bluuebunny/crossref_metadata_2025 huggingface dataset:
Comparison of laboratory wave generation techniques on response of a large monopile in irregular sea
Authors: Dadmarzi F H , Bachynski-Polić E E
Published: November 2022

As the offshore wind industry moves toward larger monopile turbines, model testing and validation of hydrodynamic load models become more important for new designs of turbines. Wave generation is an important aspect of hydrodynamic model testing. When generating irregular waves with a piston-type wavemaker, first order wavemaker theory is commonly used. This leads to generating spurious free waves in the tank. Using second order wavemaker theory reduces the generation of these spurious waves. In this study, the two wave generation techniques have been used in the measurement of the dynamic responses of a monopile with (full-scale) natural period of 5 sec. The effect of superharmonic spurious waves on the response statistics was minor. A marginal improvement in experimental repeatability of the second mode response in large wave events was observed by using second order wavemaker theory.
Design and analysis of a ten-turbine floating wind farm with shared mooring lines
Authors: Hall Matthew , Lozon Ericka , Housner Stein , Sirnivas Senu
Published: November 2022

This paper discusses the development and analysis of a novel design for a 10-turbine floating wind farm with shared mooring lines. Shared mooring lines tether adjacent floating platforms together, reducing the number of anchors required but increasing system complexity. We present a systematic, multistage design process for shared mooring systems, involving linearized analysis of array layout options, quasi-static mooring line optimization, and design refinement based on coupled dynamic loads analysis. The design developed from this process is thought to represent one of the most advantageous shared-mooring configurations for this scale of floating wind array. It features perpendicular anchor line pairs and allows shared, multiline anchors, making it an example of a shared-mooring-and-anchor array. Comparing the performance and cost characteristics of the shared-mooring design with more conventional three-line individual mooring systems shows equivalent dynamic response characteristics and stationkeeping system cost savings of 25% when using shared mooring lines and shared anchors. The design is also advantageous in the case of a mooring line failure, with offsets and redundancy characteristics similar to four-line individual mooring systems.
Seabed bathymetry and friction modeling in MoorDyn
Authors: Housner Stein , Lozon Ericka , Martin Bruce , Brefort Dorian , Hall Matthew
Published: November 2022

This paper presents the implementation and verification of a seabed bathymetry feature and seabed friction feature to the open-source, lumped-mass mooring system dynamics modeler, MoorDyn, which is part of the National Renewable Energy Laboratory’s aero-hydro-servo-elastic simulation tool, OpenFAST. Variations in seabed slope, as well as the frictional effects of mooring lines moving along the seabed, will affect the mooring line tensions of a floating platform and the consequent platform response. These new features are especially relevant for modeling mooring systems in deep-water coastal areas where seabed depth can change significantly over an entire mooring footprint. The bathymetry feature models the seabed as a rectangular grid of variable water depths in place of the existing, uniform water depth in MoorDyn. The friction force is primarily represented as a Coulombic friction force, or the product of a kinetic friction coefficient and the seabed contact normal force, with the ability to differentiate between transverse and axial motion of a line node on the seabed in any bathymetry grid. These capabilities were tested by running MoorDyn and OpenFAST simulations over a variety of seabed and environmental conditions; the resulting fairlead tensions, node tensions, and mooring line kinematics were verified against equivalent OrcaFlex simulations. The results match closely, meaning the features are verified, which will increase the overall fidelity of OpenFAST and FAST.Farm simulations.
Impacts of Competitive Seabed Allocation
Authors: Laido Ahti Simo , Kitzing Lena
Published: November 2022

Impacts of Competitive Seabed Allocation

Offshore wind is an important part of the transition to green energy sources. The industry relies on access to economical seabed, which is a scarce and valuable resource controlled and managed by governments. In this paper we analyse the impacts of the fees embedded in the seabed lease agreements for the economics of offshore wind. We describe the allocation schemes for seabed including pre-qualification, fees, and method of competition for the examples of UK (England, Wales, and Northern Ireland), UK (Scotland), the United States and the Netherlands. Then, we use a discounted cash flow analysis to assess and compare the timeline and magnitude of the fees. Finally, we describe the incentive structures that these fees create for wind farm design. We show that the costs embedded in the seabed lease agreements can be extensive and have been trending upward over time. We also find that the incentive structures are varied and not necessarily aligned with societal goals.
Influence of lateral rotor spacing on the benefits in power generated by multi-rotor configurations
Authors: Martín-San-Román Raquel , Benito-Cia Pablo , Azcona-Armendáriz José , Cuerva-Tejero Alvaro
Published: November 2022

In this work, an analysis of the rotor wakes interaction for different array configurations of onshore wind turbines, has been made using CENER’s in-house aerodynamic module, called AeroVIEW. The study focuses on how the distance between rotors affects the increases in power and thrust obtained by configurations with more than one laterally aligned rotor. Two configurations of laterally aligned multi wind turbines of NREL 5-MW type, operating at 8 m/s wind speed and 9.21 rpm, have been analyzed. The first one consists of 5 wind turbines and the second one consists of 2 wind turbines. These configurations have been studied for different separation distances between the rotors hubs, between 1.1 diameters and 3 diameters. The increases obtained in power with AeroVIEW are in line to the results of high fidelity tools found in the literature. The results show a higher increment of power with lower separation distances. Moreover, this beneficial effect on the power generated, has a counterpart in the average thrust, whose increase is around half of the power increase.
A parametric optimization approach for the initial design of FOWT’s substructure and moorings in Brazilian deep-water fields
Authors: Mas-Soler Jordi , Amaral Giovanni A. do , Silva Luccas Z. M. da , Malta Edgard B. , Carmo Lucas Henrique S. , Ruggeri Felipe , Simos Alexandre N.
Published: November 2022

