bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Challenging and Rare Case of Metastatic Follicular Dendritic Cell Sarcoma Originating from a Tonsil
Authors: Gallardo J , Watson E , Finch C J , Xu Y 
Published: October 2021

Abstract

Introduction/Objective
Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor of follicular dendritic cells. Most cases of FDCS are described in lymph nodes, but it can involve extra-nodal locations such as the tonsil.

Methods/Case Report
We report a case of a 55-year-old male with a 6-month history of a slow growing left neck mass. The patient had a poorly differentiated carcinoma of the left tonsil diagnosed based on morphologic features eight years ago, followed by chemoradiation, and subsequent left tonsillectomy without tumor identified. Imaging reported a 4.0 cm mass in the left neck level III lymph node location. Biopsy of the mass revealed a spindle cell neoplasm. It was difficult to reach a definitive diagnosis despite an extensive work-up. Review of the previous left tonsil biopsy showed a pleomorphic epithelioid cell proliferation. On the radical resection specimen of the left neck mass, a 4.5 cm, tan-gray, fleshy, necrotic mass was present. Microscopically, it demonstrated a neoplasm with spindle cell and epithelioid cell proliferation, with extensive necrosis. A wide panel of immunostains was performed; the tumor cells showed positivity for CD21, CD23, CD35, and focally weak positivity for cytokeratin OSCAR and Synaptophysin. Squamous cell markers were negative. These findings are consistent with FDCS. Further work-up on the original tonsil tumor displayed immunostaining profile of FDCS. This case was consulted with the National Institute of Health (NIH) for confirmation of the diagnosis.

Results (if a Case Study enter NA)
NA

Conclusion
Morphologically, FDCS can present with various histologic patterns, which can lead to diagnostic pitfalls, and common confusion with poorly differentiated carcinoma or soft tissue tumors, especially in needle biopsies. Generally, patients present with a prolonged clinical course, with a 50% and 25% chance of recurrence and metastasis respectively, hence the awareness and accurate diagnosis of this entity is important in biopsies of lymph nodes or of extranodal locations such as a tonsil.
A Diagnostic Pitfall in the Evaluation of CD138-Positive Plasmacytoid Cells in a Fine Needle Aspiration Biopsy
Authors: Ali H , Mai B , Wahed A , Nguyen A , Liu J , Zhu H 
Published: October 2021

Abstract

Introduction/Objective
The identification of plasmacytoid cells on rapid assessment leads to a wide range of differential diagnoses.

Methods/Case Report
We present a 51-year-old female with a parotid mass and cervical lymphadenopathy. The fine needle aspiration biopsy of the lymph node showed clusters of plasmacytoid cells. Flow cytometric studies showed cells positive for CD19, CD45, CD138 and kappa, but negative for CD38 and lambda. The kappa to lambda ratio was greater than 100. Histological sections showed plasmacytoid cells that were positive for AE1/AE3, S100, and SOX10, weakly positive for CK7 and GATA3, but negative for CD79a, CD45, EMA, p40, p63, and pan-melanoma markers. Kappa and lambda had a 1:1 ratio. Due to the discrepancy between the flow cytometric results and the immunohistochemistry, excision was recommended. The subsequent excision specimen showed classic morphology for myoepithelial carcinoma, plasmacytoid variant.

Results (if a Case Study enter NA)
NA

Conclusion
Myoepithelial carcinomas of the salivary glands are rare neoplasms, and they have various morphologies. The plasmacytoid variant can pose as a diagnostic pitfall. In fine needle aspiration biopsies, carcinoma cells with plasmacytoid morphology can be confused with a plasma cell neoplasm. CD138 is nonspecific marker, and it can be expressed in plasma cells as well as carcinomas. To further complicate matters, pancytokeratin and SOX10 can aberrantly expressed in some plasma cell myelomas. Flow cytometry studies is a valuable tool to define various cell types. However, it can be misleading if it is used as a sole source for interpreting plasmacytoid cells. Epithelial and myoepithelial neoplasms should be considered as differential diagnoses when CD138-positive plasmacytoid cells are encountered. Extended immunohistochemistry panel including multiple epithelial and myoepithelial markers play pivotal role in settling the diagnosis.
Mimics of carcinoma: pancytokeratin positive follicular dendritic cell sarcoma
Authors: Ricks E T , Hu Z 
Published: October 2021

Abstract

Introduction/Objective
Follicular dendritic cell sarcoma (FDC-S) is a malignant neoplasm of follicular dendritic cells that can present with variable morphology including epithelioid and spindle cell components. Given its morphological diversity, FDC-S can mimic neoplasms including poorly differentiated carcinomas. Here we present a rare case of FDC-S in which immunohistochemical staining for pancytokeratin was focally positive, potentially leading to an erroneous diagnosis if a full spectrum of immunohistochemical stains was not considered.

