bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Many Faces of Cardiac Sarcoidosis
Authors: Serei Virian , Fyfe-Kirschner Billie 
Published: September 2019

Abstract

Introduction
Cardiac involvement by sarcoidosis, a disease characterized by nonnecrotizing granulomas of unknown etiology, is rare and affects up to 7% of sarcoidosis patients clinically, but with an autopsy prevalence of 15% to 90%. The clinical presentation, as well as macroscopic and microscopic findings in cardiac sarcoidosis patients, varies.

Methods
Retrospective clinical chart review and gross and microscopic examination of surgical specimen from two adult cardiac sarcoidosis patients with literature review were performed.

Results
Case 1: A 23-year-old woman with no past medical history presented with 2 months of heart failure symptoms following a viral gastrointestinal infection. Endomyocardial biopsy showed a dense chronic inflammatory infiltrate, without giant cells. Anterior mediastinal lymph node biopsy and left apical core showed effacement by nonnecrotizing granulomas with giant cells and multifocal calcification, supporting cardiac sarcoidosis. Twelve months later, she underwent cardiac transplantation. The explanted heart showed concentric ventricular scarring, which microscopically corresponded to myocardial fibrosis with scattered lymphocytic inflammation with no residual granulomas.
Case 2: A 52-year-old man with a medical history significant for ischemic cardiomyopathy and cutaneous sarcoidosis with lung involvement presented with congestive heart failure. Endomyocardial biopsy and left apical core showed myocyte hypertrophy and scattered interstitial inflammation. Mediastinal lymph node showed nonnecrotizing granulomas, consistent with sarcoidosis. Eighteen months later, he underwent cardiac transplantation. The explanted heart showed biventricular dilatation with interstitial fibrosis. There was also mild diffuse inflammation and occasional granulomas with rare focal necrosis.

Conclusion
Though the gold standard for diagnosing cardiac sarcoidosis is demonstration of myocardial nonnecrotizing granulomas, the sensitivity of endomyocardial biopsy is low. This is due to sampling error and stage of disease. Chronic end-stage disease can lead to fibrous encasement of granulomas and end-stage heart failure, necessitating cardiac transplantation. Current guidelines employ a combination of clinical, radiologic, and histologic criteria for diagnosing cardiac sarcoidosis.
Unusual Left Atrium and Pulmonary Vein Mass: Correlation of Imaging and Immunohistochemical Stains
Authors: Nowroozizadeh Behdokht , Ibe Ifegwu , Nowroozizadeh Sara , Bosemani Thangavijayan , Sun Jack , Wang Beverly 
Published: September 2019

Abstract

Introduction
Intimal sarcoma derived from the pulmonary vein and left atrium is an extremely rare and highly aggressive soft tissue neoplasm. We report a 21-year-old woman with a large filling defect in the pulmonary vein. The lesion extended into the left atrium mimicking a thrombus or a myxoma.

Case History
A 21-year-old woman presented with progressively worsening weakness, orthopnea, and exertional dyspnea of 2 weeks’ duration. Chest CT scan revealed a large left atrial mass, measuring 8.6 × 4.9 cm, which invaded the right lower lobe of lung and inferior pulmonary vein. Patient underwent right lower lobectomy and surgical resection of the left atrial mass and pulmonary vein.

Result
Histologic examination showed a highly cellular, cytologically malignant spindle cell neoplasm in a fascicular pattern. The lesional cells had hyperchromatic tapering nuclei and indistinct cytoplasm. There were numerous mitoses, focal necrosis, and predominantly myxoid stroma. The histologic features suggested the possibility of a myxofibrosarcoma or an intimal sarcoma. The lesional cells were immunoreactive for MDM2 and CDK4, consistent with amplification of the genes on the long arm of chromosome 12. Additionally, the lesional cells were vimentin, CD99, and BCL2 positive while nonimmunoreactive for S100, AE1/3, CD31, CD34, desmin, SMA, MyoD1, myogenin, MPO, and calretinin. Immunohistochemical staining for H3K27me3 was retained. These immunohistochemical findings supported the diagnosis of an intimal sarcoma.

