bluuebunny/crossref_metadata_2025 huggingface dataset:
Arab Gulf States: Expanding Roles for the Military
Authors: Ardemagni Eleonora
Published: October 2020

Despite national differences, the military has usually presented a lack of political role and agency in the United Arab Emirates (UAE), Qatar, Kuwait, and Oman. This development has occurred because state formation in these nations has been mainly driven by energy revenues and external security provision. The primary task of the armed forces, especially between the 1950s and the 1970s, was rulers’ protection and regime security; for the monarchies, keeping the armies small and detached from political power was a coup-proofing strategy. As a result, researchers used to stress the dependency linkage between tribal armies and royal families, underlining the prominence of kinship loyalties (for upper echelons) and foreign manpower (for lower ones) in political–military relations. But as in a prism, these militaries reveal three coexistent faces—praetorian, neopatrimonial, and performative—with one prevailing on the others depending on the time frame. In fact, starting from the 1990s, the gradual processes of state consolidation and modernization have fostered the expansion of the military sector in the Arab Gulf states, maximizing the neopatrimonial dimension of the military. Defense procurement burgeoned, with an emphasis on hard power, as the agreements with the United States and Western allies to establish defense pacts, troop stationing, and military facilities. In the context of state transformation, the 2010s represent a turning point for the militaries that showed a rising performative dimension, especially in the UAE, and, to a lesser extent, Qatar—performative because of greater operative performances and also because of the ability to influence nation-building. Arab Gulf states’ national strategies acquired a military shape, reflecting in some cases military-driven foreign policies. Autonomy and self-reliance became national guiding stars and military reform was no longer a taboo for Emirati, Qatari, and Kuwaiti rulers. In fact, this is now functional in the improvement of military capabilities through know-how transfer, local expertise, and forms of social mobilization (as conscription, parades, exhibitions, and official rhetoric). In this sense, Oman played a vanguard role in the 1970s as the first-ever example of a performative army in the Gulf monarchies. In the performative armies of the 2010s, soldiers embody a renewed model of post-oil citizenship, based on sacrifice, duty, and national pride. As a matter of fact, the 2015 unprecedented military intervention in Yemen has turned into a watershed for Gulf militaries’ tasks and capabilities (especially for the UAE). Therefore, the military has gradually become a tool of nation-building and governments have been betting on militarized nationalism to forge a sense of shared belonging, identity, and patriotism. In times of rising Middle Eastern arms races and multidimensional threats, the military dimension has been redrawing civil–military relations, especially in the UAE and Qatar, thus offering a new research agenda for future studies on the Arab Gulf states’ militaries.
Macedonia: Troublesome Relations Among Politics, Ethnicity, and the Military
Authors: Vankovska Biljana
Published: October 2020

At first sight, relations between politics and the military in Macedonia, one of the ex-Yugoslav republics that gained independence in 1991, seem to resemble the typical evolution of civil–military relations in other countries in transition. Yet, history in Macedonia is far from straightforward and simple. First, the country’s appearance on the world scene was unique: it was practically a demilitarized state with no army! Apart from that, amid the Yugoslav imbroglio it was known as an “oasis of peace.” Only 10 years later, in 2001, Macedonia found itself on the verge of an ethnic conflict, with a powerless (Macedonian-dominated) military that confronted apparently well-organized Albanian paramilitary forces. In March 2020, Macedonia became NATO’s 30th member state. Yet, the dilemma that affects civil–military relations at both the political-military and societal-military levels has not gone away. Theoretically and practically, any meaningful analysis requires detection of the troublesome aspects of each side of the triangle: state/politics/military/society/ethnicity. Though the society–state dimension is far from inconsiderable, on methodological grounds the analysis that follows is restricted to the other two dimensions.

NATO membership for a transitional country usually presupposes a successful democratic transition, internal stability, and societal consensus over key national values and interests. Macedonia’s case belies that assumption. The Macedonian military has been practically invisible in internal politics, while it has been widely cited as a key asset for bringing the country closer to NATO by direct involvement in military interventions launched by the United States or NATO, starting with Afghanistan and Iraq and extending to the plans for involvement in Mali’s affairs. Behind the façade, there is silent internal strife within the ranks along political and ethnic lines (i.e., the same lines that sharply divide the state and society, challenging the country’s internal cohesion and democratic prospects). In addition, the military has to make do with scant essential resources, while the military officers’ self-respect is severely diminished by the low societal rewards for their profession. Macedonia’s democratic transition is far from complete, since the country is going through a deep internal crisis related to its societal/security dilemma, and the military is just one of the institutions that suffer because of ethnic competition and unprincipled power-sharing bargaining.
Stock-Flow Models of Market Frictions and Search
Authors: Smith Eric
Published: October 2020

Stock-flow matching is a simple and elegant framework of dynamic trade in differentiated goods. Flows of entering traders match and exchange with the stocks of previously unsuccessful traders on the other side of the market. A buyer or seller who enters a market for a single, indivisible good such as a job or a home does not experience impediments to trade. All traders are fully informed about the available trading options; however, each of the available options in the stock on the other side of the market may or may not be suitable. If fortunate, this entering trader immediately finds a viable option in the stock of available opportunities and trade occurs straightaway. If unfortunate, none of the available opportunities suit the entrant. This buyer or seller now joins the stocks of unfulfilled traders who must wait for a new, suitable partner to enter.

