Iranian Journal Of Pathology
Volume:11 Issue: 3, Summer 2016

Solitary fibrous tumor (SFT) is a mesenchymal tumor which is most commonly seen in the pleura; however it can be seen in other organs such as the meninge, gastrointestinal tract, soft tissue, bone, and skin. SFT should be differentiated from other mesenchymal tumors in these organs. Immunohistochemistry plays a pivotal role for the histopathologic diagnosis of this tumor. Currently, new markers have been introduced which has been very useful for definite diagnosis of SFT along with other markers in each specific location which are negative in SFT.
Here we review the reported positive and negative immunohistochemical markers of SFT in the English literature with the emphasis on the useful markers in each specific organ. We explored the English literature from 1990 through 2015 via PubMed, Google, and Google scholar using the following search keywords: Solitary fibrous tumor, Solitary fibrous tumor and immunohistochemistry, Solitary fibrous tumor and diagnosis, Solitary fibrous tumor and histogenesis, Solitary fibrous tumor and prognosis, Solitary fibrous tumor and hemangiopericytoma, Solitary fibrous tumor and differential diagnosis, Solitary fibrous tumor and markers.
The most important and valuable positive markers in SFT are CD34, CD99, Bcl-2 and STAT-6.There are consistently negative markers in this tumor as well, used according to the tumor location, such as EMA and S100
Immunohistochemistry is very useful for the diagnosis of solitary fibrous tumor and for its differentiation with other spindle cell mesenchymal tumor in different locations.

Recombinant activated factor VII induces hemostasis in patients with coagulopathy disorders. AryoSeven™ as a safe Iranian Recombinant activated factor VII has been available on our market. This study was performed to establish the safety of AryoSeven on patients with coagulopathy disorder.
This single-center, descriptive, cross sectional study was carried out in Thrombus and Homeostasis Research Center ValiAsr Hospital during 2013-2014. Fifty one patients with bleeding disorders who received at least one dose of Aryoseven were enrolled. Patients’ demographic data and adverse effect of drug and reaction related to Aryoseven or previous usage of Recombinant activated FVII were recorded in questionnaires. Finally data were analyzed to compare side effects of Aryoseven and other Recombinant activated FVII brands.
Aryoseven was prescribed for 51 Patients. Of all participants with mean age 57.18.38 yr, 31 cases were male and 26 subjects had past history of recombinant activated FVII usage. Glanzman was the most frequent disorder followed by congenital FVII deficiency, hemophilia with inhibitors, factor 5 deficiency, acquired hemophilia, hemophilia A with inhibitor, and hemophilia A or B with inhibitor. The majority of bleeding episodes had occurred in joints. Three patients (5.9%) complained about adverse effects of Aryoseven vs. 11.5 % about adverse effects of other brands. However this difference was not significant, statistically.
Based on monitor patients closely for any adverse events, we concluded that Aryoseven administration under careful weighing of benefit versus potential harm may comparable with other counterpart drugs.

Primary infection with BK virus (BKV) is occurred during childhood and usually asymptomatic, but after initial infection, BKV may persist lifelong in the kidney and genitourinary tract. Reactivation may occur in individuals with compromised immunity such as renal transplant recipients. Due to the role of BKV in BK virus-associated nephropathy (BKVAN) and potentially renal allograft rejection, the detection of BKV in renal transplant candidates is very important. The aim of this study was to evaluate the frequency of BK viremia in end stage renal disease cases who were candidates for renal transplantation.
In this cross-sectional study, 50 cases with end stage renal disease who were candidates for renal transplantation were recruited from the main dialysis unit in Tehran, Iran. Presence of BK viremia was determined in plasma samples of cases using real time PCR.
A total of 50 renal transplant candidates with mean age 37.8±13 yr were enrolled in the study. Fifty two percent of subjects were male. Forty six (92%) of them were under HD and 4 (8%) were on PD. BK virus was not detected in any plasma samples of renal transplant candidates.
This study showed absence of BK viremia in our renal transplant candidates. However, due to the important role of BKV in BKVAN and renal graft failure and rejection, further studies involving larger number of cases are required to elucidate the rate of the BKV in renal transplant candidates.