Conclusion
Intimal sarcoma commonly involves the aorta and rarely presents as a pulmonary vein or atrial mass. Correlation of imaging and pathologic findings is critical, as intimal sarcoma can mimic a thrombus. Application of immunostains and cytogenetic studies are useful to distinguish intimal sarcoma from other cardiac sarcomas and myxoma of atrium.
Metastatic Malignant Paraganglioma: A Case Report
Authors: Sahni Suhalika , Mehta Vikas 
Published: September 2019

Abstract

Objectives
Paragangliomas are rare neuroendocrine tumors that arise from chromaffin cells with the ability to secrete catecholamines. The estimated combined prevalence is between 1:6,500 and 1:2,500. Paragangliomas can have a benign or a malignant course with incidence of malignant paraganglioma estimated to be 93/400 million. The majority of paragangliomas are sporadic but may be related to syndromes like multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and neurofibromatosis type 1.

Methods
We present a unique case of a 25-year-old female who presented with a hepatic mass. Further workup showed an enhancing hypervascular mass along with lesions within the bilateral lungs, liver, spleen, and sclerotic osseous metastatic disease at cervical thoracic lumbar and sacral spine. A liver biopsy was performed, which was consistent with the diagnosis of a metastatic paraganglioma.

Results
The liver biopsy showed a tumor consisting of epithelioid and polygonal tumor cells with eosinophilic cytoplasm present in a vascularized fibrous stroma in a sinusoidal pattern. In certain areas, the tumor cells show marked pleomorphism with large cells with macronuclei and bizarre shapes. On immunohistochemistry (IHC), the tumor cells were strongly and diffusely positive for CD56 and vimentin and focally positive for synaptophysin and chromogranin. S100 highlighted sustentacular cells. Ki-67 was very low, staining less than 2%. In addition, the tumor cells showed loss of expression of SDHB by IHC.

Conclusion
Metastatic malignant nonsecretory paraganglioma is an extremely rare disease. The most unique pathological aspect of paragangliomas is that tissue acquired from different organ appears the same histologically and yet different in terms of prognosis. Metastatic hepatic paraganglioma in our patient offers interesting insights to appreciate the rare metastatic nature of paraganglioma to the liver. The elusive nature of the metastatic disease in paraganglioma poses a challenge to clinicians since the diagnosis of metastatic hepatic paraganglioma is usually clinically undiagnosed.
Perplexing Gastrointestinal Pathology: A Case Report on Ectopic Gastric Tissue Presenting as Rectal Mass
Authors: Sahni Suhalika , Mehta Vikas 
Published: September 2019

Abstract

Objectives
Gastric heterotopia (GHT) is a commonly reported epithelial heterotopia and has been described as heteroplasia (congenital) during organogenesis or metaplasia (acquired) during process of damaged epithelial repair. GHT can occur along the GI tract from nose to anus; it is predominantly seen in esophagus, duodenum, and Meckel’s diverticulum. It is the most common subtype of epithelial heterotopia. However, the presence of gastric mucosa in the rectum is a very uncommon entity. The endoscopic prevalence of GHT can reach up to 11% in foregut and midgut with a lesser prevalence in hindgut.

Methods
We present an interesting case of heterotopic gastric mucosa presenting as a rectal mass. A 27-year-old female patient presented with lower abdominal pain and episodic bloody diarrhea. After initial workup, a colonoscopy was done, which showed a 3-cm mass in the rectum. Features of inflammatory bowel disease were not identified.

Results
At the first rectal biopsy, multiple fragments of gastric mucosa, with some minimally attached rectal crypts, were identified and a diagnosis of gastric heterotopia was made. Subsequently, the mass was resected and similar findings were identified, confirming the diagnosis. Our case showed mixed oxyntic and antral mucosa comingling with rectal-type mucosa. Immunohistochemical studies were performed, which showed CDX2 and CK20 being positive in rectal and CK7 in gastric mucosa.

Conclusion
Gastric heterotopia is a rare entity that can present as a solitary lesion. The clinical signs and symptoms can mimic those of inflammatory bowel disease. Floater or contamination is an important pitfall in this entity and can lead to an erroneously diagnose. Due to high frequency of having floater in some histology laboratories, we recommend ordering multiple deeper recuts on cases when the pathologist sees closeness or attachment of gastric tissue to the rectal tissue.
Osseous Metaplasia: An Unusual and Poorly Understood Finding in Gastric Polyps
Authors: Zayko Maria , Wheeler Yurong 
Published: September 2019