Three striking empirical regularities emerge from this microstructure. First, as the stock of buyers does not match with the stock of sellers, but with the flow of new sellers, the flow of new entrants becomes an important explanatory variable for aggregate trading rates. Second, the traders’ exit rates from the market are initially high, but if they fail to match quickly the exit rates become substantially slower. Third, these exit rates depend on different variables at different phases of an agent’s stay in the market. The probability that a new buyer will trade successfully depends only on the stock of sellers in the market. In contrast, the exit rate of an old buyer depends positively on the flow of new sellers, negatively on the stock of old buyers, and is independent of the stock of sellers.

These three empirical relationships not only differ from those found in the familiar search literature but also conform to empirical evidence observed from unemployment outflows. Moreover, adopting the stock-flow approach enriches our understanding of output dynamics, employment flows, and aggregate economic performance. These trading mechanics generate endogenous price dispersion and price dynamics—prices depend on whether the buyer or the seller is the recent entrant, and on how many viable traders were waiting for the entrant, which varies over time. The stock-flow structure has provided insights about housing, temporary employment, and taxicab markets.
Ibero-Romance II: Astur-Leonese, Spanish, Navarro-Aragonese
Authors: Hualde José Ignacio
Published: October 2020

The Romance varieties spoken in the Iberian Peninsula fall into three major groups: Galician-Portuguese, Central Ibero-Romance and Catalan. All these varieties have their origins in the evolution of Latin in the northern fringe of the Iberian Peninsula and spread southward in the Middle Ages. One of the main features that distinguish the Central Ibero-Romance group from its neighbors to the west and east is the diphthongization of /ɛ/ and /ɔ/ from Latin short ĕ and ŏ in stressed syllables (as in tierra ‘land’, puerta ‘door’ vs. Pt. and Cat. terra, porta). Besides Spanish, which historically derives from the evolution of Latin in the original territory of the Kingdom of Castile, the other main Central Ibero-Romance varieties are Astur-Leonese (including Mirandese, in Portugal) and Aragonese. For the medieval period, Old Navarrese Romance is well documented, as is, to a lesser extent, the transitional variety of La Rioja. There are no documents entirely written in Andalusi Romance, the set of Romance varieties spoken in Islamic Spain in medieval times, but we can infer some of its features from short texts and several other sources.
Emerging Issues and Challenges in Transboundary Freshwater: The Role of Treaties and Treaty Design
Authors: Dinar Shlomi
Published: October 2020

Freshwater’s transboundary nature (in the form of rivers, lakes, and underground aquifers) means that it ties countries (or riparians) in a web of interdependence. Combined with water scarcity and increased water variability, and the sheer necessity of water for survival and national development, these interdependencies may often lead to conflict. While such conflict is rarely violent in nature, political conflict over water is quite common as states diverge over how to share water or whether to develop a joint river for hydropower, say, or to use the water for agriculture.

For the same reasons that water may be a source of conflict, it is also a source of cooperation. In fact, if the number of documented international agreements over shared water resources is any indication, then water’s cooperative history is a rich one. As the most important and accepted tools for formalizing inter-state cooperation, treaties have become the focus of research and analysis. While treaties do not necessarily guarantee cooperation, they do provide states with a platform for dealing with conflict as well as the means to create benefits for sustained cooperation. This also suggests that the way treaties are designed—in other words, what mechanisms and instruments are included in the agreement—is likewise relevant to analyzing conflict and cooperation.
Reliability of the Dilute Russel Viper Venom Time (dRVVT) in Determining Lupus Anticoagulant (LAC) in Anticoagulated Patients
Authors: Khan Waleed , Rinder Henry , Tormey Christopher , Siddon Alexa
Published: October 2020

Objectives

Antiphospholipid syndrome (APS) can be complicated by thrombosis in the presence of laboratory evidence of persistent antiphospholipid antibodies, usually in the form of a LAC. The diagnostic criteria for APS/LAC require persistent prolongation of two phospholipid-dependent clotting assays, most often the dRVVT and a thromboplastin-based phospholipid-neutralization (PN) assay. When there is discrepancy between tests, LAC diagnosis is inconclusive. This testing is complicated by anticoagulation, which may yield false positive results. We investigated the impact of various anticoagulants on LAC diagnosis by evaluating the rates of dRVVT and PN discrepancy between anticoagulated and non-anticoagulated patients.

Methods

dRVVT and PN (LAC panel) values were examined for 1452 consecutive patients samples since April 2017, along with anticoagulant status at the time of testing. Each LAC panel was classified as positive (both dRVVT and PN abnormal), negative (both dRVVT and PN normal), or discrepant (one normal and one abnormal). The proportion of discrepant results between anticoagulated and non-anticoagulated patients was calculated and also stratified according to the class of anticoagulant.

Results

Discrepant results occurred in 161 of 493 anticoagulated patient samples (33%) versus 135 of 1012 non-anticoagulated patient samples (13%). The odds ratio (OR) for a discrepant result for anticoagulated patient samples was 3.15 (95% CI=2.43–4.09) compared to non-anticoagulated patient samples. When narrowed to individual anticoagulant classes, Lovenox had the lowest chance of yielding discrepant results (OR = 1.16 95% CI= 0.51–2.66), whereas Rivaroxaban (OR = 6.9 95% CI= 4.49–10.55) and Warfarin (OR = 5.4 95% CI= 3.43–8.55) had the highest odds of yielding discrepant results.