As Floating Offshore Wind Turbines (FOWT) continue to demonstrate their technical feasibility in different projects, the conceivable scenarios for the application of this technology rapidly expand. Outside the mainstream perspective of floating farms connected to onshore grids, several projects currently study the technical and economic feasibility of adopting FOWTs to supply part of the electrical power required by the offshore production of oil and gas. Having this goal as a main target, the present work addresses initial results obtained in a comprehensive research project that aims to prospect potential uses for FOWT technology in the context of offshore oil and gas production in the Brazilian oil fields. In this context, a parametrical optimization procedure has been adopted to provide suitable candidates for the floating substructures and their mooring systems. The optimization framework was designed for automatic generation of geometrical concepts already considering the equilibrium involving hull weight, ballast and the tensions applied by the mooring lines. The latter are modeled in hybrid configuration, composed of sections of steel chains and polyester rope, considering both catenary and taut-leg configurations. The main goal is to minimize CAPEX costs but, knowing that the acceleration levels in the rotor hub are inevitably linked to indirect costs that are hardly predictable at this stage, a multi-objective approach is preferred, taking the minimization of the nacelle acceleration as one of the drives. Moreover, for a better resolution of the site-specific design, the optimization is done not only for a few pre-selected metocean conditions, but considering a long-term series of the simultaneous action of seas, swell waves, currents and winds. In this paper, two illustrative concepts with different floater geometries are presented, both of them optimized for a water depth of 600m. A discussion on the mooring configurations is made and the rationale involved in the optimization procedure that leads to the final floater dimensions is investigated by connecting the floater motion responses to the wave characteristics in the chosen field.
Deep ensemble with Neural Networks to model power curve uncertainty
Authors: Perez-Sanjines F , Verstraeten T , Nowé A , Helsen J
Published: November 2022

Renewable energy is an essential driver towards tackling the climate crisis. Given the increasing need to ensure sustainability and reduce greenhouse gas emissions, wind energy has become one of the most relevant areas to consider in today’s society. However, it appears challenging to rely on this type of energy because of the uncertain nature of the wind. It is essential to use monitoring tools that can accurately capture the uncertainty of the underlying environmental processes and estimate the generated power to ensure the reliability of wind-based power systems. One of these monitoring tools is the power curve, which captures the relationship between wind speed and expected power production. Many regression techniques have previously been used to predict this power production, including Artificial Neural Networks (ANN). The problem is that they do not capture the uncertainty of the predicted power production. In contrast with the power curve models already implemented when using ANNs, we propose a novel approach based on deep ensembles to predict the mean and model variance on the expected power production. We used three months of Supervisory Control and Data Acquisition data from more than 40 turbines to set up the experiments. The results show that the mean and standard deviation fit the data well. It also appears that the expected mean curve goes through the center of the cloud of original data points, while the standard deviation accurately captures the spread of the data. We conclude that that the deep ensemble approach provides an accurate prediction mechanism to estimate the power production from the wind speed while also offering an uncertainty measure. Therefore, the deep ensemble approach allows us to construct a power curve with uncertainty information.
Impact of hydrodynamic drag coefficient uncertainty on 15 MW floating offshore wind turbine power and speed control
Authors: Sandua-Fernández I , Vittori F , Eguinoa I , Cheng P W
Published: November 2022

The main objective of this study is to analyse the impact of the drag coefficient uncertainty on large floating offshore wind turbines with respect to the power performance and generator speed control. In order to do so, the recently published IEA 15 MW reference wind turbine mounted on two different floating platform types (semi-submersible UMaine VolturnUS-S and spar WindCrete) has been selected. The platforms have been divided by components (columns, heave plates and pontoons) and the drag coefficient uncertainty has been quantified for each component, using a range of values from the literature that is representative for normal sea state conditions. Dynamic simulations have been performed for both floating platforms operating in a variety of environmental conditions (wind and waves), with a range of drag coefficients to account for the variability. Then, operating variables (generated power, generator speed, blade pitch angle. . .), platform motions and hydrodynamic forces have been analysed, to determine how large the impact due to the variations of the drag coefficient is. Results have shown that the considered range of drag coefficient uncertainty, although being quite large, has negligible impact on the performance of the wind turbine control response, irrespective of the floating platform type.
Fast Harmonic compensation in hybrid HVDC offshore system
Authors: Tian Shangen , Campos-Gaona David , Peña-Alzola Rafael , Anaya-Lara Olimpo
Published: November 2022