Methods/Case Report
A 34-year-old man presented with an enlarging left neck mass, night sweats and fatigue that began approximately three months ago. CT scan showed a 5.6 x 4.2 x 3.7 cm necrotic mass in the left upper lateral carotid space. Histologic examination of the incisional biopsy specimen demonstrated portions of soft tissue comprised of pleomorphic and plump spindle cells forming whorls with no discernable node architecture (Panels A-B). The tumor cells are large with oval nuclei, vesicular chromatin, distinct nucleoli, and a moderate amount of eosinophilic cytoplasm. Occasional binucleated and multinucleated tumor cells, rare tumor cells with nuclear pseudoinclusions and atypical mitotic figures, and focal necrosis are identified. Scattered small mature lymphocytes are present. By immunohistochemistry, tumor cells are positive for pancytokeratin (focal) (Panel C), CD21 (panel D), CD23 (panel E), D2-40 (panel F), CD35 and clusterin, and negative for CD3, CD20, CD30, CD45, ALK-1, EMA, PAX5, PDL1 and S100 stains. The overall morphology and immunostaining pattern are compatible with follicular dendritic cell (FDC) sarcoma.

Results (if a Case Study enter NA)
NA

Conclusion
FDC sarcoma rarely occurs as the solitary mass in the head and neck region. The unusual focal positivity for pancytokeratin highlights the importance of comprehensive immunohistochemistry stains to confirm the diagnosis of FDC sarcoma since this case is focally positive for pancytokeratin, mimicking other neoplasms such as poorly differentiated carcinomas
CD30+ Anaplastic Enteropathy-Associated T-cell Lymphoma Mimicking Crohn’s Disease
Authors: Harbert J L , Da Silva Lameira F , Beversdorf W , Bhalla R , Rinker E 
Published: October 2021

Abstract

Introduction/Objective
Enteropathy-associated T-cell lymphoma (EATL) is an aggressive peripheral T-cell lymphoma with a very poor prognosis. It is not uncommon for patients with EATL to present with intestinal obstruction or perforation, with the diagnosis made following surgical resection or autopsy. Although EATL is associated with celiac disease, this diagnosis may be made concomitant with EATL. The symptomology is often non-specific, particularly without a known history of celiac disease, and progression may be rapid.

Methods/Case Report
We describe the case of a 57 year old male who presented with several months of diarrhea, fatigue, night sweats, severe weight loss, and failure to thrive. Initial workup indicated positivity for Clostridium difficile and cryptosporidium, leading to antibiotic treatment. His symptoms persisted without significant improvement and a presumptive diagnosis of Crohn’s disease was made based on colonoscopy with biopsy and imaging that showed inflammatory changes with entero-enteric fistula formation. Further clinical decline necessitated exploratory laparotomy which revealed multiple enteric strictures, intra-loop abscesses, and necrotic ulceration necessitating segmental ileal resections. There was no associated lymphadenopathy.
Microscopically, there was a dense, polymorphic lymphoid population within the bowel wall with associated mucosal ulceration and abundant necrosis. Striking anaplastic cytomorphology was present, with a heavy intratumoral eosinophilic infiltrate. Neoplastic lymphocytes were CD3+, CD30+ T cells with a CD7+, CD5-, CD4-, CD8-, CD56-, ALK- immunophenotype. The adjacent intestinal mucosa showed features of celiac disease (villous atrophy, intraepithelial lymphocytosis).