Conclusions

Patient anticoagulant status is an important determinant in laboratory testing for APS/LAC. The dRVVT and PN test are 3-fold more likely to yield inconclusive results when a patient is anticoagulated; the type of anticoagulant is even more critical given the large difference in rates of discrepant results for patients on Rivaroxaban and Warfarin versus patients on Lovenox. The difference in discrepancy rate is likely due to the differential impact of each class of anticoagulant on the various components of the coagulation cascade. Rivaroxaban is a direct factor X inhibitor, and Warfarin directly lowers active factor X, amongst other, levels; by contrast, Lovenox, which was not significantly discrepant, requires antithrombin to amplify inhibition of factor II and X activity.
Validation of Soluble Fms-Like Tyrosine Kinase-1 (sFlt-1) and Placental Growth Factor (PlGF) Assays on Cobas e602 System
Authors: Tang Nga Yeung , Rana Sarosh , Yeo Kiang-Teck J
Published: October 2020

Background

Preeclampsia is a leading hypertensive disorder in pregnant women. The angiogenic biomarkers, soluble fms-like tyrosine kinase-1 (sFlt-1) and placental growth factor (PlGF) ratio, have been shown to be associated with diagnosis and prediction of preeclampsia. The objective of this study is to validate the analytical performance of sFlt-1 and PlGF on the Cobas e602 system (Roche Diagnostics Corporation).

Method

Intra-day and inter-day precisions for both sFlt-1 and PlGF assays were assessed using quality control materials provided from Roche Diagnostics. The accuracies for both assays were assessed by running 60 patient samples, which have been previously analyzed on the Elecsys 411 analyzer (Roche Diagnostics Corporation) at the Beth Israel Deaconess Medical Center. Linearity studies for both assays were performed using patient plasma spiked with recombinant sFlt-1 and PlGF proteins (R&D systems). Hemolysis, icterus, lipemia and biotin interference studies were performed by spiking hemolysate, bilirubin, intralipid or biotin into either pooled patient plasma with detectable levels of sFlt-1 and PlGF or otherwise, patient plasma spiked with recombinant sFlt-1 and PlGF proteins.

Results

Total precisions for both assays demonstrated CVs of <5.0%. The sFlt-1 and PlGF assays demonstrated analytical measuring ranges of 3060,000 pg/mL and 79,000 pg/mL, respectively (r2 > 0.98). Lower limit of quantitation (10% CV) was 30 pg/mL for sFlt-1 and 7 pg/mL for PlGF, respectively. Interference studies showed sFlt-1 and PlGF were not significantly affected by hemolysis up to H-indices of 500 and 1000 respectively; both assays were not affected by bilirubin up to an I-index of 60, and lipemia up to an L-index of 2800. Biotin at concentrations >30 ng/mL caused significant negative bias for both sFlt-1 and PlGF assays. Comparison studies showed the following: Cobas e602 sFLT-1 = 1.09 [Elecsys 411 sFLT-1] +203 (r2=0.97, Sy/x=1234, n=58); Cobas e602 PlGF = 1.10 [Elecsys 411 PlGF] +47 (r2=0.99, Sy/x=22.1, n=58); Cobas e602 sFLT-1/PlGF ratio = 0.94 [Elecsys 411 sFLT-1/PlGF ratio] +3.5 (r2=0.91, Sy/x=50, n=58).

Conclusion

sFlt-1 and PlGF measured on Roche Diagnostics Cobas e602 system demonstrated excellent analytical performance and are acceptable for clinical use once approved in the US.
Co-Culture of Acinetobacter calcoaceticus-baumannii complex and Staphylococcus saprophyticus Supports Simple Point Contamination Model in Recent Cases of Transfusion-Related Sepsis
Authors: Kerantzas Christopher , Merwede Jacob , Snyder Edward , Hendrickson Jeanne , Tormey Christopher , Kazmierczak Barbara , Peaper David
Published: October 2020

The CDC recently reported a series of four septic transfusion reactions across three states resulting from contamination of apheresis platelet products. The apheresis platelets were contaminated with strains of both Acinetobacter calcoaceticus-baumannii complex (ACBC) and Staphylococcus saprophyticus (Ss). Two of the reported septic transfusion reactions occurred at our institution. The CDC investigation showed that isolates of each species were genetically related and suggested a point source of contamination. However, the contamination of blood products with ACBC is rare and the co-occurrence of these two species in all four cases was unusual. We hypothesized that there was an augmentative interaction between the clinical isolates of ACBC and Ss from these cases that contributed to their repeated co-occurrence. To test this hypothesis, we compared the growth characteristics of ACBC and Ss when cultured together versus independently. We used isolates from the contaminated platelets for our studies and performed experiments using both solid and liquid growth media. Experiments on solid media assessed density of growth and macroscopic morphology after cross-streaking on Columbia Blood Agar (CBA) and Luria-Bertani (LB) agar. Experiments in liquid media assessed growth by CFU counts in LB broth, platelet-poor plasma, and apheresis platelets. Growth in apheresis platelets was performed using standard, room temperature blood bank platelet storage conditions. Results of these experiments showed a higher CFU concentration of Ss when co-cultured with ACBC in LB broth only after several days, as compared to Ss alone under the same conditions. Otherwise, there was no evidence of augmented growth by either CFU concentration or growth rate in LB broth, plasma, or platelets at other time points. Similarly, there was no evidence of augmented growth by colony density or morphology when cross-streaking strains on either CBA or LB agar. As a result, we conclude that the co-occurrence of these two species in platelets is likely a coincidence of the point contamination suggested by the CDC investigation and not the result of growth augmentation between the two species.
Utilization and Laboratory Monitoring of Andexanet Alfa Reversal of Factor Xa Inhibitors in Bleeding Patients
Authors: Alipour Zahra , Jackups Ronald
Published: October 2020