Hybrid HVDC systems have been proposed as an alternative for nominal VSC-Based HVDC for offshore applications. Hybrid HVDC systems consist of an offshore power station composed of the connection of high-power diode rectifiers in series with a fractional power VSC-HVDC. This hybrid configuration allows large power transfer from offshore sites, with the added robustness, simplicity and efficiency of uncontrolled rectifiers. In this research, a robust and fast-acting controller, the Two Degrees of Freedom Internal Model controller (2DF-IMC), is used to control the active power filter features of the fractional-power VSC-HVDC system, resulting in a much faster overall THD reduction in the offshore AC currents in dynamic conditions (i.e. time-varying wind power) when compared with standard active power filter controllers. This improvement is the direct consequence of the fast closed-loop dynamics of the 2DF-IMC controller that do not require filtering stages. Additionally, the increased closed-loop response time did not affect the overall robustness of the control system, thanks to the enhanced disturbance rejection capabilities of the 2DF-IMC configuration.
A review of failure prognostics for predictive maintenance of offshore wind turbines
Authors: Zhang Wanwan , Vatn Jørn , Rasheed Adil
Published: November 2022

Offshore wind turbines (OWTs) are important facilities for wind power generation because of their low land use and high electricity output. However, the harsh environment and remote location of offshore sites make it difficult to conduct maintenance on turbines. To upkeep OWTs cost-effectively, predictive maintenance (PdM) is an appealing strategy for offshore wind industry. The heart of PdM is failure prognostics, which aims to predict an asset’s remaining useful life (RUL) based on condition monitoring (CM). To provide references to PdM of OWTs, this paper presents a systematic review of failure prognostic models for wind turbines. In this review, data-driven models, model-based models, and hybrid models are classified and presented for model selection. The findings reveal that it is promising to develop hybrid models in the future and combine the advantages of data-driven and model-based models. Currently, the internal combinations of machine learning methods and statistical approaches in data-driven models are more common than exterior linkages between data-driven models and model-based models. The limitations and strengths of different models are discussed, and opportunities for developing hybrid models are highlighted in the conclusion.
Distribution and intensification of pig production in China 2007–2017
Authors: Zhao Q , Dupas M C , Axelsson C , Artois J , Robinson T P , Gilbert M
Published: November 2022

Driven by population growth and rising incomes, the demand for animal source foods in low and middle-income countries is increasing rapidly. Pork is one of the most commonly consumed animal-based food, with the highest demand being in China due to its largest population and changing dietary habits linked to increasing wealth. Here, we show the changes in pig production systems in terms of farms capacity, productivity and production at the national and provincial levels by analyzing several censuses of China. In addition, we used a downscaling methodology to provide a recent and highly detailed map of the distribution of pigs in China. Between 2007 and 2017, pork production in China increased by 26.6%, up to 55 million tons and the number of large-scale farms with a yearly production of over 10 000 heads increased by 145%. Much of the production has changed from extensive backyard subsistence farming to intensive corporate farming. Moreover, the pig distribution has shifted from watercourse-intense southeast to northeast and southwest of China due to environmental policy in 2015. These policy-driven transitions primarily aimed to increase pig production efficiency and reduce environmental impacts and resulted in a profound transformation of geographic production patterns.
Algebraic structure of Dirac Hamiltonians in non-commutative phase space
Authors: Falomir Horacio , Liniado Joaquin , Pisani Pablo
Published: November 2022

In this article we study two-dimensional Dirac Hamiltonians with non-commutativity both in coordinates and momenta from an algebraic perspective. In order to do so, we consider the graded Lie algebra

s
l

(
2

|

1
)

generated by Hermitian bilinear forms in the non-commutative dynamical variables and the Dirac matrices in

2
+
1

dimensions. By further defining a total angular momentum operator, we are able to express a class of Dirac Hamiltonians completely in terms of these operators. In this way, we analyze the energy spectrum of some simple models by constructing and studying the representation spaces of the unitary irreducible representations of the graded Lie algebra

s
l

(
2

|

1
)
⊕

s
o

(
2
)

. As application of our results, we consider the Landau model and a fermion in a finite cylindrical well.
The growth constant for self-avoiding walks on the fcc and bcc lattices
Authors: Clisby Nathan
Published: November 2022

We extend a binary tree implementation of the pivot algorithm to the face-centered cubic and body-centered cubic lattices, and use it to calculate the growth constant, µ, for self-avoiding walks on these lattices. We find that

μ

f
c
c

=
10.037
057
85
±
0.000
000
14

and

μ

b
c
c

=
6.530
511
501
±
0.000
000
084

. In addition, we estimate the amplitudes

A

f
c
c

=
1.17119
±
0.00003

and

A

b
c
c

=
1.17637
±
0.00003

, and provide convincing numerical evidence in support of the hypothesis that the critical exponent γ is a universal quantity.
Evolution of ionization waves in a multi-pulsed plasma jet: the role of memory charges
Authors: BABAEVA N Yu , NAIDIS G V , TARASENKO V F , SOROKIN D A , ZHANG Cheng , SHAO Tao
Published: November 2022

In this paper, we discuss the properties of ionization waves (IWs) in a multi-pulsed plasma jet while using the two-dimensional computational approach. The IWs are generated by application of three short negative pulses with a repetition frequency 12.5 MHz. The simulations are performed continuously during a single run while accounting for charges accumulated inside (surface charges) and outside (space charges) the tube. The plasma forming gas mixture (He/O2 = 99.8%/0.2%) is injected through the discharge tube into the surrounding humid air. We show that an IW can emerge from the tube exit at a pulse rising edge (as a negative IW) and at a falling edge of the same pulse (as a positive IW). It is demonstrated that remnants of the negative and positive charges play an essential role in the discharge evolution. The first pulse travels the shortest distance as it propagates through the initially non-ionized environment. The IWs developing during the second pulse essentially enlarge the plasma plume length. At the same time, the IWs generated by the third pulse eventually decay due to the remnants of charges accumulated during the previous pulses. Accumulated memory charges can lead to the IW extinction.
Clinical Utility of Next-Generation Sequencing Panel Testing in the Evaluation of Arteriovenous Malformations
Authors: Hernandez Patricia , King Katherine , Evenson Michael , Corliss Meagan , Schroeder Molly , Krysiak Kilannin , Heusel Jonathan , Neidich Julie , Cao Yang
Published: November 2022