Nowadays, the immune response to hepatitis C (HCV) treatment has become a crucial issue mostly due to the interleukin 28B (IL-28B) polymorphism effects in chronic HCV patients. The aim of this study was to detect the polymorphism of IL-28B gene (rs12979860) in HCV genotype 1 patients treated with pegylated Interferon and Ribavirin.
From the 2010 to 2012, a total of 115 peripheral blood mononuclear cells (PBMCs) of HCV patients who presented to Gastrointestinal & Liver Disease Research Center (GILDRC), Firoozgar Hospital, Tehran, Iran were enrolled in this retrospective cross sectional study. Samples were then categorized based on the presence of sustained virologic response (SVR and no-SVR). Variables including age, gender, serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels of the two groups were investigated based on different IL-28B genotypes.
Analysis by the variables of age and gender showed a mean age ± SD of 42.1±14.0 and gender variability of 44 females (38.2%) and 71 males (61.8%). Adding up these results, the analysis of ALT levels revealed that there was between 293 and 14 mg/ml; AST levels ranged between 217 and 17 mg/ml; the viral load (HCV RNA) ranged between 7,822,000 and 50 IU/ml; the prevalence of CC, CT and TT genotypes were 90.9%, 54% and 25.0%.
IL-28B polymorphism has an effective impact on the therapeutic response to ribavirin and peginterferon combination therapy in chronic HCV patients infected by different genotypes. This polymorphism is crucial in natural clearance.

Hepatitis C virus (HCV) infection is one of the most prevalent infectious diseases responsible for high morbidity and mortality worldwide. Therefore, designing new and effective therapeutics is of great importance. The aim of the current study was to construct a DNA vaccine containing structural proteins of HCV and evaluation of its expression in a eukaryotic system.
Structural proteins of HCV (core, E1, and E2) were isolated and amplified from JFH strain of HCV genotype 2a using PCR method. The PCR products were cloned into pCDNA3.1 () vector and finally were confirmed by restriction enzyme analysis and sequencing. The eukaryotic expression of the vector was confirmed by RT-PCR.
Recombinant vector containing 2241bp fragment of HCV structural genes was constructed.The desired plasmid was sequenced and corresponded to 100% identity with the submitted sequences in GenBank. RT-PCR results indicated that the recombinant plasmid could be expressed efficiently in the eukaryotic expression system.
Successful cloning of structural viral genes in pCDNA3.1 () vector and their expression in a eukaryotic expression system facilitates the development of new DNA vaccines against HCV. A DNA vaccine encoding core-E1-E2 antigens was designed. The desired expression vector can be used for further attempts in the development of vaccines.

Nasal inflammatory disorders such as chronic rhinosinusitis and nasal polyp are among the most prevalent complications with high socioeconomic costs. Vascular Endothelial Growth Factor (VEGF) plays a key role in angiogenesis and cell proliferation. In the present study the effect of VEGF on the development and prognosis of chronic rhinosinusitis and nasal polyp was investigated.
This cross sectional study was performed on the nasal histological specimens of two groups of patients suffering from nasal polyp or chronic rhinosinusitis, and the expression of VEGF in the two groups was compared immunohistochemically. Based on the percentage of VEGF-positive cells the specimens were classified into four scores. Furthermore, the relations between the VEGF expression and some demographic characteristics were evaluated.
The VEGF immunohistochemistry findings indicated a significantly higher expression of VEGF in nasal polyp group compared to chronic rhinosinusitis without nasal polyp group. In terms of VEGF-expression scoring, in both groups most of the specimens were classified as score-2, namely indicating 10-50% of VEGF-positive epithelial cells. In both groups no significant relation between VEGF expression and age or sex of the patients could be seen.
Local modulation of VEGF expression might be taken as a putative therapeutic strategy in management of sinunasal inflammatory disorders, especially nasal polyps.

Brucellosis is an endemic zoonotic disease in the Middle East. This study intended to design a uniplex PCR assay for the detection and differentiation of Brucella at the species level and determining the antibiotic susceptibility pattern of Brucella in Iran.
Sixty-eight Brucella specimens (38 animal and 30 human specimens) were analyzed using PCR (using one pair of primers). Antibiotic susceptibility patterns were evaluated and compared using the E-Test and disk diffusion susceptibility test. Tigecycline susceptibility pattern was compared with other antibiotics.
Thirty six isolates of B. melitensis, 2 isolates of B. abortus and 1 isolate of B. suis from the 38 animal specimens, 24 isolates of B. melitensis and 6 isolates of B. abortus from the 30 human specimens were differentiated. The MIC50 values of doxycycline for human and animal specimens were 125 and 10 μg/ml, respectively, tigecycline 0.064 μg/ml for human specimens and 0.125μg/ml for animal specimens, and trimethoprim/ sulfamethoxazole and ciprofloxacin 0.065 and 0.125μg/ml, respectively, for both human and animal specimens. The highest MIC50 value of streptomycin in the human specimens was 0.5μg/ml and 1μg/ml for the animal specimens. The greatest resistance shown was to tetracycline and gentamicin, respectively.
Uniplex PCR for the detection and differentiation of Brucella at the strain level is faster and less expensive than multiplex PCR, and the antibiotics doxycycline, rifampin, trimethoprim-sulfamethoxazole, ciprofloxacin, and ofloxacin are the most effective antibiotics for treating brucellosis. Resistance to tigecycline is increasing, and we recommend that it be used in a combination regimen.