Introduction

Andexanet alfa, a reversal agent for factor Xa inhibitors rivaroxaban and apixaban, was approved by the Food and Drug Administration in May 2018 for patients with life-threatening bleeding. It acts by competing with endogenous factor Xa to block anticoagulant activity. Intravenous administration rapidly reduces anti-factor Xa activity. Because andexanet became available only recently, there is high potential for inappropriate use. Our objective was to investigate utilization and laboratory monitoring of andexanet in our hospital system in order to improve utilization.

Methods

This is a retrospective study with review of charts in the electronic medical record from April 2019 to February 2020. Charts were reviewed for the time of andexanet administration, anti-factor Xa testing before and after administration, turnaround time (TAT) for anti-factor Xa testing, and patient’s status after treatment.

Results

We identified 33 patients who received andexanet in our hospital system. Intracranial hemorrhage was the most common clinical symptom. Of 33 patients, 12 patients (36%) expired, one transferred to hospice, 18 (55%) were discharged alive, and two recent cases are alive awaiting disposition. Only 12 patients (36%) had anti-factor Xa testing performed prior to administration of andexanet, with one additional patient receiving testing only after administration. Factor Xa activity was misordered for anti-factor Xa in one patient. TAT for anti-factor Xa in these patients ranged from 25–112 minutes, with median 35.5 minutes. Of the 12 patients who had anti-factor Xa testing prior to administration, 4 expired and 8 survived. Pre-administration anti-factor Xa activity was significantly lower in patients who expired than in patients who survived (0.3 vs. 1.8 IU/ml, P value = 0.02).

Conclusion

Currently, anti-factor Xa testing is not ordered for most patients receiving andexanet alfa in our hospital system. Although not necessary for dosing decisions, anti-factor Xa testing may be useful prior to administration to identify patients unlikely to benefit from anticoagulant reversal. Despite a small sample size, there was a statistically significant difference in anti-factor Xa activity by patient outcome, suggesting that andexanet may be unhelpful in patients with low anti-factor Xa activity. Based on these findings, we are building an electronic order set to facilitate proper administration of this medication.
What’s in a Name? Comparative Analysis of Laboratory Test Naming Guidelines as Applied to Common Confusing Test Names
Authors: Garnett Emily , Wang Julia , Jackson Brian , Procop Gary , Bierl Charlene , Singh Ila
Published: October 2020

Laboratory test names frequently do not enable easy understandability or promote correct test utilization, which leads to difficulty for providers in finding the correct test and results in unnecessary cost and medical errors. As a further complication, laboratory test names are largely unstandardized and are not named based on a consistent set of conventions. To address these issues, the TRUU-Lab (Test Renaming for Understanding & Utilization) initiative aims to generate a consensus laboratory test naming guideline for better human understandability of laboratory test names. These studies address the first and second aims of the TRUU-Lab initiative: 1) to identify root causes and challenges in understanding and using laboratory test names, and 2) to share resources related to potential solutions. We initially conducted survey studies to capture the most commonly problematic laboratory test names, then performed analysis of these names to identify aspects of these names that led to confusion among providers. 274 survey responses yielded ~100 unique laboratory tests that respondents felt were confusing, and highlighted substantial diversity both in the names of these tests between institutions and in respondent opinion on the best alternative names, with the top 10 most commonly-cited tests having at least 3 unique names, and the top 2 tests (Vitamin D and anti-factor Xa) having at least 10 unique names. Post-survey analysis identified eight common characteristics associated with poor understandability of a test name, including ambiguity, abbreviations, homophones, multiple indications for a single test, non-descriptive proprietary names, synonyms, truncation due to software limitations, and €œpanels where test components are obfuscated. A subset of the survey-identified confusing test names were used to evaluate existing laboratory test naming guidelines for their ability to produce understandable test names. Five guidelines, including LOINC, ONC TigerTeam, Pan-Canadian iEHR Viewer Name, Standards for Pathology Informatics (Australia), and ARUP Laboratories internal style guides, were evaluated, and produced highly variable names given the same test name prompt. Further, existing guidelines also varied in their ability to avoid pitfalls previously identified as associated with poor understandability. Together, these studies highlight the aspects of existing laboratory test names that lead to confusion among ordering providers, and identify the inability of existing laboratory test naming practices to adequately address these issues. Efforts are ongoing within TRUU-Lab to use these results to inform novel laboratory test naming guidelines that promote universal human understandability. Work is also ongoing to apply these novel guidelines to generate new candidate test names, and conduct survey analysis to evaluate the effects of new test naming guidelines on understandability and correct test utilization.
Utilization of P16 in Head and Neck Cytology and Surgical Specimens After the Release of CAP Guidelines
Authors: Laharwani H , Manucha V , Jefferson G , Jackson L
Published: October 2020