Arteriovenous malformations (AVMs) are vascular lesions in which an overgrowth of blood vessels of varying sizes develops with one or more direct connections between the arterial and venous circulation. AVMs are seen in sporadic and syndromic conditions that are caused by a variety of genomic alterations. Here, we performed a retrospective review of the cases sent for next-generation sequencing (NGS) analysis for diseases of somatic mosaicism (DoSM). Specimens from 54 patients with clinical indication of AVMs were submitted for the DoSM NGS panel between October 2013 and Dec 2021. DNA extraction and sequence analysis were performed on affected tissues, including fresh tissue, formalin-fixed paraffin-embedded tissue, fibroblast cultures, and buccal specimens. Single-nucleotide variants (SNVs) with variant allele fractions (VAFs) greater than 3% and small insertions and deletions (indels, <21 bp) were called using VarScan2, Genome Analysis Toolkit (GATK), and Pindel. Variants with VAFs between 1-3% were manually reviewed based on the sequencing data quality and the known clinical significance. 38/54 (69.1%) patients were females and 16/54 (30.9%) were males. Ages ranged from 1 month to 73 years (median age 17 years). Most of the patients were indicated with sporadic AVM (81%), although additional clinical indications were noticed, including unspecified congenital vascular malformation (9.1%), capillary malformation (5.5%), arteriovenous fistula, and AVM associated with capillary and lymphatic malformations (1.8% each). The biopsied lesion was most often located in the head (43.6%), followed by the limbs (30.9%), and unspecified areas of the body in the remaining 25.5%. Among the 54 cases, 37 (68.5%) cases had pathogenic and/or likely pathogenic (P/LP) variants identified, 2 cases (3.7%) had variants of unknown significance, and the remaining 15 cases (27.8%) had negative results. MAP2K1 variants were found in 12 samples, followed by KRAS (8), TEK (7), PTEN (5), BRAF (4), TSC2 (2), HRAS (2), RASA1 (2), and PDGFRB (1). Of note, four cases had two P/LP variants. Among the 37 positive cases, 32 cases had somatic alterations; the remaining 5 cases had at least one germline P/LP variant, including PTEN (4) and RASA1 (1). In summary, the diagnostic yield of cases with clinical indication of AVMs was 68.5% using our in-house DoSM NGS panel. This study demonstrates the clinical utility of the DoSM NGS panel testing in the evaluation of a cohort with clinical presentations of AVMs.
Magnitude and Histopathological Patterns of Liver Diseases among Forensic Autopsy Decedents at Muhimbili National Hospital, Dar-es-salaam- Tanzania
Authors: Mlaga S M
Published: November 2022

Introduction/Objective

Liver diseases are not infrequent. Majority of liver disorders proceed silently and manifests at later stages. To date no data has been published on the prevalence of liver diseases discovered during autopsy in our country and thus, occurrence and frequency of these diseases is not known. This work aimed at determining the prevalence and the histopathologic features ascribed to specific disease entities, utilizing the opportunity of autopsy cases.

Methods/Case Report

A prospective cross-sectional study, autopsy based was conducted at Muhimbili National Hospital Histopathology Laboratory premises in which Medical-legal autopsies received at MNH mortuary facility with causes of death other than liver diseases were enrolled for the study. Demographic information and circumstance of death were established from respective forms. Gross appearance of the liver was recorded prior to sampling of the tissue for histopathological studies. The sampled tissue specimen were fixed in 10% neutral buffered formaline prior to grossing and processing. Special stains were use when deemed necessary for enhancing definitive diagnosis.

Results (if a Case Study enter NA)

This study recruited 253 forensic autopsy cases, in which majority were males 218(86%) and the mean apparent age at death was 36 +/- 12.98(SD) with the peak age group of the study participants was 26-35yrs 77(30.4%). Bulk of cases had fat change 83(32.8%), followed by steatohepatitis 37(14.6%), and granulomatous lesions 27(10.7%), of the 27, the majority 18 cases were specified as hepatic schistosomiasis 8(3.2%). +Combined infective lesions summed up to 34(13.4%) when suspects of viral hepatitis were added to granulomatous lesions. Majority of cases crowded in the younger age groups.

Conclusion

To the best of my knowledge, this study is the first of its kind in our region. It shows there are diverse and prevalent liver diseases afflicting younger forensic decedents estimating the odds among apparently healthy population in our setting.
Sudden Cardiac Death due to Giant Cell Myocarditis in a 37-Year-Old Female: Case Report
Authors: Nobee A , Yin J , Hassler J , Rong Y
Published: November 2022

Introduction/Objective

Giant cell myocarditis (GCM) is a rare and aggressive inflammatory process that targets the myocardium and is often rapidly fatal. Most cases have been reported in young to middle aged adults with a slight male predominance. The etiology of this disease is largely unknown, however there is an association with multiple autoimmune disorders. Most patients present with rapidly progressive or fulminant heart failure, arrhythmias, heart block or sudden cardiac death. The incidence of GCM ranges from 0.007% to 0.051% and most cases are confirmed on autopsy examination. Here we present a case of GCM in a young female diagnosed on autopsy.

