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.

The effects of kiwifruit dressing on hypertrophic scars in a rabbit ear model.

AIMS: Hypertrophic scars show abnormal responses during healing. These scars, associated with dysregulated growth and excessive collagen formation, can have both functional and cosmetic consequences for patients. The present study evaluated the effects of kiwifruit on hypertrophic scars in a rabbit ear model. METHODS: This study included 13 New Zealand albino rabbits with full thickness wounds down to the cartilage (four wounds per ear; total: 104 scars). Sixteen days after initial wound formation, one ear of each rabbit was treated with daily kiwifruit dressing, while the other ear (control group) was dressed after normal saline irrigation for 10 days. Harvested skin samples were examined for histopathological, morphometric and immunohistochemical results. RESULTS: In comparison with the control group, early kiwifruit dressing significantly reduced the scar elevation index, fibroblast count and dermal collagen organisation. The ratio of collagen type III to total collagen immunoreactivity, inflammation and dermal capillary count increased significantly in the treated group, compared to the untreated controls. CONCLUSION: Based on the findings, early kiwifruit dressing improved the histological features of cutaneous wounds in rabbits. Therefore, this approach may be effective in clinical practice.

Association of immunohistochemical markers of tumor subtype with response to neoadjuvant chemotherapy and survival in patients with muscle-invasive bladder cancer.

PURPOSE: A readily accessible biomarker to identify which patients with bladder cancer are more likely to respond to neoadjuvant chemotherapy (NAC) could help clinicians avoid unnecessary chemotherapy and prevent its subsequent complications in some patients. The primary objective of this study was to investigate the association of immunohistochemical markers of tumor subtype with response to NAC and survival of patients with muscle-invasive bladder cancer (MIBC). MATERIALS AND METHODS: MIBC patients treated with NAC were retrospectively included. The tissue microarrays were assembled from transurethral resection of bladder tumor (TURBT) specimens and immunohistochemistry (IHC) was performed. The association of independent variables, including IHC markers, and clinical covariates with clinical complete response to NAC and with overall survival was assessed by using logistic regression and Cox proportional hazard regression analysis, respectively. Kaplan-Meier curves were plotted for different IHC-based tumor subtypes. RESULTS: Data from 140 MIBC patients treated with NAC were retrospectively reviewed. A total of 63 patients with available TURBT specimens were eligible to be included in the analysis. Our results showed that the IHC signature of KRT5/6(+)/KRT20(-), as a combined marker of basal subtype, was the only covariate significantly associated with complete response to NAC (p=0.037). Moreover, we found no statistically significant differences in overall survival between different IHC-based subtypes (p=0.721). CONCLUSIONS: The IHC expression of KRT5/6 and KRT20, as a readily accessible combined marker, may help us to identify the patients most likely to benefit from chemotherapy. The clinical utility of this marker needs to be established in larger prospective studies.

Data-Driven Discovery of Molecular Targets for Antibody-Drug Conjugates in Cancer Treatment.

Antibody-drug conjugate therapy has attracted considerable attention in recent years. Since the selection of appropriate targets is a critical aspect of antibody-drug conjugate research and development, a big data research for discovery of candidate targets per tumor type is outstanding and of high interest. Thus, the purpose of this study was to identify and prioritize candidate antibody-drug conjugate targets with translational potential across common types of cancer by mining the Human Protein Atlas, as a unique big data resource. To perform a multifaceted screening process, XML and TSV files including immunohistochemistry expression data for 45 normal tissues and 20 tumor types were downloaded from the Human Protein Atlas website. For genes without high protein expression across critical normal tissues, a quasi H-score (range, 0-300) was computed per tumor type. All genes with a quasi H - score ≥ 150 were extracted. Of these, genes with cell surface localization were selected and included in a multilevel validation process. Among 19670 genes that encode proteins, 5520 membrane protein-coding genes were included in this study. During a multistep data mining procedure, 332 potential targets were identified based on the level of the protein expression across critical normal tissues and 20 tumor types. After validation, 23 cell surface proteins were identified and prioritized as candidate antibody-drug conjugate targets of which two have interestingly been approved by the FDA for use in solid tumors, one has been approved for lymphoma, and four have currently been entered in clinical trials. In conclusion, we identified and prioritized several candidate targets with translational potential, which may yield new clinically effective and safe antibody-drug conjugates. This large-scale antibody-based proteomic study allows us to go beyond the RNA-seq studies, facilitates bench-to-clinic research of targeted anticancer therapeutics, and offers valuable insights into the development of new antibody-drug conjugates.

Ameliorative effect of a nano chromium metal-organic framework on experimental diabetic chronic kidney disease.

