bluuebunny/crossref_metadata_2025_split huggingface dataset:
Two Cases of Cutaneous Sarcomatoid Squamous Cell Carcinoma Resembling Cutaneous Giant Cell Tumor of Soft Tissue
Authors: McAlpine Sarah G. , Carrasquillo Osward Y. , Miedema Jayson , Googe Paul B. 
Published: June 2024

Abstract:
Cutaneous sarcomatoid squamous cell carcinoma is well-described with histology resembling pleomorphic undifferentiated sarcoma featuring collagenous or myxoid stroma with or without elements of keratinizing squamous carcinoma. This report presents 2 cases of dedifferentiated squamous cell carcinoma (SCC) composed of sheets of malignant mononuclear cells with malignant osteoclast–like multinucleated giant cells, extravasated blood, and hemosiderin resembling cutaneous giant cell tumor (cGCT). In the first case, an exophytic facial mass of a 96-year-old woman removed by shave showing extensive cGCT-like tumor but with microscopic elements of SCC in situ and positivity for cytokeratin 5/6 in the malignant spindle cells and SCC. The second case involved a 32-year-old man with a pedunculated penile mass removed by shave biopsy, displaying malignant cytology resembling cGCT, focal staining for cytokeratin AE1/AE3 and p63, and CD68 highlighting the osteoclast-like giant cells. Molecular analysis revealed CDKN2A, TP53, and TERT. Upon reexcision, case 2 showed focally invasive keratinizing SCC associated with differentiated penile intraepithelial neoplasia and lichen sclerosus. Skin specimens with an exophytic mass histologically resembling cGCT but with malignant cytology should be meticulously evaluated for elements of SCC. Molecular analysis, detecting mutations like H3F3 or HMGA2-NCOR2 fusion, can aid in distinguishing cutaneous sarcomatoid squamous cell carcinoma from GCT bone or GCT soft tissue.
Pseudolymphomatous Cutaneous Angiosarcoma Presenting With Persistent Firm Facial Edema in a Patient With Systemic Sclerosis
Authors: Méndez-Flores Silvia , Saeb-Lima Marcela , Fragoso-Loyo Hilda Esther 
Published: June 2024

Abstract:
Pseudolymphomatous cutaneous angiosarcoma (cAS) is a rare subtype characterized by a prominent lymphocytic infiltrate, posing diagnostic challenges due to its resemblance to lymphoid neoplastic processes. We present a novel case highlighting the clinical and histopathological features, notably its association with persistent firm facial edema in a patient with systemic sclerosis (SSc). A 47-year-old woman with a 21-year history of SSc presented with firm palpebral edema evolving to involve the entire face and cervical region over six months. Diagnostic imaging revealed inflammatory changes in orbital regions, supradiaphragmatic lymphadenopathies, and lytic lesions. Skin biopsy demonstrated a diffuse neoplasm with vascular channels and solid areas, accompanied by dense lymphocytic proliferation. Pseudolymphomatous cutaneous angiosarcoma, a rare malignant neoplasm, exhibits variable clinical presentations and rapid progression. Histologically, it manifests as irregularly shaped vascular channels lined by prominent endothelial cells. Immunohistochemistry, particularly markers such as v-ets erythroblastosis virus E26 oncogene homolog (avian) (ERG), aids in diagnosis. Notably, this case marks the first presentation of cAS with persistent facial edema in SSc, highlighting the association between SSc and cancer risk. This case underscores the diagnostic challenges posed by cAS and emphasizes the importance of early detection for optimal patient outcomes. Further understanding of its association with autoimmune disorders such as SSc is crucial for comprehensive management strategies.
Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months
Authors: Shaker Nada , Blankenship Heath , Masatkar Vaishali , Niu Shuo , Sangueza Omar P. 
Published: June 2024