Methods/Case Report

A 37-year-old female presented with shortness of breath and chest pain for a 5-day duration. She had no past medical history and was otherwise well before the onset of symptoms. On admission, she was noted to have clinical findings consistent with heart failure and progressed to cardiogenic shock with ventricular arrhythmias within hours. Despite high dose corticosteroids and other supportive therapy, the patient remained in refractory, vasodilatory shock, in cardiac standstill and eventually died. Findings on autopsy included mild ventricular hypertrophy, petechial hemorrhages through out the atria and ventricles, as well as pulmonary edema and congestion. Microscopic examination showed a diffuse infiltrating pattern of inflammation within the heart, that was composed predominantly of lymphocytes, giant cells, scattered neutrophils, and eosinophils. The lymphocytes were CD3, CD4, CD8 positive and CD20 negative, consistent with a T-cell infiltrate. Gram and GMS stains were negative for bacterial and fungal microorganisms. Focal areas of necrosis without granuloma formation are also noted ruling out sarcoidosis.

Results (if a Case Study enter NA)

NA.

Conclusion

This case demonstrates a rapid manifestation of GCM in a previously healthy individual. GCM must be considered in the differential diagnosis when a young patient presents with cardiac symptoms. Early diagnosis and initiation of targeted therapy is critical for GCM patients' survival.
Lewy Body Pathology and Alzheimer Disease in Down Syndrome
Authors: Movassaghi M , Lou J J , Wright S , Silva J , Leavy K , Kim R , Monuki E S , Perez-Rosendahl M , Head E , Yong W H
Published: November 2022

Introduction/Objective

Aging adults with Down syndrome (DS) develop Alzheimer disease neuropathology (AD) by the age of 40 years, primarily due to the overexpression of the amyloid precursor protein on chromosome 21. Lewy bodies (LBs), containing alpha-synuclein protein, are observed in 7-60% of AD patients in the amygdala and in cortex. Prior DS studies (n=20-56 cases) find the frequency of LB pathology to range between 8-50% of cases being affected. We hypothesized that LB pathology would also be present in DS brain with similar locations and prevalence to AD. Thus, we evaluated the frequency of LB in our UCI cohort of DS cases that we have collected over the past 25 years.

Methods/Case Report

Neuropathology reports from 55 cases with DS from the UCI-ADRC were included in this study. Cases were stained for beta-amyloid, phosphor-tau, alpha-synuclein and TDP-43 as per NACC protocols (one case each v7,8,9 and three v11).

Results (if a Case Study enter NA)

We identified 6 cases (10.9%), all male, with a mean age of 57 years (SD=3) that showed LB and/or Lewy neurites. LB pathology was classified as amygdala predominant in 3 cases, brainstem predominant in one, intermediate/transitional in one, and diffuse/neocortical in one. Five cases were BRAAK stage 6 and one was stage 5. Five cases had CERAD neuritic plaque score C and one case had a B score. Two of 3 cases were Thal phase 5, and one was phase 4. The case with diffuse/neocortical LB pathology demonstrated hippocampal sclerosis.

Conclusion

The observation that all our LB positive cases were male may reflect a sample bias. In our study, Lewy pathology was most common in amygdala but other sites of involvement are seen similar to a prior DS study and AD studies. Prior DS studies (n=20-56 cases) find the frequency of LB pathology to range between 8-50% of cases being affected. The prevalence of LB in our DS cohort (10.9%) is in the low end of the range seen in other DS and AD studies.
Lipoblastoma-like tumor of the vulva: A rare benign mesenchymal neoplasm mimicking myxoid liposarcoma
Authors: Surucu A , Warmke L
Published: November 2022

Introduction/Objective

Lipoblastoma-like tumor of the vulva (LLTV) is a rare, benign mesenchymal neoplasm of adipocytic differentiation that typically presents as a vulvar mass in young women, clinically mimicking a Bartholin gland abnormality. The tumor is well-circumscribed and multilobulated, composed of mature adipocytes, uniloculated lipoblasts and bland spindle cells. Morphologic features often overlap with lipoblastoma, myxoid liposarcoma, and spindle cell lipoma; however, absent alterations of PLAG1 and HMGA2 distinguish it from lipoblastoma, and lack of DDIT3 gene rearrangement distinguishes it from myxoid liposarcoma. While Rb1 usually demonstrates a “mosaic” pattern of expression, no definitive evidence of a structural abnormality in 13q has been identified.

Methods/Case Report

A 24-year-old female presented with mild discomfort and persistent swelling of the right vulva for one year, which was initially thought to be a Bartholin gland cyst. Physical examination revealed marked asymmetry between the right and left vulva, and ultrasound examination revealed an ovoid, hypoechoic, and solid- appearing vulvar mass (4.7 x 2.5 x 1.1 cm). She subsequently underwent excision of the vulvar mass for diagnosis and treatment.