Metal-Organic Frameworks (MOFs) are a new class of crystalline porous structures which can be used as a novel structure in diverse fields of medical science. Several studies have shown that chromium supplementation can be effective in amelioration of biochemical parameters of diabetes and its renal complications. Therefore, a chromium-containing MOF (DIFc) was synthetized by nanochelating technology in the present study and then its effect on biochemical indices in diabetic rats was evaluated. Diabetes was induced by high-fat diet consumption and streptozotocin (35 mg/kg) injection and then the treatment started 8 weeks after disease induction and continued for 8 weeks. The results showed that DIFc treatment decreased HOMA-IR index, blood urea nitrogen, uric acid and malondialdehyde in plasma samples. This nano MOF also reduced albumin, malondialdehyde and 8-isoprostane in urine specimen, while it increased creatinine clearance. In conclusion, DIFc MOF demonstrated promising results in the present study, indicating that it can be developed and evaluated in future investigations with the aim of designing a novel agent for management of diabetes and its renal complications.

Possible association of vitamin D status with lung involvement and outcome in patients with COVID-19: a retrospective study.

PURPOSE: Vitamin D deficiency has been reported as a key factor in the development of infectious diseases such as respiratory tract infections and inflammatory processes like acute respiratory distress syndrome. However, the impact of vitamin D on the severity and outcome of COVID-19 is still not fully known. Herein, we aimed to evaluate the prognostic role of serum vitamin D concentration on the extent of lung involvement and final outcome in patients with COVID-19. METHODS: Seventy-three subjects with confirmed diagnosis of COVID-19 were investigated in this study. The patients had been admitted to our academic hospital from February 28, 2020 to April 19, 2020. Demographic and clinical data, serum 25(OH)D levels, and findings of initial chest computed tomography were recorded. Linear and binary logistic regression, cox regression and ROC curve tests were used for statistical analysis. RESULTS: The mean age of patients was 55.18 ± 14.98 years old; 46.4% were male. Mean serum 25(OH)D concentration was significantly lower in the deceased (13.83 ± 12.53 ng/ mL compared with discharged patients (38.41 ± 18.51 ng/mL) (P < 0.001). Higher levels of 25(OH)D were associated with significantly less extent of total lung involvement (ß = - 0.10, P = 0.004). In addition, vitamin D deficiency [25(OH) D < 25 ng/mL] was associated with a significant increase in the risk of mortality (hazard ratio = 4.15, P = 0.04). CONCLUSION: This study suggests that serum vitamin D status might provide useful information regarding the clinical course, extent of lung involvement and outcome of patients with COVID-19. However, further studies with larger sample size are needed to confirm these findings.

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.

DIBc nano metal-organic framework improves biochemical and pathological parameters of experimental chronic kidney disease.

BACKGROUND: The growing morbidity and mortality rate of chronic kidney disease (CKD) has forced researchers to find more efficient strategies for controlling this disease. Studies have proven the important role of alteration in iron, zinc and selenium metabolism in CKD pathological process. Nanotechnology, through synthetizing nano metal-organic framework (NMOF) structures, can be employed as a valuable strategy for using these trace elements as the key for modification and improvement of CKD-related pathological events. After proving the anti-diabetic property of DIBc NMOF (which contains selenium and zinc) in the previous study, the impact of this NMOF on some important biochemical and pathological parameters of CKD was evaluated in the current study. METHODS: Knowing that diabetic nephropathy (DN) is the leading cause of CKD, male wistar rats were selected and given a high fat diet for 2 weeks and then were injected with streptozotocin (35 mg/kg) to induce DN. Six weeks after streptozotocin injection, DIBc or metformin treatment started and continued for 8 weeks. RESULTS: Eight weeks of DIBc treatment decreased plasma fasting blood glucose, blood urea nitrogen, uric acid, malondialdehyde (MDA) and HOMA-IR index compared to DN control and metformin groups. This NMOF significantly reduced urinary albumin excretion rate, MDA and 8-isoprostane, while it increased creatinine clearance in comparison to the above-mentioned groups. Renal histo-pathological images indicated that DIBc ameliorated glomerular basement membrane thickening and wrinkling, mesangial matrix expansion and hypercellularity and presence of intra-cytoplasmic hyaline droplets in proximal cortical tubules of kidney samples. CONCLUSION: The results showed the therapeutic effect of DIBc on important biochemical and histo-pathological parameters of CKD, so this NMOF could be regarded as a promising novel anti-CKD agent.

Vitamin D Treatment Attenuates Heart Apoptosis After Coronary Artery Bypass Surgery: A Double-Blind, Randomized, Placebo-Controlled Clinical Trial.