Abstract:
Primary cutaneous gamma/delta T-cell lymphoma (PCGD-TCL) is a rare yet highly aggressive subtype of primary cutaneous lymphoma. Characterized by its challenging diagnosis and poor prognosis, PCGD-TCL presents unique clinical and histopathological features that distinguish it from other primary cutaneous lymphoma subtypes. Here, we report the case of a 75-year-old man who initially presented with multiple erythematous indurated plaques over his back and bilateral lower extremities. The initial biopsy suggested primary cutaneous T-cell lymphoma (PCTCL) with a CD30-negative phenotype. However, within a 2-month interval, the disease progressed rapidly, manifesting as extensive skin involvement across the chest and upper extremities. A repeat skin biopsy was performed, revealing dermal atypical lymphocytes without epidermotropism. Immunohistochemical analysis demonstrated positivity for CD3, CD5, and CD4, as well as T-cell receptor delta (TCR delta) expression, along with the loss of CD8 and CD30 expression. These findings were consistent with a diagnosis of PCGD-TCL. Despite therapeutic interventions, including systemic treatments, the patient's condition deteriorated rapidly, ultimately leading to his demise within a month of receiving the PCGD-TCL diagnosis. This case highlights the diagnostic complexities associated with PCGD-TCL, emphasizing the importance of careful histopathological examination and immunophenotypic characterization. Given its aggressive nature and propensity for rapid dissemination, early recognition of PCGD-TCL is paramount for initiating appropriate therapeutic interventions. However, effective treatment options for PCGD-TCL remain limited, and the disease typically carries an unfavorable prognosis. Further research is needed to elucidate the underlying molecular mechanisms driving the pathogenesis of PCGD-TCL, to identify novel therapeutic targets, and to improve patient outcomes. In addition, increased awareness among clinicians and pathologists regarding the clinical presentation and diagnostic criteria of PCGD-TCL is crucial for facilitating timely diagnosis and management of this challenging malignancy.
Superficial Atypical Lipomatous Tumor With Pleomorphic Features: Case Report and Discussion of the Literature
Authors: Adeuyan Oluwaseyi , Gordon Emily R. , Lapolla Brigit A. , Schreidah Celine M. , Jobanputra Vaidehi , Gru Alejandro A. , Geskin Larisa J. 
Published: June 2024

Abstract:
Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient's ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.
Schwannian and Perineuriomatous Differentiation in a Series of Giant Congenital Melanocytic Nevi
Authors: Kletskaya Irina , Belousova Irena , Makarova Olga , Narbutov Anton , Oganesyan Raisa , Donati Michele , Říčař Jan , Salgado Claudia M. , Reyes-Múgica Miguel , Kazakov Dmitry V. 
Published: June 2024

Abstract:
Close relationship between melanocytes and neural cells is accepted to reflect their common derivation from the neural crest and tumors combining both elements. We present a series of 10 patients with giant congenital melanocytic nevi (CMN) in which a secondary proliferation (11 lesions) with schwannian and/or perineuriomatous differentiation developed in the course of the disease. The age of the patients (4 male and 6 female) at the time of surgery and histological assessment varied from 3 months to 57 years. Histopathologically, the following subgroups were delineated: (1) nodular/tumoriform “neurotization” in CMN, (2) diffuse neurofibroma–like proliferation within CMN, (3) plexiform neurofibroma–like proliferation within CMN, and (4) diffuse perineuriomatous (hybrid schwannomatous–perineuriomatous) differentiation in CMN. We review the pertinent literature, including the role of recently identified Schwann cell precursors which are believed to represent the nerve-associated state of neural crest-like cells that persists into later developmental stages.
Primary Cutaneous Cryptococcosis in an Elderly Patient: A Case Report and Review of Literature
Authors: Zhang Yuyan , Jiang Faxing , Zhang Siping , Liu Jinli 
Published: June 2024

Abstract:
This article reports an elderly male patient with nodules and ulcers on the face and behind the left ear after trauma. Primary cutaneous cryptococcosis was confirmed using pathological biopsy, special staining, tissue culture, and fungal sequencing. The patient received a therapeutic intervention involving the administration of the antifungal agent itraconazole. Substantial amelioration of cutaneous manifestations was observed after a 3-month course of treatment. After an elapsed interval, the patient was diagnosed with esophageal tumor. Moreover, the literature on 33 patients with primary cutaneous cryptococcosis published in the past 10 years was also reviewed.
Coma Blister After Trazodone Misuse: A Case Report
Authors: Yang Seanna , Chen Vivien , Bitar Carole 
Published: June 2024