Results (if a Case Study enter NA)

The excision specimen showed a relatively well-circumscribed and multi-lobulated subcutaneous mass with myxoid stroma and thin fibrous septa. The tumor was composed of a mixture of bland spindle cells, uniloculated lipoblasts and mature adipocytes. No significant nuclear atypia, necrosis or mitotic activity was identified. Immunohistochemical stains showed that the tumor cells were negative for keratin cocktail, S100 protein, SMA, and Desmin. CD34 highlighted vessels within the lesion, and Rb1 showed a "mosaic" pattern of weak and negative expression.

Conclusion

Lipoblastoma-like tumor of the vulva (LLTV) is a rare, benign tumor that tends to occur in young women, often clinically mimicking a Bartholin gland cyst. Recognition of this rare entity with distinction from mimics including myxoid liposarcoma is essential to avoid overly aggressive treatment. Complete conservative excision is recommended.
Penile Ewing's Sarcoma - A Rare Entity
Authors: kaur J , Banerjee N
Published: November 2022

Introduction/Objective

Ewing sarcoma is a cancer involving the bones or the tissue around bones. It is the second most common malignant bone tumour comprising 10-15% of all primary bone tumours, most commonly affecting children and young adults. It can also be seen in adults also. Males are more commonly affected then females. Any bone can be affected but the lower extremity, the pelvis, upper extremity, axial skeleton and ribs, and face cases. Similar histology tumours when seen in soft tissue are peripheral primitive neuroectodermal tumour (pPNET), neuroepithelioma, and Askin tumour. It is always difficult to know the primary site when soft tissues are involved as it can be infiltration from bone or from soft tissue to bone. Penis is least common site involved with Ewing’s sarcoma in young male.

Methods/Case Report

This is a case report of a 22 year old male with penile lesion. He presented with the chief complaints of mass at the shaft of penis. The partial penectomy specimen was received along with the bilateral inguinal lymph node. On gross there was a ulcero-proliferative growth.

Results (if a Case Study Enter NA)

On histopathological examination showed small blue round cell tumour and further immunohistochemistry with CD99 and NKX2.2 revealed the diagnosis of Ewing’s sarcoma, however lymph nodes showed reactive lymphoid hyperplasia.

Conclusion

The Ewing sarcoma at the penile region is rarest entity. Only 7 cases are reported worldwide. The imaging modalities like CT or MRI can be used to know the extension. The diagnosis is primarily based on morphological features, immunohistochemical analysis and translocation analysis. However it is important to find out if any other soft tissue site is involved. The important prognostic markers are presence and absence of metastasis, size of tumour, necrosis and presence of fusion transcripts.
Clinical-Pathological Correlation of Breast Non-mass Enhancing Lesions
Authors: Huang W , Han B
Published: November 2022

Introduction/Objective

MRI imaging of breast is mainly reserved for screening high-risk individuals with occasional application in women bearing moderate or average risk. Areas showing enhancement in MRI without space-occupying mass are defined as non-mass enhancement (NME). As biopsy of NME lesion is rare encounter in clinical practice, thorough clinical-pathological investigation is warranted.

Methods/Case Report

289 patients who underwent core biopsy for NME lesion(s) in our institution between 2011 to 2021 were included in this retrospective study. Pathological diagnoses were lumped into 3 categories, namely benign, precursor lesions (atypical ductal hyperplasia, atypical lobular hyperplasia, and lobular carcinoma in situ), and malignant lesions (ductal carcinoma in situ, pleomorphic lobular carcinoma in situ, and invasive carcinoma). The age distribution, MRI indications, and pathological diagnoses stratified by indications were studied. MRI patterns of NME lesions were compared between malignant versus benign lesions.

Results (if a Case Study enter NA)

Patient’s age ranges 25-79 yo (mean age 51.9 yo, median age 50 yo). Indications of MRI include high-risk screening (162 cases, 162/289, 56%), pre-operative staging (116 cases, 116/289, 40%), and problem-solving (11 cases, 11/289, 4%). There are 220 benign cases (220/289, 76%), 50 malignant cases (50/289, 17%), and 19 precursor lesions (19/289, 7%) in this study. There were 16 malignant cases identified in the high-risk group (16/162, 10%), 24 in the pre-operative staging group (24/116, 20.7%), and 2 in problem-solving group (2/11, 18.2%). Focal distribution is significantly associated with malignant lesions as compared to other patterns (linear, segmental, and regional) (Chi-square test, p<0.001).

Conclusion

High-risk screening is the major indication for biopsy NME lesions, followed by pre-operative staging, and problem-solving. Benign lesions are the predominant pathological findings of NME lesions, followed by malignant and then precursor lesions. Malignant lesions were mostly identified in high-risk group, followed by pre-operative staging and problem-solving group. Malignant lesions tend to assume the “focal distribution” than other MRI patterns.
Interstitial Duplication on Chromosome 3p14.3p13 in an Adolescent with Dysmorphic Features and Autism, Case Report
Authors: Abdelwahed M , Jackson R , Yurtsever N , Singh R , Trunca C , Dompreh R , Herman C , Cohen N , Simotas C , Rousseau-pierre T
Published: November 2022

Introduction/Objective

The genetic etiology of autism spectrum disorders is only partially understood. Here we describe a 16-year-old male diagnosed with autism at two years of age. He has dysmorphic features, severe cognitive disability, and history of cryptorchidism. A review of systems was significant for slightly coarse features overall, with deep-set eyes, pinched nasal bridge with wide nasal tip, and widely spaced teeth. A large pectus excavatum deformity was also noted. Although the parent’s genetic testing concluded that this likely represents de novo mutation, it is worth mentioning that our patient has a 13-year-old female maternal first cousin with learning difficulties that were said to be less severe. There is no other family history of autism.