Results (if a Case Study enter NA)
NA

Conclusion
The prominent anaplastic morphology in combination with strong, diffuse CD30 expression prompted consideration of other T cell lymphomas, including anaplastic large cell lymphoma and peripheral T cell lymphoma, NOS, within the differential. The presence of coexisting histologic changes of celiac disease, dense eosinophilic infiltrate, and absence of lymphadenopathy were clues to the correct diagnosis. Notably, the frequency of CD30 expression in EATL is variable but common among cases manifesting large cell morphology.
The Practicality of Viscoelastic Testing in Acute Hemorrhage to Guide Appropriate Transfusion Management
Authors: Weng K 
Published: October 2021

Abstract

Introduction/Objective
Acute hemorrhage often leads to multiple blood product transfusions and warrants efficient and dependable laboratory tests to elucidate the etiology for appropriate transfusion management. Conventional coagulation tests (aPPT, PT, INR, platelet count, fibrinogen) are inexpensive and have minimal labor requirements, but have slow turnaround times and may not accurately represent in vivo coagulopathies leading to inappropriate use of transfusion products, which in turn may lead to increased adverse events and mortality. Viscoelastic testing (VET) overcomes some of the disadvantages of conventional coagulation tests, but is not without its own limitations. This case study will summarize the advantages and disadvantages of VET by presenting two cases to highlight clinical situations in which VET is used and its impact on guiding management and clinical outcomes.

Methods/Case Report
We present 2 cases of acute hemorrhage to illustrate the importance of correct interpretation of VET in the appropriate clinical setting. Case 1 features a clinical situations in which TEG was misinterpreted due to lack of communication with the laboratory while Case 2 presents an acute hemorrhage situation in which Rapid TEG was used to guide transfusion strategy. While VET has many advantages in acute hemorrhage, clinicians must be aware of the disadvantages and the potential for misinterpretation of data.

Results (if a Case Study enter NA)
NA

Conclusion
VET is useful in acute hemorrhage if its functionality is understood and interpretation is correct. Appropriate use of VET may lead to effective transfusion management and improve clinical outcomes. However, it is imperative clinicians are aware of the limitations of VET when making clinical decisions. VET can only improve patient outcomes if it is used in the proper clinical situation and with proper interpretation of the data. Appropriate use of VET has the potential to improve transfusion management and ultimately lead to better patient outcomes.
Acquired Glanzmann Thrombasthenia in a Pediatric Patient with Alagille Syndrome
Authors: Cox C A , Hastings H 
Published: October 2021

Abstract

Introduction/Objective
Acquired Glanzmann Thrombasthenia is a rare bleeding disorder that is characterized by inhibition of glycoprotein IIb/IIIa signaling, usually by an autoantibody, leading to an interference in platelet aggregation. Clinically, this disorder presents with spontaneous mucocutaneous bleeding in the setting of a normal platelet count. Acquired Glanzmann Thrombasthenia has been associated with primary immune thrombocytopenic purpura (ITP), several types of hematologic and solid malignancies, solid organ transplants, and other autoimmune disorders.

Methods/Case Report
A 4-year-old female patient with a history of Alagille Syndrome requiring liver transplant at age 3 was admitted to the hospital after presenting to the emergency department with complaints of bruising, nosebleeds, and a petechial rash. The patient was found to have a platelet count of 11 K/mm3 and was diagnosed with ITP. The patient received a single dose of IVIG at 1g/kg with subsequent resolution of bleeding and a recovering platelet count of 27 K/mm3 12 hours after administration. However, two months later, the patient presented again with worsening bruising, multiple nosebleeds per day, and worsening petechiae. Lab studies revealed the patient’s platelet count was within normal limits. A platelet antibody screen was positive with a subsequent Platelet Antibody Bead Array revealing anti-Gp IIb/IIIa HPA-1 and HPA-3 positivity.

Results (if a Case Study enter NA)
N/A

Conclusion
Acquired Glanzmann Thrombasthenia is a rare bleeding disorder that is the result of interference with platelet aggregation. Antibodies that may be associated with any of several underlying conditions lead to impaired platelet function and subsequent mucocutaneous bleeding. The present case represents an occurrence of Acquired Glanzmann Thrombasthenia in a patient with multiple risk factors for development of the disorder.
Machine learning based decipherment of Cell Population Data: a promising hospital front-door screening tool for COVID-19
Authors: Haider R , Shamsi T S , Khan N A 
Published: October 2021

Abstract

Introduction/Objective
Key challenges against early diagnosis of COVID-19 are its symptoms sharing nature and prolong SARS-CoV-2 PCR turnaround time. Hither machine learning (ML) tools experienced by routinely generated clinical data; potentially grant early prediction.

Methods/Case Report
Routine and earlier diagnostic data along demographic information were extracted for total of 21,672 subsequent presentations. Along conventional statistics, multilayer perceptron (MLP) and radial basis function (RBF) were applied to predict COVID-19 from pre-pandemic control. Three feature sets were prepared, and performance evaluated through stratified 10-fold cross validation. With differing predominance of COVID-19, multiple test sets were created and predictive efficiency was evaluated to simulate real-fashion performance against fluctuating course of pandemic. Models validation was also inducted in prospective manner on independent dataset, equating framework forecasting to conclusions from PCR.