There is a paucity of information about the oral pathology related articles published in a pathology journal. This study aimed to audit the oral pathology related articles published in Iranian Journal of Pathology (Iran J Pathol)from 2006 to 2015.
Bibliometric analysis of issues of Iran J Pathol from 2006 to 2015 was performed using web-based search.The articles published were analyzed for type of article and individual topic of oral pathology. The articles published were also checked for authorship trends.
Out of the total 49 published articles related to oral pathology, case reports (21) and original articles (18) contributed the major share. The highest number of oral pathology related articles was published in 2011, 2014 and 2015 with 8 articles each and the least published year was 2012 with 1 article. Among the oral pathology related articles published, spindle cell neoplasms (7) followed by salivary gland tumors (5), jaw tumors (4), oral granulomatous conditions (4), lymphomas (4), oral cancer (3) and odontogenic cysts (3) form the major attraction of the contributors. The largest numbers of published articles related to oral pathology were received from Tehran University of Medical Sciences; Tehran (7) followed by Mashhad University of Medical Sciences, Mashhad (6) and Shahid Beheshti University of Medical Sciences, Tehran (5).
This paper may be considered as a baseline study for the bibliometric information regarding oral pathology related articles published in a pathology journal.

Desmoplastic small round cell tumor (DSCRT) is a rare variant of sarcoma with a highly aggressive behavior. It usually affects abdominal cavity and has a male predominance. Its correct diagnosis and treatment is sophisticated and requires an experienced multidisciplinary team. Hereby we present a 25 yrold man from Kerman Province in 2013 with abdominal mass and ascites who underwent sonograghy guided percutaneous needle biopsy which was misleading and inconclusive for diagnosis. Thus an open biopsy was fulfilled which revealed solid nests of small round cells with hyperchromatic nuclei and clear cytoplasm surrounded by a desmoplastic stroma suggestive for DSCRT. The diagnosis was confirmed by positive immunohitochemical reaction for cytokeratin, desmin and neuron specific enolase(NSE).Ultimately the patient underwent chemotherapy on the basis of P6 protocol without surgical debulking.Diagnosis and treatment of DSCRT could be a dilemma due to its rarity, various clinicopathologic mimickers and lack of a consensus about its management.

Round ligament leiomyoma of uterus is rare. It can be presented as inguinal swelling mimicking the inguinal hernia or lymph node. Surgical excision is its curative treatment. Definitive diagnosis is made by histopathological examination. A 32 year old pregnant patient having round ligament leiomyoma as diagnosed histopathologically in Recep Tayyip Erdogan University Hospital in 2014 was presented here as the sixth case in literature.

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion. In laboratory tests she had pancytopenia, positive ANA and Anti DNA (ds), hypocomplementemia, hypertriglyceridemia and high ferritin level. Gradually she had signs of RPGN and ARDS. The patient had no skin and musculoskeletal signs of SLE and no liver failure nor coagulopathy of MAS. Her lymph node biopsy was reported as Castleman syndrome. Unlike other studies, the patient showed MAS before treatment with cytotoxic for lupus nephritis.

Chondromyxoid fibroma (CMF) is a rare benign cartilaginous tumor with a predilection for the bones of lower extremities and about one fourth of the tumors involve the foot.Radiologically, an eccentric lytic lesion with well defined margins is seen in the metaphysis of the bone. We hereby, report an 18 yr old young male who presented to Orthopedic Outpatient Department, JN Medical College, Aligarh Muslim University, India diagnosed with giant cell tumor of thethird metatarsal bone of right foot on radiography but on fine needle aspiration cytology (FNAC) the diagnosis of CMF was made. Preoperative diagnosis of this benign condition helped in doing minimum surgical intervention in the form of curettage along with bone grafting. Histopathology further confirmed the diagnosis of CMF. The case is being discussed to highlight the importance of FNAC to diagnose these uncommon benign bone lesions.

