the major public health problems. The goal of this study is to determine the relationship between the MTHFR C677T SNP and the prostate adenocarcinoma in Iranian males attending to the Labbafi-nezhad hospital in Tehran. In this Case-control unmatched study, 67 and 75 paraffinized tissue samples were taken out of the specimens diagnosed previously as the prostatic adenocarcinoma and nodular prostatic hyperplasia for the case and control groups respectively. MTHFR C677T genotyping was done by the use of multiplex ARMS-PCR and frequencies of the alleles were compared between the case and control groups as well as calculating the deviation from Hardy-Weinberg equilibrium and Odds Ratio for the "T" allele regarding the prostatic carcinoma. The observed rates in the control group were not too different from that of expected from Hardy-Weinberg equilibrium (P=0.407). Frequencies of the possible genotypes were as follows: CC, 43.28% vs. 42.67%; CT, 49.25% vs. 52% and CT, 7.46% vs. 5.33% in the case and control groups respectively (P=0.85). 1.37 times increased risk was found for the homozygote carriers of C677T variant (OR: 1.37, 95% CI: 0.33-5.6; P=0.653) which is however statistically not significant. No association has been evident between the MTHFR 677C>T polymorphism and the risk of prostatic carcinoma in this study confirming the findings of some of the previous attempts; however, (OR: 1.37, 95% CI: 0.33-5.6) implies a slight effect of the homozygote on the carcinogenesis. Thus larger studies especially with a greater number of the smaples are recommended.