Abstract
Osseous metaplasia (OM) is an uncommon finding in gastrointestinal tract biopsies. Metaplastic bone formation has been predominantly reported in the lower GI tract in a background of benign and malignant neoplasms, as well as nonneoplastic inflammatory conditions. OM in the stomach is extremely rare, with less than 10 cases reported in the English literature to date. We present two cases of gastric OM encountered at one institution among more than 10,000 stomach polyps examined since 1994. The first is a 46-year-old female with spastic quadriplegia, peptic ulcer disease, and a jejunal feeding tube who had multiple gastric hyperplastic polyps removed over a 13-year period. The most recent polypectomy revealed a 3-cm gastric adenoma with benign bone formation in the stroma. The second case is of a 67-year-old asymptomatic male with a remote history of intestinal metaplasia and atrophy of the stomach who underwent upper endoscopy and removal of a 1-cm polyp. Sections consisted of hyperplastic foveolar epithelium with focal ossification. Both polyps showed acute and chronic inflammation and stromal fibrosis, and they lacked Helicobacter infection. Mechanisms driving gastrointestinal OM have been proposed since the first reported cases in the early 1900s. The most widely accepted theory is that osteogenic factors released by the neoplastic or inflammatory tissue induce undifferentiated stromal mesenchymal cells or fibroblasts to undergo transformation to osteoblasts. However, with gastrointestinal biopsies being one of the most common specimens, the picture remains incomplete as only very rare cases show bone formation, warranting further research. Although gastrointestinal OM appears to be clinically insignificant, long-term studies have yet to be performed. Nevertheless, it is crucial for pathologists to be aware of this phenomenon in order to avoid misdiagnosing OM as a primary mesenchymal neoplasm or bone invasion in cases of OM arising in the background of malignancy.
Histopathological Pattern of Prostate Cancer in a Tertiary Hospital in Ghana
Authors: Aboagye Evans , Asante Emmanuel , Owusu-Afriyie Felicia , Titiloye Nicholas , Duduyemi Babatunde 
Published: September 2019

Abstract

Objectives
Prostate adenocarcinoma (PCa) accounts for considerable morbidity in aging men in Africa. With fewer studies reporting on PCa within the subregion, this study was set out to analyze various clinicopathological patterns in PCa lesions, providing additional data for comparison with reported PCa cases in other parts of the globe.

Methods
The study included prostatic adenocarcinoma tissues received in the Department of Pathology, Komfo Anokye Teaching Hospital, over a period of 6 years (from 2008 to 2013). Relevant clinical data including age, histopathological diagnosis, and Gleason’s microscopic grading were extracted from the surgical day book of the department. In a consecutive order, archived tissue blocks and slides of the selected cases were also retrieved for review and confirmation of diagnosis.

Results
Mean age at presentation was 71.7 ± 8.72 years (age ranged from 53 to 96 years) with a peak in the seventh decade of life (n = 41, 52.6%). Majority of the cases were poorly differentiated (n = 43, 55.1%), followed by moderately differentiated (n = 20, 25.6%) and well-differentiated samples (n = 15, 19.2%). Highest occurrence of prostate adenocarcinoma was recorded in the year 2009 (n = 46), followed by 2008 and 2009 (n = 16 and 13, respectively), together accounting for 96.2% of the cases reported.

Conclusion
Histopathological evaluation of prostatic biopsies remains the mainstay of diagnosing prostate adenocarcinoma in Ghana. The pattern of incidence in the population is in line with several reported studies, with majority of the cases diagnosed at the late stage of the disease and thus portending worse prognosis.
Histiocytic Sarcoma in the Kidney: A Rare Presentation
Authors: Johal Priya 
Published: September 2019

Abstract

Background
Histiocytic sarcoma is a rare neoplasm of mature histiocytes with a wide range of clinical presentations and manifestations. The heterogeneity of this neoplasm can cause difficulty in both diagnosis and treatment. It is a diagnosis of exclusion, requiring verification of the histiocytic lineage and the exclusion of other B-cell related malignancies. Although cases of postrenal transplant histiocytic sarcoma have been reported, incidences in a native kidney are rare.

Case Report
We present the case of a 70-year-old male with a history of colorectal adenocarcinoma, status post chemotherapy, with an incidental exophytic renal mass found on imaging, which was determined to be a sarcomatoid renal cell cancer on biopsy. Radical nephrectomy was performed, revealing a 4.5 × 3.5 × 3.2-cm mass invading the renal pelvis and fat. Microscopic examination of the tumor showed large, poorly cohesive neoplastic cells with abundant pale, eosinophilic cytoplasm that stained positive for vimentin, CD4, CD163, and CD68 and weakly/focally positive for lysozyme. Negative immunostains included PAX-8, pan-CK, AE1/AE3, HMB45, S100, CD34, CD30, ALK1, CD1a, myeloperoxidase, CD138, myo-D1, SMA, and CD21. A diagnosis of histiocytic sarcoma, grade 4 was made based on these findings.

