North American Journal of Medicine and Science
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Malignant Pelvic Solitary Fibrous Tumor: Histologic and Immunohistochemical Features of a Rare Case with Literature Review
No full textSolitary fibrous tumor (SFT) is a rare mesenchymal neoplasm. First described as a pleural lesion, SFTs have been reported in extra-thoracic sites as well. SFT arising in the pelvis is extremely rare, and its malignant form is even rarer. Here we report a case of malignant pelvic SFT with analysis of its histologic and immunohistochemical features. Clinically, the tumor was slow-growing and remained unchanged in size for six years. However, a sudden increase in size by 2 cm occurred within a few months. Gross examination of the surgically excised tumor showed a well-encapsulated tan white mass. Microscopically, the tumor was composed of interlacing bundles of spindle cells with focal high cellularity, moderate cytologic atypia and a mitotic count up to 12/10 HPF. Scattered collagen bundles and staghorn blood vessels were noted. Immunostains revealed that the tumor cells were strongly positive for CD34, CD99, Bcl-2 and vimentin, and negative for smooth muscle actin, muscle specific actin, pancytokeratin, S100, CD117, CD68 and calretinin. Ki67 stain indicated a very high proliferation index. These features are consistent with malignant SFT. Malignant SFTs have a much worse prognosis compared with its benign counterpart, and hence it is important to recognize this entity. We emphasize that histologic features, such as increased mitotic activity, high cellularity and nuclear pleomorphism, as well as a panel of immunohistochemical stains, especially CD34 and CD99, are invaluable in the diagnosis of malignant SFT
Nodal Marginal Zone Lymphoma with Aberrant CD10 Expression: A Case Report and Literature Review
No full textCD10+ nodal marginal zone lymphoma is very rare, with only one case report in the English literature currently. Here we report a case of nodal marginal zone lymphoma with strong CD10 expression occurring in a 76 year-old female with extensive lymphadenopathy. The lymph node biopsy showed a population of small lymphocytes diffusely infiltrating into the interfollicular areas and colonizing disrupted follicles. Immunohistochemical staining revealed that these small lymphocytes were positive for CD20, PAX-5, Bcl-2 and bright CD10. Bcl-6, Cyclin D1 and CD43 were negative in this population of cells. In contrast, the residual germinal center B-cells demonstrated expression of CD10 and Bcl-6 and negative staining for Bcl-2. Flow cytometry study demonstrated a CD10+CD20+ kappa-restricted B-cell lymphoma. FISH studies for translocation of IgH, Bcl-2 and Bcl-6 were negative. Therefore, a diagnosis of CD10+ nodal marginal zone lymphoma was established. This rare disease entity illustrates a possible diagnostic pitfall within the group of mature small B-cell lymphomas, especially for follicular lymphomas with a diffuse pattern. The clinical significance of CD10 positivity in nodal marginal zone lymphomas is unknown. More cases and additional clinical follow up are required to address this question
Hyaline Globules in Epithelial Neoplasm of Kidney
No full textHyaline globules (HGs) are eosinophilic inclusion bodies found in many benign and malignant tumors as well as in non-neoplastic tissues. It has been reported that renal epithelial tumor cells may contain variable amounts of HGs that can be stained red by Masson’s trichrome. Intracytoplasmic HGs are occasionally found in renal cell carcinoma (mainly clear cell and papillary RCC) and are thought to be associated with high Fuhrman nuclear grade and metastasis. In contrast, extracellular HGs most often occur in benign epithelial tumors such as oncocytoma. The biological significance and nature of HGs are not clear. It has been speculated that HGs may represent a stage of tumor cell apoptosis. In this review, we report a case of clear cell renal cell carcinoma with extensive intracellular HGs. The histological, cytochemical, immunohistochemical, and ultrastructural characteristics of HGs are reviewed and their clinical significance is discussed. In addition, other types of inclusions occurring in the tumors of the kidney are also briefly summarized
An Update on Actions of Mineralocorticoid Receptor in the Cardiovascular System: From Clinical Studies to Basic Research
Get PDFRecent clinical studies continued to show beneficial outcome of mineralocorticoid receptor (MR) antagonists for treating heart failure. However, the side effects of these drugs, particularly in the kidney, justified more studies to explore the cell type-specific effects of this receptor. Experimental data from cell type-specific knockout mouse models revealed that deletion of MR in myeloid cells, cardiomyocytes, or vascular smooth muscle cells in general protected mice from cardiovascular disease. Overexpression of MR in cardiomyocytes or endothelial cells, on the other hand, exacerbated cardiovascular disease. These results indicated direct function of MR in these cells. In all these cell types, oxidative stress and inflammation may be unified mechanisms of MR actions. Future clinical trials may broaden the indications of MR antagonists and basic research may provide more insightful understanding of MR actions in the cardiovascular system.[N A J Med Sci. 2013;6(2):91-94. DOI: 10.7156/najms.2013.0602091
Can Awareness of Medical Pathophysiology in Autism Lead to Primary Care Autism Prevention Strategies?