Results (if a Case Study enter NA)
RBF model attained superior cross entropy error 20.761(7.883) and 20.782(3.991) for Q-Flags and Routine Items respectively while MLP outperformed for cell population data (CPD) parameters with value of 6.968(1.259) for ‘training(testing)’. Our CPD driven MLP framework in challenge of lower (&lt;5%) COVID-19 predominance affords greater negative predictive values (NPV &gt;99%). Higher accuracy (%correct 92.5) was offered during prospective validation using independent dataset. Sensitivity analysis advances illusive accuracy (%correct 94.1) and NPV (96.9%). LY-WZ, Blasts/Abn Lympho?, ‘HGB Interf?’, and ‘RBC Agglutination?’ are among novel enlightening study attributes.

Conclusion
CPD driven ML tools offer efficient screening of COVID-19 patients at presentation to hospital to backing early expulsion and directing patients’ flow-from amid the initial presentation to hospital.
Unique case of 19p13 syndrome with storage pool disease
Authors: Liaquat S , Riley R , Massey G , Gunning W T 
Published: October 2021

Abstract

Introduction/Objective
Microdeletion of a region of the short arm of chromosome 19 results in a very rare syndrome called 19p13.3 deletion syndrome, which manifest itself in developmental delay as well as structural abnormalities such as facial dysmorphism and macrocephaly.

Methods/Case Report
We present a case of 14-month-old patient, born at term and was large for her gestational age. She had dysmorphic facial features including posterior cleft palate for which, she required placement of G-tube. Post-delivery, she experienced respiratory distress as well as hypoglycemic episodes. Over the period of time, her mother also noticed occasional bleeding through her gums with teething. Genetic workup was performed, which revealed 2.4 Mb of microdeletion at chromosome 19 region p13.3, including deletion of PIAS4, MAP2K2, GNA11, TBXA2R, RAX2 genes. TBXA2R mutation is associated with bleeding disorder due to a defect in platelet aggregation. The mutation in TBXA2R can lead to platelet type 13 bleeding disorder. For this purpose, a platelet aggregation study was performed to evaluate platelet function disorders. However, the result of the platelet aggregation study was inconclusive as it showed decrease responses to all agonists including arachidonic acid, epinephrine, ADP, collagen and ristocetin. Further work-up by electron microscopy (EM) of platelets (PL) revealed a significant decrease of delta granules (DG) (0.89 DG/PL, normal 4-6 DG/PL), consistent with delta granule storage pool deficiency (δ-SPD). Other abnormalities observed by EM included occasional gray platelets, platelets with immature and/or decreased numbers of α-granules, and rare giant α-granules.

Results (if a Case Study enter NA)
NA

Conclusion
To the best of our knowledge, no other case of 19p13.3 microdeletion syndrome with δ-SPD and associated abnormalities in α-granules has previously been described in the literature. Although it is unclear if there is any relationship between δ-SPD and 19p13.3 deletion syndrome, further investigation is warranted.
Anemia prevalence in mothers with children under five years of age in Dodi Papase, Volta region of Ghana and preventive practices
Authors: Sanyaolu A , Dzando G , Okorie C , Jaferi U , Marinkovic A , Prakash S , Patidar R , Desai P , Younis K 
Published: October 2021

Abstract

Introduction/Objective
Sociodemographic factors influence the prevalence of anemia in endemic areas. The purpose of this study is to establish the prevalence and causes of anemia and to determine anemia preventive practices carried out by mothers with children under five years of age in the Kadjebi District of the Volta region of Ghana.

Methods/Case Report
This homogenous sampling study involved women of reproductive age with children less than five years of age from Saint Mary Theresa Hospital. Interview guides were administered, and the data collected were analyzed using the Statistical Package for Social Sciences (SPSS) version 21.

Results (if a Case Study enter NA)
A total of 150 participants were studied. 52.7% of the mothers indicated that their children had never been diagnosed with anemia; however, Hb levels recorded for these children showed that 73.3% were anemic, even though 93.8% of the mothers had been given iron supplements during their pregnancy. Furthermore, anemia prevention practices comprised of whether the child had been given any anti-malaria prophylaxis (98.4% denied / 1.6% confirmed) if the child had been dewormed in the last three months (89.9% denied / 10.1% confirmed), whether the child was given iron supplements in the last three months (59.7% denied / 40.3% confirmed), if the child had been given vitamin supplements in the last three months (24.0% denied / 76.0% confirmed).