BACKGROUND: Vitamin D plays an important role in immune system and in the regulation of inflammatory cytokines. Coronary artery bypass graft (CABG) with cardiopulmonary bypass (CPB) is associated with an extensive inflammatory response. The aim of this study is to examine the effect of vitamin D treatment on the apoptosis and inflammatory changes developed after CABG. METHODS: This trial was conducted on 70 patients undergoing CABG with CPB. Patients were randomly administered either in placebo or in the group of orally consuming 150 000 IU vitamin D daily for 3 consecutive days before surgery. The right atrium sample was taken to assess caspases 2, 3, and 7 activity using immunohistochemistry method. The serum level of interleukin-10 (IL-10) and insulin-like growth factor 1 (IGF-1) were compared at intervals. RESULTS: The average number of positive cells for caspases 2 and 3 were less in vitamin D group (P = .006 and P < .001, respectively). There was an increase in serum levels of IL-10 after 3 days from vitamin D treatment before surgery (vitamin D group = 4.4 ± 4.9 ng/mL and control group = 1 ± 0.5 ng/mL, P = .001). After operation, IL-10 increased in both groups, higher level in vitamin D group (P < .001). The comparison of serum IGF-1 showed significant difference after 3 days (P = .006) and remained higher in vitamin D group after CPB (P < .001). CONCLUSIONS: These findings suggest the apoptosis rate after CPB can be reduced by vitamin D. Vitamin D treatment may improve the inflammatory status before and after surgery. Further studies are needed to confirm the antiapoptotic property of vitamin D and clinical implication.

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.

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.

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.

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.

Microbiological Profile of Corneal Ulcers at a Tertiary Referral Center.

The aim of this study was to describe patient demographics, microbiological profile, and antibiotic susceptibility of corneal ulcer at a tertiary referral center to improve and optimize diagnosis and treatment of this potentially blinding entity and to reduce antibiotic misuse. Detailed external and slit-lamp bio-microscopic examination of 123 consecutive patients with suspected corneal ulcer was performed at an ophthalmology clinic. Corneal scraping was carried out under slit-lamp bio-microscopy. The obtained material was inoculated on culture media and smeared on a slide for Gram's staining for morphological identification of bacteria and fungus. For samples that developed colony in culture media, antibiotic susceptibility testing was performed. In a significant percentage of patients (72%) neither bacterial agents nor fungi were the cause of corneal ulcer. Of the 34 culture-proven corneal ulcers, in 79% of the cases, bacteria were detected while in 21% of cases, fungi were found. Of the 27 bacterial corneal ulcers, the majority were (67%) caused by Gram-positive bacteria, of which 50% were Streptococcus pneumoniae, and in the Gram-negative bacterial corneal ulcers, most of the cases (44%) were caused by Pseudomonas aeruginosa. In the antibiotic susceptibility report, Streptococcus pneumoniae, Staphylococcus aureus, Pseudomonas aeruginosa, and Escherichia coli were resistant to Cotrimoxazole (TS), Streptococcus pneumoniae to Erythromycin (E), Staphylococcus aureus to Peniciline (PG), Pseudomonas aeruginosa to Ceftriaxone (CRO) and Nitrofurantoin (NI), and finally, Escherichia coli to Gentamicin (GM). In conclusion, in a significant number of the patients neither bacterial agents nor fungi were offending microorganisms and bacteria were the most common agent of microbiological corneal ulcer, found in 79% of culture-proven corneal ulcers, followed by fungus, found in 21% of culture-proven corneal ulcers.

Mesenchymal Chondrosarcoma of the Orbit Attached to the Optic Nerve.

Mesenchymal chondrosarcoma (MCS) is a rare tumor in the orbit. Although optic nerve displacement is a common finding in intraorbital MCS, optic nerve tissue involvement in tumor has rarely been reported in huge tumors associated with intracranial extension. Herein the authors report a patient with MCS involving optic nerve tissue without intracranial extension. A 59-year-old woman with a 2-month history of progressive proptosis and normal vision presented to us. Computed tomography revealed a clearly outlined heterogeneous mass with calcified foci in its center, which was attached to the optic nerve, magnetic resonance imaging showed the mass to be isointense to gray matter on T1- and T2-weighted images. She underwent lateral orbitotomy and partial tumor excision. Histopathologic study confirmed MCS. She refused exenteration till 1 year but the tumor recurred and her vision decreased to no light perception. Then exenteration was performed with obtaining free margin and she is now free of tumor after 6 months without radiotherapy or chemotherapy. Mesenchymal chondrosarcoma must be differentiated from more common calcified tumors attached to optic nerve like meningioma.

C3 Glomerulonephritis With Multiple Mutations in Complement Factor H.

Complement C3 glomerulopathy refers to a disease process in which abnormal control of complement activation or degradation results in predominant C3 fragment deposition within the glomerulus and causes glomerular damage. Abnormal control of the complement alternative pathway is a well-established risk factor for the occurrence of C3 glomerulonephritis. It is the first reported case in Iran with multiple mutations in complement factor H, with one of these mutations we have expected in hemolytic uremic syndrome rather than C3 glomerulopathy Genetic analysis showed that the molecular abnormalities of factor H led to complement factor H malfunction that were polymorphous and not restricted to the C-terminal domains of the protein.