Introduction/Objective

HPV-positive oropharyngeal squamous cell carcinoma is biologically and clinically unique and has a survival advantage over other head and neck squamous cell carcinomas. In December 2017 College of American Pathologist published guidelines for testing HPV status in head and neck cancer. It was recommended that pathologists perform HR-HPV testing on head and neck squamous cell carcinomas from all patients with known oropharyngeal SCC not previously tested for HR-HPV, with suspected oropharyngeal SCC, or with metastatic SCC of unknown primary. The aim of this study was to determine the compliance of pathologists following the CAP guidelines.

Methods

Cases that underwent HPV testing using p16 immunohistochemistry for the years 2017 and 2019 were retrieved. Based on the guidelines, p16 testing was designated as “indicated” or “not indicated”.

Results

There were 196 cases in which p16 testing was performed in a period of 3 consecutive years. Of these, 175 were FNA/ biopsies and 21 were surgical resections. In 69 cases (56 FNAs and 13 biopsies) the biopsy was performed on neck masses with unknown primary. The compliance for p16 testing in OPC and Lymph nodes with metastatic SCC of unknown primary was 100%. In 34 (17.3%) cases p16 testing was not indicated, the most common reason being wrong site (85%) including the larynx, oral tongue, the floor of the mouth, buccal mucosa, and nasal mass. Of the unindicated p16s, 20 (58%) were received in consultation for continuity of care.

Conclusion

Not being clear about the site of the tumor is the most common reason for unindicated p16 testing. A clear designation of biopsy site and proper communication between pathologist and surgeon can improve utilization of p16 testing in head and neck carcinomas.
Pathological Study of Stillbirths Placentas at Muhimbili National Hospital, Tanzania
Authors: Malango A E
Published: October 2020

Introduction/Objective

Stillbirth is defined as fetal death that occurs at gestational age of ≥28 weeks. In our setting clinical assessment is the only method used to determine cause of stillbirths, with no reported proportion of unknown clinical diagnosis. Studies showed that unknown cause of stillbirths can be reduced by examination of placenta.

Causal identification aids in the mourning process and identifying recurrence risks. The study aimed to describe pathological changes in the placentas of stillbirths which have risk to cause fetal death

Methods

A descriptive cross-sectional study done for the period of 6 months, it involved examination of 80 placentas of stillbirths born at gestational age of ≥ 28 weeks, placentas were fixed in 10% neutral buffered formalin for 8–12 hours. Grossing and interpretation of placenta pathology was according to Amsterdam Placental Workshop Group Consensus Statement.

Results

Out of 80 stillbirths, 32(40%) had unknown clinical diagnosis. Majority of stillbirth placentas 71(91%) found with either one or combined pathologies with the risk to cause stillbirth. Maternal vascular malperfusion was the commonest pathology and was significantly associated with preterm stillbirths. Maternal floor infarction, a placenta pathology with risk to cause fetal death and high risk of recurrence was among the pathologies found, was seen in 4(5%) of stillbirth placentas.

Conclusion

Findings in this study clearly indicated the importance of pathological examination of placenta in determining cause of stillbirth. Placenta examination in stillbirths can identify more pathology related to stillbirths than clinical assessment alone.
Omphaloceles Associated with Chromosomal Segmental Alterations Carry a Poor Prognosis
Authors: Zhou H
Published: October 2020

Introduction/Objective

Omphaloceles are frequently associated with chromosomal abnormalities, including aneuploidy and segmental alterations. High resolution chromosomal microarray analysis (CMA) can detect segmental alterations < 5 Mb, which is not detectable by G-banding. However, the prognostic significance of the segmental alteration in infant with omphalocele is not elucidated.

Methods

To identify omphalocele cases with genetic studies, a CoPath database search (1/2000 - 7/2017) was performed with key words “omphalocele” and “genetic”. From 1/2000 to 12/2008, only G-banding was performed. From 1/2009 to 7/2017, omphalocele cases were screened with karyotyping. Cases with normal karyotype were reflexed to CMA. Copy number gains/losses and corresponding genes were analyzed by the Affymetrix Chromosome Analysis Suite.

Results

Follow-up data are available in 75% (67/89) cases. There is no significant difference of the overall survival rate of male and female patients (80.5% vs 76.9%; χ 2, p = 0.7645). There are 16.9% (15/89) omphaloceles with aneuploidy, 10.1% (9/89) cases with segmental alterations by CMA, and 73.0% (65/89) cases with normal CMA and/or normal karyotype. Although patients with segmental alterations have a significantly higher survival rate than those with aneuploidy (44.4% vs 0%, χ2, p = 0.0119), their overall survival rate is significantly lower than infant with normal CMA and/or normal karyotype (44.4% vs 82.8%; χ2, p < 0.0001). Infants with segmental alterations carry a significantly worse prognosis than infants with normal genetic study.