Conclusion
Nutritional deficiencies, worm infestation, and malaria were identified as the major causes of anemia among the children. Mothers were educated about the possible causes and prevention methods of anemia.
Using digital pathology in competency based case scenarios as an efficient pathology learning platform
Authors: Saxena R , Ejutse T , Salzle M , Carnevale K 
Published: October 2021

Abstract

Introduction/Objective
Case-based learning has been utilized for implementation in learning pathological and clinical features of diseases in an integrated, facile manner for some time now. We applied certain novel technological advances to improve case-based learning - short clinical vignettes describing all pertinent pathologic features with the help of digital pathology images - including both negative and positive findings. The clinical cases combined with digital pathological and radiological images and labs were uploaded on Moodle platform for student access, to be followed later by an online interactive discussion via Zoom.

Methods/Case Report
Clinical cases meticulously woven with digital pathological and radiological images and labs were centralized on 3 fundamental competencies – disease processes, systemic pathology and diagnostic & therapeutic pathology. This was followed by corresponding Q-A session using the “breakout room” feature of Zoom to form a differential diagnosis, reach a final diagnosis and discuss appropriate management options. Students exited the breakout rooms after individual groupwork to return to the main Zoom session for final discussion. Novel web- based tools, social media assistance, and other online resources were utilized to enhance the online learning experience.

Results (if a Case Study enter NA)
Student feedback confirmed that our online instructional methodology was incredibly stimulatory, particularly in topics that were difficult to grasp using traditional pedagogical methods. Summative and formative assessments indicated performance gain, enhanced motivation, improved critical thinking and evolved ability to solve clinical cases. Drawbacks included irregular student participation, lack of face-to-face interaction, network issues and time differences.

Conclusion
Our pathology pedagogy provides flexibility and self-directed learning skills, while simultaneously covering the geographical limitations. This not only helps students in developing critical clinical reasoning, but also enables them to acquire life-long learning and problem-solving skills. The technique proved to be a powerful educational tool that facilitated an interactive learning experience beyond the classroom setting, with integration of multidisciplinary aspects in learning Pathology. Additionally, the communication competency was also covered during case discussion among students, promoting literary, verbal and team-working capabilities.
DOG1 expression is in common human tumors: A tissue microarray study on more than 15,000 tissue samples
Authors: Jansen K , Steurer S 
Published: October 2021

Abstract

Introduction/Objective
Introduction: DOG1 (Discovered on GIST1) is a voltage-gated calcium-activated chloride and bicarbonate channel that is highly expressed in interstitial cells of Cajal and in gastrointestinal stromal tumors (GIST) derived from Cajal cells.

Methods/Case Report
Methods: To systematically determine in what tumor entities and normal tissue types DOG1 may be further expressed, a tissue microarray (TMA) containing 15,965 samples from 121 different tumor types and subtypes as well as 608 samples of 76 different normal tissue types was analyzed by immunohistochemistry.

Results (if a Case Study enter NA)
Results: DOG1 immunostaining was found in 67 tumor types including GIST (95.7%), esophageal squamous cell carcinoma (31.9%), pancreatic ductal adenocarcinoma (33.6%), adenocarcinoma of the Papilla Vateri (20%), squamous cell carcinoma of the vulva (15.8%) and the oral cavity (15.3%), mucinous ovarian cancer (15.3%), esophageal adenocarcinoma (12.5%), endometrioid endometrial cancer (12.1%), neuroendocrine carcinoma of the colon (11.1%) and diffuse gastric adenocarcinoma (11%). Low level-DOG1 immunostaining was seen in 17 additional tumor entities. DOG1 expression was unrelated to histopathological parameters of tumor aggressiveness and/or patient prognosis in cancers of the breast (n=1,002), urinary bladder (975), ovary (469), endometrium (173), stomach (233), and thyroid gland (512).

Conclusion
High DOG1 expression was linked to estrogen receptor expression in breast cancer (p&lt;0.0001) and absence of HPV infection in squamous cell carcinomas (p=0.0008). In conclusion, our data identify several tumor entities that can show DOG1 expression levels at similar levels as in GIST. Although DOG1 is tightly linked to a diagnosis of GIST in spindle cell tumors, the differential diagnosis is much broader in DOG1 positive epithelioid neoplasms.