Abstract:
Coma blisters are epidermal and subepidermal bullous lesions that can arise throughout the body after a prolonged impairment of consciousness. Coma blister-like lesions have been well-documented in adults after barbiturate-induced intoxication. More recently, other drugs and substances have been associated with the development of these bullae, which has broadened the scope of medications that put patients at risk of developing coma blister-like lesions. We present a unique case of a noncomatose patient who developed coma blister-like lesions after trazodone misuse. This case illustrates the need to further investigate the mechanism behind drug-induced coma bullae-like lesions so that clinicians can better identify and discontinue drugs that precipitate such lesions.
Three-dimensional Hemifacial Microsomia Classification with New Subtypes Based on the Pruzansky and Kaban Classification
Authors: Shibazaki-Yorozuya Reiko , Yamada Akira , Sasaki Ryo , Watanabe Yorikatsu 
Published: June 2024

Background:
Various classifications of hemifacial microsomia (HFM) have been described previously. Although some of these classifications are used widely, others use external outlines of reference organs, even in three-dimensional (3D) images. The purpose of this study was to investigate the 3D characteristics of the mandibular condyle in HFM and to update the Pruzansky and Kaban classification as a 3D classification.

Methods:
Fifty-three patients with HFM were classified according to the Pruzansky and Kaban classification (type I, IIA, IIB, and III) using computed tomographic scan images. 3D images of the mandible were isolated, and the 3D characteristics were observed; furthermore, the angle of inclination of the mandibular condyle was measured in 3D.

Results:
Subtypes of retroflexed mandibular condyle in 3D were observed in the Pruzansky and Kaban classification type IIA and IIB, termed as type IIAβ (33.3% in type IIA) and type IIBβ (100% in type IIB). Although some differences were observed in the inclination of the mandibular condyle between the control and the affected sides in type I and IIAα, multiple differences were observed in type IIAβ and IIBβ.

Conclusions:
To the best of our knowledge, this is the first report that identified the retroflexed mandibular condyle as subtypes type IIAβ and IIBβ. Notably, this could not be identified in the two-dimensional images (lateral cephalogram) yet. We proposed to update the Pruzansky and Kaban classification as a 3D classification with a new 3D subtype. The angle of the retroflexed mandibular condyle may predict mandibular growth in HFM.
Incidental Diagnosis of Four Lid Orbital Lymphoma during a Blepharoplasty
Authors: Anderson Daniella L. , Gruizinga Brandt A. , Dean Hannah C. , Hassan Adam S. 
Published: June 2024

Summary:
Chronic lymphocytic leukemia is characterized by the accumulation of mature CD5-positive B-cells in the lymphoid organs.1 Extranodal involvement occurs in up to 10% of cases and can arise in various tissues, including the orbit. Less than 400 cases of orbital lymphoma are diagnosed per year in the United States, typically manifesting as a form of B-cell non-Hodgkin lymphoma, with extranodal marginal zone B-cell lymphoma being the most common subtype. Orbital lymphoma typically presents with proptosis and a palpable mass; however, patients may also have a relatively benign examination. Here, we present a 76-year-old man with symmetric dermatochalasis and marked fat prolapse of all four lids, who was incidentally diagnosed with secondary orbital lymphoma in all four eyelids during a cosmetic four lid blepharoplasty. His history was significant for RAI Stage 0 chronic lymphocytic leukemia diagnosed 15 years before consultation. Orbital lymphoma presenting as orbital fat prolapse has only been reported a few times in the literature. To our knowledge, this is the first case of secondary orbital lymphoma in all four eyelids found incidentally during an aesthetic four lid blepharoplasty.
A Shaped Pectoralis Major Muscle Flap under Indocyanine Green Fluorescence Angiography for Sternal Wound Infection
Authors: Sato Hideyoshi , Nakai Yosuke , Suda Hisao , Tsunekawa Yukiyo , Koyama Chisato , Fujioka Urara , Toriyama Kazuhiro 
Published: June 2024