Conclusion

To date, this is the first study of the prognostic significance of segmental alteration in infants with omphalocele. Our data demonstrated that omphaloceles with segmental alterations carry a significantly worse prognosis than those with normal CMA and/or karyotype. It is crucial to convey the prognosis to the parents with a fetus carrying segmental alterations; so the family could make an informed decision and get ready for an infant with special needs.
Carcinosarcomas of Ovary/Fallopian Tube: A Rare Entity
Authors: Vo D M , Verma K , Khullar P , Vintzileos W , Jimenez E
Published: October 2020

Introduction/Objective

Adnexal carcinosarcomas (MMMTs) are rare tumors (1–4% ovarian carcinomas) with worse prognosis than high grade serous carcinomas (HGS) at similar stage. They typically present at age 64–66, often with peritoneal involvement. They are biphasic tumors with stem cells undergoing divergent epithelial and sarcomatous differentiation. The epithelial component is usually HGS and drives progression of the tumor. The mesenchymal component can be homologous with high-grade spindled cells or heterologous with malignant cartilage, bone, muscle or fat. Metastases are mostly epithelial; metastatic sarcomatous components are unusual. We reviewed our single- institutional experience of adnexal MMMTs.

Methods

We reviewed our pathology database (2001–2019) to find all cases of adnexal MMMTs. We reviewed their histological features, histology of metastases and clinical outcomes.

Results

Our series consisted of 12 cases. Patients aged 41–82 years. The primary tumor sites were ovary (6 cases, 50%), fallopian tube (4, 33%), 1 each (8%) in paratubal region and infundibular ligament. Fallopian tube was involved in 8/12 cases (4 cases as primary MMMT, 4 cases with STIC or HGS). Epithelial component was serous (75%), endometrioid (17%) and mucinous (8%). Sarcomatous component was homologous in 5 cases (41%), heterologous in 7 cases: cartilage (33%), cartilage/muscle (8%), muscle (8%), cartilage/muscle/fat (8%). 3 cases were FIGO stage I, 9 had peritoneal metastases (8 stage III, 1 stage IV). Six cases had metastatic HGS; 3 had metastatic HGS with sarcomatous component, 2 with heterologous elements. Aberrant p53 pattern was seen in 7/12 and TP53 mutation was noted in 6/12. Ten patients received cytoreductive surgery and chemotherapy. 7 patients are alive with progression free survival ranging 6–59 months, 2 survived for 25 and 29 months, 3 are lost to follow-up.

Conclusion

Metastatic sarcomatous heterologous elements are rare in uterine carcinosarcomas and may suggest adnexal origin. They may correlate with worse outcome; in our series, 1/2 died after 29 months, the other was lost to follow-up. Fallopian tube involvement (75% of our cases) is of significance as identical TP53 mutation has been identified in a case report of ovarian carcinosarcoma with fallopian tube STIC. Also, our 2 cases of infundibular ligament and paratubal region may indicate seeding from fallopian tube. Further studies are needed to confirm the correlation.
Retinal Ependymoma: A Rare Extra-Axial Presentation
Authors: Abou Shaar R , Arora K , Keller C
Published: October 2020

Introduction/Objective

Ependymomas are well-demarcated and slow-growing neuroepithelial neoplasms that comprise 3–9% of primary CNS tumors. The vast majority of ependymomas arise either intracranially, mostly in children, or in the spinal cord and are associated with ependymal lining. Histologic hallmarks are perivascular pseudorosettes, ependymal rosettes and alternating zones of nuclear crowding and nuclear free zones composed of coarse cell processes. In high grade ependymomas increased mitoses, necrosis and nuclear pleomorphism may be seen.

Methods

We present the case of a 63-year-old woman in with a past medical history of retinopathy of prematurity, glaucoma and right eye enucleation. She presented with a painful blind left eye refractory to medical treatment and subsequently underwent left eye enucleation.

Results

On histologic examination, an incidental retinal ependymoma was identified. The neoplastic cells were fusiform and had long coarsely fibrillar cell processes. Characteristic periodicity of nuclear crowding and scarcity was observed. In places, neoplastic cell processes extended radially to delicate and sometimes hyalinized blood vessels, forming so-called perivascular pseudorosettes. Definite ependymal rosettes were not recognized in the examined sections. Stigmata of chronicity were found, such as ischemic type necrosis, blood vessels with dystrophic calcifications and foci of ossification replete with fibroadipose tissue in the marrow spaces.

The neoplastic cells labeled with antibodies against glial fibrillary acidic protein (GFAP), thus confirming glial lineage in the neoplastic cell. Additionally, there was scattered intracytoplasmic expression of epithelial membrane antigen (EMA) in a dot-like or circular pattern. The latter is well described in ependymomas and often used to support the diagnosis.