Discussion
Histiocytic sarcoma is a disease of unknown etiology accounting for less than 1% of all hematolymphoid neoplasms. Our literature review revealed no other cases arising de novo in the kidney, but several cases were reported in postrenal transplant patients.
Diagnosis of histiocytic sarcoma is difficult as it is a diagnosis of exclusion. The immunohistochemical profile most often expresses lysosome-related markers, such as CD68, CD163, or lysozyme. Additionally, negative staining is expected for markers related to B cells, T cells, and melanocytes. The cells are often large with eosinophilic cytoplasm, well-defined borders, and vesicular chromatin. Although there are numerous differentials, distinct morphologic and immunohistochemical features allow for accurate diagnosis.
Validation of a Thiocyanate Method on the Shimadzu UV-1800 Spectrophotometer
Authors: Zot Patricija , Gin Gregory , Bachmann Lorin 
Published: September 2019

Abstract

Objectives
Sodium nitroprusside is used in treatment of hypertensive emergency. In the body, nitroprusside is metabolized to cyanide and cyanide to thiocyanate. Thiocyanate concentration is used to monitor nitroprusside therapy and to evaluate possible exposure to cyanide. A method validation study was performed to assess analytical performance of a new spectrophotometry-based laboratory-developed test for serum thiocyanate.

Methods
Serum proteins were precipitated using trichloroacetic acid and ferric nitrate. The resulting red-colored solution was measured spectrophotometrically at 450 nm using the Shimadzu UV-1800 spectrophotometer (Shimadzu Scientific Instruments, Columbia, MD). Method precision, linearity, method comparison, and effect of interferences were evaluated according to Clinical Laboratory and Standards Institute guidelines. The Microsoft Excel Analyze-It application was used for statistical analysis.

Results
Between-run coefficients of variation (CVs) for quality control materials at thiocyanate concentrations of 5 mg/L, 20 mg/L, and 50 mg/L were 9.64%, 6.26%, and 4.25%, respectively. The assay was linear over the analytical measuring range of 5 mg/L to 200 mg/L, with an r2 value of 0.9982. The Deming regression equation for the UV-1601 (comparator) and UV-1800 (new) method was: UV-1800 = 0.998 (95% CI, 0.9762-1.001) UV-1601 + 0.79 (95% CI, –0.1379 to 1.733), and the standard error of the regression was 1.8 mg/L. Interference of <7% was observed in the presence of hemolysis, icterus, and lipemia.

Conclusion
The analytical performance of the new Shimadzu UV-1800 spectrophotometer method is adequate for measurement of serum thiocyanate in patients on nitroprusside therapy or with cyanide poisoning.
A Report of Submucosal Intravascular Paraganglioma of the Gallbladder and Review of the Literature
Authors: Bennett Brian , Webb David 
Published: September 2019

Abstract

Objectives
The purpose of this case study and review of the literature is to allow pathologists to recall an appropriate differential diagnosis for submucosal epithelial tumors of the gallbladder. It is also important to note the immunohistochemical evaluation of a paraganglioma.

Methods
Intraoperative findings were multiple adhesions of the omentum to the gallbladder itself, with a mildly dilated cystic duct measuring 6 to 7 mm externally. The intraoperative cholangiogram was normal with no evidence of retained stones. The gallbladder was 9.5 cm and essentially unremarkable with no stones upon gross examination. Palpation revealed a 5 × 4 × 4-mm gray-tan cystic duct lymph node that was microscopically unremarkable. Microscopically, there was a 2.7-mm submucosal intravascular epithelioid tumor. Cytologically, the cellular infiltrate was cohesive, filled, and expanded a single submucosal vessel. A broad panel of immunohistochemical stains was performed, with the results listed below.

Results
Carbonic anhydrase IX: Negative; Monoclonal CEA: Negative; CK7: Negative; CK 20: Negative; Gata-3: Positive; Mart-1: Negative; OCT3/4: Strong diffuse positive; P 63: Negative; Pancytokeratin: Negative; PAX-8: Negative; S-100: Negative; WT1: Negative; PLAP: Negative; Hep par 1: Negative; AFP: Negative; CD117: Negative; CD 30: Negative; CDX2: Negative; Glypican 3: Negative; SALL4: Negative; Inhibin: Negative ERG: Negative; CD34: Negative; CD56: Strong diffuse positive; Synaptophysin: Strong diffuse positive; Chromogranin: Strong diffuse positive.