Central giant cell granuloma is a benign, aggressive neoplasm composed of multinucleated giant cells that almost exclusively occurs in the jaws though extra-gnathic incidence is rare.Multifocal CGCGs of the jaws are very rare and suggestive of systemic diseases such as hyperparathyroidism,an inherited syndrome such as Noonan-like multiple giant cell lesion syndrome or other disorders.Very few cases of multifocal CGCGs in the jaws without any concomitant systemic disease have been reported. This paper describes an unusual case reported to the Oral Surgery Department of Dr. D.Y.Patil Dental College & Hospital, Nerul, Navi-Mumbai in 2014ina45-year-old male with multifocal central giant cell granuloma involving maxilla and mandible. The serum alkaline phosphatase, calcium and phosphorus levels were within the normal limits. After complete clinical examination hyperparathyroidism and clinical characteristic of any syndromes such as Noonan-like syndrome and neurofibromatosis were ruled out. Thus this paper reports a non-syndromic multifocal central giant cell granuloma.

Intracranial hemangiopericytomas (HPC) are rare vascular tumors. They account for 0.4% of primary central nervous system tumors. HPC is more commonly located supratentorially and tends to occur in a younger age group, with average age at presentation of 38–42 years. The tumor was found throughout the entire CNS, usually superficially and closely related to the meninges. Moreover, they have a strong tendency for local recurrence and extracranial metastasis. Given the clinical, pathological and imaging similarities between Hemangiopericytoma and angioblastic/anaplastic meningioma and the necessity of differentiating these two (choosing the proper treatment and prognosis), we present a report of meningeal Hemangiopericytoma tumor in a 33-year-old female. Our study suggests that in addition to routine histopathological examination, immunohistochemical study is essential to differentiate it from other differential diagnosis.

Dermatofibrosarcoma protruberans is a relatively uncommon slow growing, locally aggressive fibrous tumor of the skin. It has a prospensity of progressing to fibrosarcomatous change in 5% of the cases. We present a case of a 56 yr old male with presented to the outpatient department of surgery, Sri Siddhartha Medical College, Tumkur with a chest swelling in 2013. FNAC was inconclusive and the mass was excised. On histopathology, areas of benign fibrohistiocytic tumor, dermatofibrosarcoma protruberans and fibrosarcomatous dermatofibrosarcoma were identified in the same tumor. Immunohistochemistry confirmed the diagnosis of DFSP with fibrosarcomatous change. Although, transformed DFSP is more aggressive, the prognosis is influenced by the extent of excision and with wide excision, there may be little increased risk for recurrence and metastasis over that of conventional DFSP.

Meningioangiomatosis is regarded as a rare benign hamartomatous condition mostly involving the cerebral cortex and overlying leptomeninges. A strong association of MA with neurofibromatosis type 2 has been documented in published articles. Herein we report a case of an otherwise healthy 13-year-old boy with no family history or stigmata of neurofibromatosis who presented with intractable seizures. MRI revealed a 2x2 cm mass lesion in the frontal lobe. The patient underwent complete surgical resection of the lesion. Although the primary radiologic impression of the lesion was glioma, pathological evaluation of the resected specimen showed mainly proliferation of meningothelial cells and fibroblast-like cells with many thickened blood vessels, which are typical for diagnosis of meningioangiomatosis. After surgical removal of the lesion, the patient is free of seizures.

Hemangiomas are considered as vascular malformations which are categorized by the type of vascular channel to capillary, cavernous, venous or arteriovenous(1). Their usual locations are soft tissue , cutaneous or subcutaneous tissue and bone especially vertebra(2).However, intradural extramedullary hemangiomas are rare and most of them fall in cavernous type category. Hereby, authors report a woman with spinal intradural extramedullary capillary hemangioma which is exceedingly rare.

Any patient with bilateral lymphadenopathy especially in Indian subcontinent is regarded as suffering from tuberculosis unless proved otherwise. This sometimes leads to unwarranted delay in correct diagnosis and management if there is ignorance regarding other rarer etiologies. Rosai-Dorfman Disease (RDD) may present in the same manner and should always be kept on the back of the mind to help avoid unnecessary empirical anti-tuberculous therapy that is usually prescribed in so called difficult-to-diagnose tuberculosis infections.

The recent report on “The Adverse Effects of Pregnancies Complicated by Hemoglobin H (HBH) Disease” is very interesting (1). Rabiee et al. reported a pregnant case complicated with HBH disease. Indeed, this problem might not common in the Middle East but it is very common in Southeast Asia. The authors hereby would like to share the experience on this topic. In the recent report by Tongsong et al. (2), the maternal outcomes of normal mothers and those with HBH disease were not different. The common identified problems are fetal growth restriction, preterm birth and low birth weight (2).