Methods/Case Report

Chromosome analysis showed an abnormal karyotype identifying a duplication of the short arm of chromosome 3 from p13 to p14.3, 46,XY,dup(3)(p13p14.3). Additional microarray testing confirmed this duplication and defined the size as 14.9 Mb. Regions of homozygosity of 29.8 Mb were also identified, representing about 1% of the autosomal genome. The duplicated area includes over 70 genes, thirteen of which are known Mendelian disease genes (IL17RD, HESX1, APPL1, FLNB, DNASEIL3, PDHB, ACOX2, ATXN7, SLC25A26, EOGT, LMOD3, MITF, and FOXP1). Duplications of 3p are extremely rare and can be de novo or inherited from a parent with a balanced translocation. Individuals with these better-described chromosome 3p duplications typically present with intellectual and developmental disabilities, such as autism, as 3p duplications typically present with intellectual and developmental disabilities, such as autism and distinctive dysmorphic features. This duplication has never been reported as a known syndrome and has minimal overlap with copy number variants (CNVs) among healthy individuals.

Results (if a Case Study enter NA)

NA

Conclusion

Autism spectrum disorders are largely characterized by speech, communication, and social impairment of varying degrees. Diagnosis is typically made on clinical grounds, but in 14-35% of cases, a genetic basis for the disorder, typically either due to a single gene disorder or a chromosomal deletion/duplication, may be found. This case report describes a rare finding of a 14.9 Mb interstitial duplication on chromosome 3p.
Supernatant Fluid from Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration for Rapid Next-Generation Sequencing
Authors: Jager L , Jennings L J , Blanco J , Choy B , Nayar R
Published: November 2022

Introduction/Objective

The aims of our study were to optimize the workflow of non-small cell carcinoma (NSCC) endobronchial ultrasound-guided bronchoscopy with transbronchial needle aspiration (EBUS-TBNA) samples to maximize tissue available for next-generation sequencing (NGS), preserve formalin-fixed paraffin-embedded (FFPE) cell blocks (CBs) for future testing, and shorten turnaround time (TAT) of NGS results. We evaluated the performance of supernatant fluid (SNF) processed from a dedicated aspirate for NGS testing.

Methods/Case Report

20 EBUS-TBNA samples positive for NSCC on rapid on-site evaluation were collected and processed using a new workflow (Figure 1). Five aspirates were collected in formalin. One additional dedicated pass was collected fresh and centrifuged. The resulting cell pellet was added to the passes in formalin for FFPE CB processing. The SNF was recentrifuged. DNA and RNA were extracted from concentrated SNF for targeted testing using the Oncomine™ Precision Assay (Thermo Scientific™, Waltham, MA). NGS results from the corresponding FFPE CBs were used as “controls” for comparison.

Results (if a Case Study enter NA)

A total of 31 mutations were detected in SNF (Table 1). The most frequently mutated genes were TP53 (35%), EGFR (23%), KRAS (13%), CTNNB1 (6%), and ERBB2 (6%). EGFR and KRAS amplification, CDKN2A deletion, and SQSTM1-NTRK3 fusion alteration were also detected. There was 100% concordance between the mutations detected in SNF and corresponding FFPE CBs with comparable variant allele frequencies. TAT of NGS results was 1 day for SNF compared to 4 – 10 days for FFPE CB.

Conclusion

In our study, we were able to demonstrate the usefulness of NGS on SNF to provide reliable, rapid molecular results. This testing strategy was successfully incorporated into the workflow for tissue handling and processing between our clinical, cytopathology, and molecular teams. Molecular results were available at the same time as the cytologic diagnosis, allowing for timely reporting of a comprehensive diagnosis. This approach is particularly useful in patients with advanced disease requiring urgent management.
Interleukin 17 Receptor C, but not Polo-like Kinase 4, Plays a Critical Role in the Development of Skin Papilloma and Psoriasis in Mouse Models
Authors: Jin B , Zhang Y , Miller H , He L , You Z
Published: November 2022

Introduction/Objective

Hyperproliferation of epidermis is a histopathological hallmark of skin cancers and psoriasis. As a master regulator of centriole replication, overexpression of Polo-like kinase 4 (PLK4) has been identified in skin cancers. The aberrant proliferation of epidermal keratinocytes provoked by interleukin 17 (IL17) leads to psoriasis. Thus, targeting centriole replication and IL17 signaling simultaneously has been speculated as a potential therapeutic strategy. We hypothesized that inhibition of centriole duplication enhances the blockade of epidermal proliferation through Il17rc knockout.

Methods/Case Report

To test our hypothesis, 37 mice were used to imbed a two-stage model of skin carcinogenesis using wild-type (WT), IL17 receptor A (T779A) knock-in (Il17ra(T779A)-KI), and IL17 receptor C knock-out (Il17rc-KO) C57BL/6J mouse strains. Furthermore, an imiquimod-induced psoriasis model was established using 69 mice with the same strains.