Get PDFEmerging research suggests that the timing of environmental factors in the presence of genetic predispositions has influenced the increase in autism spectrum disorders over the past several decades. A review of the medical literature suggests that autism may be impacted by environmental toxicants, breastfeeding duration, gut flora composition, nutritional status, acetaminophen use, vaccine practices and use of antibiotics and/or frequency of infections. The author reports her retrospective clinical research in a general pediatric practice (Advocates for Children), which shows a modest trend toward lower prevalence of autism than her previous pediatric practice or recent CDC data. Out of 294 general pediatrics patients followed since 2005 there were zero new cases of autism (p value 0.014). Given the prevalence of autism for that cohort of 1 in 50 children in the United States, it is important to consider implementing strategies in primary care practice that could potentially modify environmental factors or affect the timing of environmental triggers contributing to autism. [N A J Med Sci. 2013;6(3):134-144. DOI: 10.7156/najms.2013.0603134
Tetrahydrobiopterin May Be Transported into the Central Nervous System by the Folate Receptor α
Get PDFThe aim of this study was to determine if tetrahydrobiopterin (BH4), a cofactor that is essential for several critical neurometabolic pathways, is transported across the blood-brain barrier (BBB) using the same transport mechanism as folate. In this study we examined 30 children with ASD (mean age 7.5 years, standard deviation 3.2 years; 23% female). We studied autoantibodies that interfere with the binding of folate to the folate receptor α (FRα) – a receptor that is critically involved in the transport of folate across the BBB. The relationships between cerebrospinal fluid (CSF) BH4 concentrations with FRα autoantibody titers as well as with the interaction between CSF 5-methyltetrahydrofolate (5MTHF) concentration and FRα autoantibody titers were studied. CSF BH4 concentration was found to be lower in individuals with higher FRα blocking, but not binding, autoantibody serum titers, suggesting that interference with FRα dependent BBB transport interferes with BH4 transport across the BBB. This effect was not explained by lower CSF 5MTHF concentrations, thereby reducing the possibility that low CSF BH4 concentrations were secondary to low central folate. In addition, CSF BH4 concentration was inversely correlated with the interaction between CSF 5MTHF and FRα blocking autoantibody titers suggesting that BH4 competes with folate for FRα dependent transport across the BBB. These data suggest that FRα dependent transport mechanisms may be involved in the transportation of BH4 across the BBB. [N A J Med Sci. 2013;6(3):117-120. DOI: 10.7156/najms.2013.0603117
Distribution Patterns of Colorectal Cancer and its Precursors
No full textGrowing evidence shows that not only colorectal cancer (CRC) incidence but also its distribution patterns are changing. It is important to understand these changes to guide CRC screening, management, and public health strategies. We retrospectively analyzed the database of Miraca Life Sciences Research Institute for a one-year period (January 1 to December 31, 2009). We extracted all patients’ records with a diagnosis of CRC signed by all Miraca pathologists and compared with patients with a diagnosis of tubular adenoma (TA), sessile serrated adenoma/polyp (SSA/P), and traditional serrated adenoma (TSA) signed by a single GI pathologist for diagnosis consistency during the same period. Patients’ age, gender, and anatomic locations were analyzed and mapped in the study. A total of 1,757 patients with CRC, 1,341 with TA, 199 with SSA/P and 36 TSA were studied. The distribution patterns of CRC and TSA showed a significant left-sided colon predominance (62.9% of CRC, p < 0.001; 76.3% of TSA, p = 0.013), especially in the sigmoid and rectum regions. In contrast, SSA/P showed a significant right-sided distribution pattern (67.9%, p < 0.001) and TA showed a slightly predominant toward the right-sided colon (53.1%, p = 0.041). Although the distribution pattern of CRC was left-sided predominant, it shifted toward right-sided colon among patients with advancing age and being female gender. This study indicates that dynamic shift of CRC from left-sided colon toward right-sidedness was largely due to patients’ advancing age and being female gender. The right-sided predominant distribution patterns of SSA/P might play an important role in the increased risk of right-sided CRC. Therefore, it is important to take extra effort to examine right-sided colon endoscopically in high risk patients