Summary:
The treatment of a sternal wound infection is challenging because it requires radical debridement and reconstruction with a well-vascularized flap. The defects after debridement are three-dimensionally complex, especially if synthetic grafts are involved. Although the pectoralis major muscle (PMM) flap is useful for reconstruction, it is difficult to fill up the complex dead space surrounding the vascular prosthesis when using a conventional PMM flap. Herein, we describe a new technique of splitting and shaping the PMM flap to fit the complex defect. Intraoperative indocyanine green fluorescence angiography was used to assess dynamic blood flow of the PMM supplied by internal mammary artery perforators. This technique allows the PMM flap to be split and shaped to securely fit the dead space, which may improve the healing rate.
Enhanced Recovery after Surgery Protocol Decreases Length of Stay and Postoperative Narcotic Use in Tissue Expander-based Breast Reconstruction
Authors: Taylor Jeremiah M. , Moman Precious D. , Chevalier Jose M. , Tseng Charles Y. , Festekjian Jaco H. , Delong Michael R. 
Published: June 2024

Background:
Enhanced recovery after surgery (ERAS) protocols have demonstrated success in reducing hospital stay and opioid consumption, but are less well studied in patients undergoing tissue expander-based breast reconstruction (TEBR). This study evaluates the effectiveness of an ERAS postoperative protocol for TEBR at a high-volume center.

Methods:
All patients undergoing immediate tissue expander reconstruction after the introduction of ERAS were prospectively included from April 2019 to June 2023. An equivalent number of similar patients were retrospectively reviewed before this date as the non-ERAS control. Data included demographics, operative details, postoperative length of stay, inpatient and discharge narcotic quantities, inpatient pain assessments, postoperative radiation, and complications within 90 days.

Results:
There were 201 patients in each cohort with statistically similar demographics. Patients in the ERAS cohort were more likely to undergo prepectoral reconstruction (83.1% versus 4.5%, P < 0.001), be discharged by day 1 (96.5% versus 70.2%, P < 0.001) and consume lower inpatient milligram morphine equivalent (MME) median (79.8 versus 151.8, P < 0.001). Seroma rates (17.4% versus 3.5%, P < 0.001) and hematoma incidence (4.5% versus 0%, P = 0.004) were higher in the ERAS cohort. Adjusting for implant location, ERAS was associated with a 60.7 MME reduction (β=-60.7, P < 0.001) and a shorter inpatient duration by 0.4 days (β =-0.4, P < 0.001). Additionally, prepectoral reconstruction significantly decreased MME (β=-30.9, P = 0.015) and was the sole predictor of seroma development (odds ratio = 5.2, P = 0.009).

Conclusions:
ERAS protocols significantly reduce opioid use and hospital stay after TEBR.
Superficial Thoracic Artery Perforator Flap for Volume Replacement Oncoplastic Breast-conserving Surgery
Authors: Hayakawa Masashi , Nakao Junichi , Tadokoro Yukiko , Nakamura Shogo , Suzuki Shinya , Matsubara Ken , Mori Hiroaki , Araki Jun , Nishimura Seiichiro , Yasunaga Yoshichika 
Published: June 2024

Summary:
Lateral chest wall perforator flaps, such as the lateral intercostal artery perforator flap, lateral thoracic artery perforator flap, and thoracodorsal artery perforator flap, have been used for volume replacement oncoplastic breast-conserving surgery (VR-OPBCS) in the lateral and central breast. However, there are cases in which these perforators are missing or too thin, making it difficult to raise a flap for partial breast reconstruction. A 58-year-old woman underwent VR-OPBCS for breast cancer in the lower quadrant of the right breast. Preoperative imaging studies did not identify lateral thoracic artery perforator or thoracodorsal artery perforator but identified a well-developed superficial thoracic artery perforator (STAP). A flap based on the STAP was dissected, and partial breast reconstruction was performed. The flap survived with no complications. No deformity of the lower breast or displacement of the nipple–areola complex was observed 8 months after the completion of postoperative radiotherapy. The STAP flap can be used as an alternative to VR-OPBCS when other lateral chest wall perforator flaps are unavailable.
Measuring Access to Mental Health Services Among Primary Care Patients
Authors: Hargraves J. Lee , Cosenza Carol , Cleary Paul D. 
Published: June 2024

Background:
The lifetime risk of mental health disorders is almost 50% and, in any year, about 25% of the population have a psychiatric disorder. Many of those people are cared for in primary care settings.