Conclusion
Expert opinion was requested to confirm the presumed diagnosis on this case. The consulting pathologist was in agreement that this was best classified as an intravascular paraganglioma. The patient was seen in follow-up and a CT of the chest, abdomen, and pelvis was ordered. Interestingly, a 1.9-cm enhancing upper pole right renal mass was identified. The patient was sent to urologic surgical consultation in which they believed this tumor to represent a stage 1a primary renal cell carcinoma and subsequently sent for radiofrequency ablation.
Androgen Receptor Overexpression in Patients With Prostate Cancer in Kampala, Uganda
Authors: Yahaya James , Ajok Florence , Odida Michael , Wabinga Henry 
Published: September 2019

Abstract

Objectives
This study was aimed at determining the status of androgen receptor overexpression in patients with PCa and also correlating overexpression with Gleason score, patient age, and prostate-specific antigen (PSA).

Methods
This was a cross-sectional analytical laboratory-based study using retrospectively retrieved formalin-fixed, paraffin-embedded (FFPE) tissue blocks of 93 PCa patients who were diagnosed histologically between January and December 2018. Immunostaining was done using AR antibody. Cases with ≥10% positive cells having staining intensity of +1 and above were considered positive. Confirmation of the histological diagnosis and interpretation of the IHC-stained slides were done by two independent expert pathologists in a blinded manner. The association between AR overexpression and categorical variables was tested using the chi-square test, while continuous variables were tested using the t test with a 95% confidence interval (CI). A P < 0.05 was taken as statistically significant.

Results
AR overexpression was found in 54.8% (51/93) of the cases in this study. Majority of patients, 73.1% (68/93), had a Gleason score of 8 to 10, whereas a Gleason score of 6 to 7 was found in 26.9% (25/93). The mean Gleason score for the cases was 8.47 ± 1.52. Patients with a low Gleason score (≤7) had a higher proportion of AR overexpression than those with a high Gleason score (>8) and the difference was statistically significant (P = .013).

Conclusion
The clinical relevance of this study was that AR overexpression is associated with a Gleason score but not with age and prostate-specific antigen (PSA). AR overexpression was an independent predictor of PCa prognosis.
Application of the CLSI EP15-A3 Guideline as an Alternative Troubleshooting Tool for Verification of Assay Precision
Authors: Aguirre Jose Jara , Ness Karl , Algeciras-Schimnich Alicia 
Published: September 2019

Abstract

Introduction
The CLSI EP15-A3 guideline “User Verification of Precision and Estimation of Bias” provides a simple experimental approach to estimate a method’s imprecision and bias. The objective is to determine if the laboratory precision performance of repeatability (SR) and within-laboratory imprecision (SWL) are in accordance to the manufacturer specification claims (MSCs).

Objectives
Evaluate the utility of the EP15-A3 protocol to verify method precision during a troubleshooting investigation and after major instrument maintenance, using a carcinoembryonic antigen (CEA) immunoassay as an example.

Methods
CEA was performed on the Beckman Coulter DxI (Beckman Coulter, Brea, CA). Quality control (QC) levels (L1: 2.89; L2: 21.10; L3: 39.10 ng/mL) (Bio-Rad Laboratories, Irvine, CA) were used. Each QC level was measured before and after instrument maintenance as follows: five replicates per run, one run per day, and during 5 days. Imprecision estimates (IEs) for SR (%CVR) and SWL (%CVWL) were calculated by one-way analysis of variance using Microsoft Excel Analyse-it software. Estimated imprecision was compared to MSC and desirable imprecision specifications based on biological variation (BV).

Results
A change in the analytical performance of CEA was detected by a decreased sigma-metric indicator. After a bias problem was ruled out, the observed %CVR for L1, L2, and L3 were 7.2%, 3.7%, and 4.8%, respectively. The %CVWL were 8.3%, 5.0%, and 5.5%, which exceeded the MSC of %CVWL~4.0% to 4.5%. After a laboratory investigation, major instrument maintenance was performed by the manufacturer. The %CVR and %CVWL estimates for L1, L2, and L3 after maintenance were 3.2%, 3.8%, 3.5% and 3.9%, 4.2%, 4.0%, respectively. After maintenance, the CEA performance was consistent with the MSC for each of the levels analyzed and within the BV impression goal of %CV ≤6.4.

Conclusion
CLSI EP15-A3 guideline is an alternative troubleshooting tool that can be used to investigate and verify method precision performance before and after significant instrument maintenance.