Results (if a Case Study enter NA)

In our two-stage skin carcinogenesis model, Il17ra(T779A)-KI mice showed significantly decreased tumor incidence, tumor multiplicity, and tumor volume compared to the WT mice. Il-17rc-KO mice didn’t develop any skin papilloma. The skin papilloma formed never progressed into squamous carcinoma. However, centrinone, a selective inhibitor of PLK4, didn’t affect skin papilloma formation or epidermal thickening. In our psoriasis model, the epidermis thickness of Il-17rc-KO mice was dramatically decreased compared to WT and Il17ra(T779A)-KI mice. There was no significant difference between WT and Il17ra(T779A)-KI mice in terms of skin lesion and thickening of the epidermis. Centrinone didn’t stall the thickening of the epidermis in the psoriasis model. IHC staining showed significantly increased Ki67+ basal keratinocytes in the untreated skin of Il17ra(T779A)-KI male mice compared to WT mice. In imiquimod-treated skin, the percentage of Ki67+ basal keratinocytes significantly decreased in the order from WT, Il17ra(T779A)-KI to Il17rc-KO mice.

Conclusion

Our data suggest that the proliferation of keratinocytes is not stalled by centrinone but is inhibited by Il17rc-KO. Il17ra(T779A)-KI significantly inhibits skin papilloma formation, but slightly decreases epidermal thickening in the psoriasis model. However, Il17ra(T779A)-KI increases keratinocyte proliferation based on Ki67 staining in the untreated normal skin.
Sarcomatoid Adrenal Cortical Carcinoma: Report of a Rare Case
Authors: Hosseini H , Sham S , Unger P , Yanke B
Published: November 2022

Introduction/Objective

Adrenal cortical carcinoma (ACC) is a rare neoplasm with oncocytic, myxoid, and sarcomatoid variants. Sarcomatoid is the least common variant. To our knowledge, there are only 23 cases of sarcomatoid ACC reported in the English literature. Typically, ACC’s express SF1, Melan A, inhibin, and synaptophysin. It has been reported that the sarcomatoid component does not express the aforementioned immunomarkers; however, it can display mutational expression of p53 and β-catenin.

Methods/Case Report

A 77-year-old man was incidentally found to have a left adrenal gland mass. Hormone levels were unremarkable, and adrenalectomy was performed. Gross examination of the specimen revealed a 4.5 cm well-circumscribed tan and focally hemorrhagic mass confined to the adrenal gland. Histologically, the mass was composed of pleomorphic round to polygonal cells with clear to eosinophilic cytoplasm and spindle cells. The mitotic count exceeded 20 in 50 high power fields. Lymphovascular invasion and extensive necrosis were present. Immunohistochemical studies revealed that the sarcomatoid component expressed SF1 and synaptophysin focally. The non-sarcomatoid component exhibited focal expression of synaptophysin and diffuse expression of SF1 and Melan A. Both components displayed null expression of p53, and neither expressed β-catenin. Ki67 proliferation index was > 95% in the sarcomatoid component.

Results (if a Case Study enter NA)

NA

Conclusion

In sarcomatoid ACC, both sarcomatoid and non-sarcomatoid components have been shown to harbor TP53 and CTNNB1 mutations, with the latter leading to activation of Wnt pathway and causing diffuse and strong nuclear expression of β-catenin. In the present case, tumor cells lacked expression of p53, which might have played a role in the evolution of the tumor.
Elastotic Mass-Forming Lesion of the Omentum; A New Pathological Entity
Authors: Ali M , Zhou Z
Published: November 2022

Introduction/Objective

Elastotic lesions are rare benign mass-forming entity. Although, few reports of mucosal and submucosal elastotic lesions have been previously described, localization of these lesions in the omentum is not previously described in the literature.

Methods/Case Report

We report a case of 60-year-old man with medical history of obesity, hypertension, diabetes mellitus, and a remote abdominal surgery in 1980s who presented to the emergency room complaining of abrupt severe constant epigastric pain that started few hours before. The pain was not relieved by antiacid or analgesic medications. CT of the abdomen showed multiple distended fluid-filled loops of small bowel in mid abdomen with infiltrative changes of mesentery. A diagnosis of small bowel obstruction was made, and patient proceed to have an exploratory laparotomy. During the procedure, extensive adhesions between small bowel and omentum and small bowel obstruction due to a phytobezoar were noted for which he underwent lysis of adhesion, excision of thickened omentum, and enterotomy for removal of significant food bezoar. Grossly, the omentum has multiple chalky tan-gray masses ranging between 1.2 and 1.0 cm in greatest dimension surrounded by indurated fatty tissue. Microscopically, multiple hypocellular foci are seen that are composed almost entirely of truncated elastic fibers, with sparse delicate bands of collagen and scattered prominent thick-walled vessels. Elastic stain highlights these elastic fibers while trichrome stains the elastic fibers pale gray in contrast to the bright blue color of the collagen fibers. Mucicarmine, and Congo red are negative. Patient tolerated the procedure well however, postoperatively he had local wound infection leading to prolonged skin dehiscence.

Results (if a Case Study enter NA)

NA.

Conclusion

Elastotic mass-forming lesions in the omentum is a new entity that has not been described before in this location that can clinically manifest with signs and symptoms of bowel obstruction. We suggested the name of elastomatosis omentum.