Conclusion

Ependymomas rarely occur at extracranial sites, such as the chest, abdomen and pelvis. We are presenting the fourth case of retinal ependymoma reported in literature. The tumor had classical immunomorphologic findings.
Ciliated Carcinoma Associated With Human Papiloma Virus: Presentation As Cervical Metastasis Of Unknown Primary
Authors: Ibarra A , Las Heras F , Antonio L , Montero P , Besa P , Henriquez J , Labra Y
Published: October 2020

Introduction/Objective

There is a significant increase in high-risk human papilloma virus (HPV) -related carcinomas of the head and neck. Half of them debut with metastases in cervical nodes, of expansive growth, usually cystic-necrotic. Most are oropharyngeal primaries. They are non-keratinizing squamous carcinomas with mild atypia, sometimes with exceptional characteristics, such as glandular differentiation and inclusion of ciliated cells

Methods

Clinical Case: 39-year-old male patient, operated two years ago, in another institution, for cervical metastasis from HPV-related Ciliated Carcinoma (HPV-RCC), 4.5 x 2 cm. No primary cancer was found in his study. Two months ago, a new 4.3 x 2.7 cm metastasis was resected, performing a right cervical dissection, with another 36 lymph nodes without tumor. Palatine tonsils, adenoids are removed and a biopsy of the oral mucosa is taken, and no malignancy was found. In our center, biopsies of the tongue and right glosso-tonsillar fold are taken, without objectify malignancy.

Results

The metastases had macroscopic cystic-necrotic appearance. Histology corresponded to nonkeratinizing squamous carcinoma with microcystic spaces and the presence of ciliated columnar cells. The immunohistochemical study gave strong and diffuse positivity for p16. The real-time multiple PCR study detected HPV genotype 16.

Conclusion

We present an exceptional case of Ciliated Carcinoma Related to HPV, a recently described entity. These are nonkeratinizing squamous neoplasms, which include cystic spaces and ciliated columnar cells. Both components reveal HPV positivity, with p16 expression and high-risk viral typing. HPV-RCC should be considered in the differential diagnosis of well-differentiated squamous lesions of the oropharyngeal cavity and at the cervical level, with non-neoplastic cysts. Our patient will undergo radiotherapy protocol, for unknown primary
Malignant Proliferating Pilar Tumor: A Rare Mimic of Squamous Cell Carcinoma
Authors: Beversdorf W , Rinker E
Published: October 2020

Introduction/Objective

Proliferating pilar tumor is a rare cutaneous adnexal neoplasm, closely resembling and potentially misdiagnosed as conventional squamous cell carcinoma. Malignant forms are recognized; however, differentiating criteria have not been firmly established due to their exceptional rarity. Prognosis is variable, with recurrence or metastasis reported in up to 25% of patients within a very small data set.

Methods

We present a case of a 31 year old male who underwent excision of a posterior neck mass clinically presumed to be a recurrent benign cyst in 2018. Initial excision was performed at an outside institution approximately 10 years prior, and a pathology report was not included in his record.

Results

Our resection specimen contained a 5.5 cm lobulated, cystic and solid mass involving the dermis and subcutis with superficial ulceration. Tumor histology was consistent with malignant proliferating pilar tumor as described in other reported cases, showing a proliferation of pleomorphic squamous cells infiltrating the dermis and associated desmoplasia. The tumor featured abundant pilar keratinization with focal necrosis and increased mitotic activity. Numerous smaller pilar cysts exhibiting varying degrees of squamous atypia studded the periphery of the dominant mass. The immunohistochemical profile of this case also exhibited similarities to other reports, with focal positivity for calretinin and CD34 as well as an increased Ki-67 proliferative index (up to 70–80%). While other reports document diffuse expression of p53, our case demonstrated complete absence of p53 staining.

Conclusion

We present this case to highlight the importance of recognizing pilar differentiation within squamous lesions, examine pathologic features associated with malignant behavior, and discuss the relationship between histologic characteristics and clinical outcomes in this exceedingly rare tumor group.
SMARCA4 Deficient Thoracic Sarcoma Presenting with a Pathologic Fracture of Proximal Tibia and L5 Vertebral Body: a Case Report and Review of Literature
Authors: Elzamly S , Murzabdillaeva A , Taha H , Shitawi M , Zhu H
Published: October 2020

Introduction/Objective

SMARCA4 (BRG1) is a central component of the Switch/Sucrose-Non-Fermentable (SWI/SNF) chromatin remodeling complex which plays a critical role in the initiation, progression and dedifferentiation of a variety of cancers arising in different anatomical sites. The recently discovered SMARCA4-deficient thoracic sarcoma (DTS) can present as a mediastinal, pleural or pulmonary mass and constitutes a unique and highly lethal entity.

Methods

Here we present an interesting case of a 66-year-old, smoker male with past medical history of COPD, polysubstance abuse, hepatitis C virus, and alcoholic cirrhosis who presented with left proximal tibia pathological fracture, L5 vertebral body lytic lesion, left leg cellulitis and hypercalcemia. Imaging studies, tibia biopsy, thoracocentesis, histopathological examination, and immunohistochemical stains were performed.

Results

Imaging studies revealed a 3.1 cm, right upper lobe speculated lung mass along with multiple other lung nodules. Numerous arterially enhancing hepatic masses up to 2.8 cm were also identified. The patient underwent intramedullary nail, cementation and tibia biopsy followed by thoracentesis a month later.The histological sections revealed sheets of poorly differentiated malignant epithelioid cells showing high nuclear pleomorphism, prominent nucleoli, eosinophilc cytoplasm with extensive necrosis, and high mitosis (Ki-67 around 100%). The tumor cells lacked the expression of SMARCA4 (BRG1), cytokeratin, SALL4, CD34, TTF-1, P40, S100, HMB45, pan melanoma, desmin, CD31, ERG, CD30, CD56, chromogranin, CD38, CD45, CD3, ALK-1, myeloperoxidase, glypican-3, hepatocyte, and inhibin with mixed kappa and lambda. Tumor cells expressed SATB2 and focal synaptophysin, consistent with SMARCA4 deficient thoracic sarcoma metastasizing to the bone. The patient’s condition rapidly deteriorated and he died within two months.