Preferred Play Activities of Children with Autism Spectrum Disorder in Naturalistic Settings
Get PDFAutism Spectrum Disorders (ASDs) are lifelong, neurobehavioral disorders that impact behavioral, social and communication skills. Introducing and designing appropriate play opportunities for children with ASD is of primary concern for educators, clinicians, and parents. The researchers set out to research the types of play most often preferred by children with autism spectrum disorders. Data collected in a children’s museum over a six month period resulted in a sample size of 1,506 observations for children with ASD. Data for the six months were aggregated for each of 20 different exhibits. Each of the top five exhibits preferred by children with ASD provided strong and distinct sensory feedback and featured cause/effect results or repetitive motions. Conversely, the five least popular exhibits for children with ASD were pretend play activities, and play activities which focused on arts/crafts. At a 95% confidence interval, eleven of the twenty exhibits showed a statistically significant difference for children with ASD than would be expected by a normal distribution. Of those eleven, six were preferred less than the expected average and five were preferred more than the expected average. Preliminary results of this research study support the researchers’ hypotheses that children with ASD prefer play activities with a strong sensory component and are far less likely to engage in activities involving pretend play. [N A J Med Sci. 2013;6(3):128-133. DOI: 10.7156/najms.2013.0603128]
Autism and Diet: Is There a Connection?
Get PDFAutism spectrum disorder is a relatively common developmental brain disorder that typically presents in children under the age of two. Autism is characterized by a broad range of functional brain deficiencies centered in the area of emotional intelligence. There is little doubt that autism has been increasing in all countries, but the question arises why is this so and what can be done about it? Because little is known about the cause of autism, efforts to answer these important questions cause anguish for both medical communities and patients and their families who live with this challenging disorder every day. [N A J Med Sci. 2013;6(3):158-162. DOI: 10.7156/najms.2013.0603158
A Commentary on Autism and Moral Development: What Can We Learn from the Sandy Hook School Shooting?
Get PDFThe Sandy Hook Elementary School shooting has left many people wondering whether the shooter’s diagnosis along the autism spectrum is what caused the unthinkable crime that transpired. This commentary explores moral development in both the typical and autism populations. It reviews previous studies done on the autism population regarding their understanding of right and wrong actions even when they lack theory of mind. A lack of empathy is often attributed to individuals with autism spectrum disorders, but many studies show that while they may fail to articulate another person’s point of view, they are not completely unresponsive to the distress or moral dilemmas of others. This commentary aims to dispel any misunderstanding that autism spectrum disorder equals lack of empathy, which leads to violence. It also hopes to alert the medical community treating those with autism spectrum disorders that the diagnosis of the patient may not end at autism alone. Thus other personality disorders may be present requiring intensive treatment and referral. [N A J Med Sci. 2013;6(3):163-166. DOI: 10.7156/najms.2013.0603163