Research Objective:
Measure access to mental health services, such as getting counselling or prescription mental health medications, using new patient survey questions that can be added to Consumer Assessment of Healthcare Providers and Systems (CAHPS) surveys.

Study Design:
Surveys were conducted with a stratified probability sample of patients receiving primary care services in a single state in 2018–2019. Medicaid and privately insured patients were surveyed by mail or telephone, respectively.

Results:
Approximately 14% of sampled patients responded to a survey. More than 10% of privately insured respondents and about 20% of Medicaid respondents got or tried to get appointments for mental health care. About 15% of privately insured respondents and 11% of Medicaid respondents reported problems getting appointments with counselors. Only 8%–9% of respondents seeking mental health medicines reported problems getting appointments for prescriptions. A composite measure combining access to counselors and prescribers of mental health medicines evidenced adequate internal consistency reliability. Group level reliability estimates were low.

Conclusions:
Many respondents got or tried to get mental health services and a substantial number reported problems getting appointments or getting mental health prescriptions. The tested questions can be combined into an Access to Mental Health Care measure, which can be included in patient experience surveys for ambulatory care to monitor access to behavioral health care.
Current and future microbiome-based therapies in inflammatory bowel disease
Authors: Montrose Jonathan A. , Kurada Satya , Fischer Monika 
Published: June 2024

Purpose of review
The role of the microbiome and dysbiosis is increasingly recognized in the pathogenesis of inflammatory bowel disease (IBD). Intestinal microbiota transplant (IMT), previously termed fecal microbiota transplant has demonstrated efficacy in restoring a healthy microbiome and promoting gut health in recurrent Clostridioides difficile infection. Several randomized trials (RCTs) highlighted IMT's potential in treating ulcerative colitis, while smaller studies reported on its application in managing Crohn's disease and pouchitis.

Recent findings
This review delves into the current understanding of dysbiosis in IBD, highlighting the distinctions in the microbiota of patients with IBD compared to healthy controls. It explores the mechanisms by which IMT can restore a healthy microbiome and provides a focused analysis of recent RCTs using IMT for inducing and maintaining remission in IBD. Lastly, we discuss the current knowledge gaps that limit its widespread use.

Summary
The body of evidence supporting the use of IMT in IBD is growing. The lack of a standardized protocol impedes its application beyond clinical trials. Further research is needed to identify patient profile and disease phenotypes that benefit from IMT, to delineate key donor characteristics, optimize the delivery route, dosage, and frequency.
Pancreatic and cardiometabolic complications of severe hypertriglyceridaemia
Authors: Bashir Bilal , Ferdousi Maryam , Durrington Paul , Soran Handrean 
Published: June 2024

Purpose of review
This review endeavours to explore the aetiopathogenesis and impact of severe hypertriglyceridemia (SHTG) and chylomicronaemia on cardiovascular, and pancreatic complications and summarizes the novel pharmacological options for management.

Recent findings
SHTG, although rare, presents significant diagnostic and therapeutic challenges. Familial chylomicronaemia syndrome (FCS), is the rare monogenic form of SHTG, associated with increased acute pancreatitis (AP) risk, whereas relatively common multifactorial chylomicronaemia syndrome (MCS) leans more towards cardiovascular complications. Despite the introduction and validation of the FCS Score, FCS continues to be underdiagnosed and diagnosis is often delayed. Longitudinal data on disease progression remains scant. SHTG-induced AP remains a life-threatening concern, with conservative treatment as the cornerstone while blood purification techniques offer limited additional benefit. Conventional lipid-lowering medications exhibit minimal efficacy, underscoring the growing interest in novel therapeutic avenues, that is, antisense oligonucleotides (ASO) and short interfering RNA (siRNA) targeting apolipoprotein C3 (ApoC3) and angiopoietin-like protein 3 and/or 8 (ANGPTL3/8).

Summary
Despite advancements in understanding the genetic basis and pathogenesis of SHTG, diagnostic and therapeutic challenges persist. The rarity of FCS and the heterogenous phenotype of MCS underscore the need for the development of predictive models for complications and tailored personalized treatment strategies. The establishment of national and international registries is advocated to augment disease comprehension and identify high-risk individuals.