Conclusion

An accurate diagnosis of these tumors is paramount and can be challenging. However, recognizing SMARCA4-DTS from other types of epithelioid tumors that involve the thoracic region is clinically relevant, as targeted therapies for SMARC-deficient tumors are currently being investigated and new clinical trial data show therapeutic benefit.
A Diagnostically Challinging Case of Primary Thyroid Kappa-restricted Plasma Cell- Rich Hematolymphoid Neoplasm with Focal Plasmablastic Differentiation
Authors: Haleema S O , Akbar S , Rowley E , Polski J M , Mneimneh W
Published: October 2020

Introduction/Objective

Most low-grade B-cell lymphomas of the thyroid are extranodal marginal cell lymphomas (MZL) and may exhibit focal plasma cell differentiation (PCD). PCD could occasionally be extensive, mimicking a true plasma cell neoplasm (PCN). PCN of the thyroid are extremely rare, representing either a component of plasma cell myeloma (PCM), or a primary thyroid extraosseous plasmacytoma (EOPC). We present a case of primary thyroid plasma cell (PC)-rich neoplasm with considerable diagnostic challenge.

Methods

A 46-year-old male underwent total thyroidectomy for hypothyroidism and compressive symptoms. Microscopically, sheets and nodules of PC with focal plasmablastic features were demonstrated in a background of fibrosing chronic thyroiditis. One benign perithyroidal lymph node was seen. The differential diagnosis included B-cell neoplasm with extensive PCD (ie, MZL or lymphoplasmacytic lymphoma) and PCN.

Results

Immunohistochemically, the PCs were positive for CD79a, MUM1, BCL2 and IgM with Kappa light-chain restriction and partial CD138 expression, and negative for CD20, PAX5, HHV8, Cyclin-D1, SOX11, CD117 and IgG. EBER was negative. Rare reactive-appearing B-cell aggregates with associated T-cells were noted. FISH for API/MALT1-fusion t(11;18) and PCR for MYD-88 mutation were negative. However, polysomy 18 (seen in 15–33% of B-cell lymphomas, including MZL) was detected. Serum proteins and PCM workup were negative.

The findings were consistent with primary thyroid Kappa-restricted PC-rich neoplasm. While the absence of detectable B-cell neoplastic component was suggestive of EOPC, the diagnosis of MZL with extensive PCD was favored due to the thyroid involvement in a background of thyroiditis, absence of lymph node involvement, detection of polysomy 18, and absence of MYD-88. Follow-up of the patient was thought to be most reasonable.

Conclusion

Plasma cell-rich hematolymphoid neoplasms of the thyroid may cause a considerable diagnostic challenge. Differentiating between B-cell lymphomas with extensive PCD and PCN may be difficult or even impossible by morphology alone. Additional ancillary tests may be necessary.
Case Report: Coexistence of Adenoid Cystic Carcinoma and Adenoid Basal Carcinoma in the Uterine Cervix
Authors: Tran T T , Bhuyan R , Peng S
Published: October 2020

Introduction/Objective

Adenoid cystic carcinoma (ACC) and adenoid basal carcinoma (ABC) are very rare tumors, accounting for less than 1% of all cervical adenocarcinomas. ACC is an aggressive neoplasm and has poor prognosis while ABC has a favorable prognosis. Coexistence of ACC or ABC and squamous intraepithelial lesion/invasive squamous cell carcinoma in the uterine cervix has been reported in the literature; however, coexistence of ACC and ABC in the uterine cervix is very rare.

Methods

Case presentation: Here we present a case of coexistence ACC and ABC in a 66-year-old woman who suffered from heavy bloody vaginal discharge for one year with weight fluctuations. The cervical exam and CT scan showed a friable mass at the uterine cervix, measuring up to 5.5 cm, and cervical mass punch biopsy was performed. Histopathological examination of the specimen revealed an intact nonkeratinizing stratified squamous epithelium overlying proliferation of nests punctuated by small round spaces, resembling cribriform pattern in the stroma. The tumor cells were small and basaloid with hyperchromatic nuclei and scant cytoplasm. Palisading of tumor cells at the peripheral of the tumor nests were observed in some areas. Mitotic figures and necrosis were not seen.

Immunohistochemical stains were performed and showed two distinct patterns of ACC and ABC. ACC tumor cells were positive for CD117, focally positive for CAM5.2 and S100, and negative for p63. ABC tumor cells were positive for p63 and EMA and negative for CD117 and S100. Both ACC and ABC components were positive for p16, Sox 10, BCL-2 and vimentin and negative for CK7, p40, CEA and ER. PAS stain showed positive staining in the basement membrane-like material in ACC component. A final pathologic diagnosis of coexistence of ACC and ABC of the uterine cervix were made.

Conclusion

Literature review revealed that this was an exceedingly rare case of coexistence of ACC and ABC in the uterine cervix. It is extremely important to correctly diagnose ACC and ABC as there is very poor prognosis in the former and good prognosis in the latter.