Urticarial mycosis fungoides: A distinctive presentation with blood involvement and a peculiar immunophenotype.

Mycosis fungoides (MF) has been widely reported to mimick a considerable number of different dermatoses, including scarring alopecia, bullous dermatoses or cysts, and comedones. In atypical presentations, histopathology is essential for the diagnosis. We present two cases of MF with clinical urticarial lesions and a striking blood involvement that responded to mogamulizumab treatment. Histopathologically, both cases had classic MF features and shared a peculiar immunophenotype, with positivity for CD25 and FOXP3. Differential diagnoses included urticarial lymphomatoid drug reactions and other lymphomas, like T-cell prolymphocytic leukemia, atypical Sézary syndrome, or adult T-cell lymphocytic leukemia. A low suspicion threshold is necessary for the diagnosis of atypical presentations of MF.

Perineural Infiltration: A Comprehensive Review of Diagnostic, Prognostic, and Therapeutic Implications.

ABSTRACT: Perineural infiltration refers to a neoplastic cell involvement in, around, and through the nerves. It is considered as one of the neoplastic dissemination pathways. Thus, its identification is crucial to establish the prognosis of some malignant skin neoplasms, such as squamous cell carcinoma, and explains the locally aggressive behavior of cutaneous neoplasms, such as microcystic adnexal carcinoma. We have conducted a review of malignant and benign skin tumors in which perineural infiltration has been described, and we also discuss some histopathological findings that may simulate perineural infiltration.

A shiny app for modeling the lifetime in primary breast cancer patients through phase-type distributions.

Phase-type distributions (PHDs), which are defined as the distribution of the lifetime up to the absorption in an absorbent Markov chain, are an appropriate candidate to model the lifetime of any system, since any non-negative probability distribution can be approximated by a PHD with sufficient precision. Despite PHD potential, friendly statistical programs do not have a module implemented in their interfaces to handle PHD. Thus, researchers must consider others statistical software such as R, Matlab or Python that work with the compilation of code chunks and functions. This fact might be an important handicap for those researchers who do not have sufficient knowledge in programming environments. In this paper, a new interactive web application developed with shiny is introduced in order to adjust PHD to an experimental dataset. This open access app does not require any kind of knowledge about programming or major mathematical concepts. Users can easily compare the graphic fit of several PHDs while estimating their parameters and assess the goodness of fit with just several clicks. All these functionalities are exhibited by means of a numerical simulation and modeling the time to live since the diagnostic in primary breast cancer patients.

"Delaying Rot Emergence in Persian Lime (Citrus×latifolia) Through Antifungal Hybrid Films Containing Litsea glaucescens Essential Oil".

Herein we describe the in situ inhibitory activity of three hybrid films (FL1, FL2, and FL3) against two wild strains of Colletotrichum gloeosporioides and Penicillium digitatum as causal agents of rot in Persian limes. The films FL2 and FL3 contained 1.0 and 1.3 % weight/volume Litsea glaucescens essential oil (LgEO) and significantly (p<0.05) delayed rot emergence in Persian limes caused by both pathogens up to 10 days. The physicochemical properties of LgEO and hybrid films were obtained, whereas detailed HPLC profiling revealed that fruit covered with these films significantly (p<0.01) preserved reducing sugars (sucrose, fructose, and glucose), organic acids (citric acid, ascorbic acid, malic acid, and oxalic acid), and flavonoids with nutraceutical activity (hesperidin, eriocitrin, naruritin, neohesperidin, diosmin, vitexin, rutin, and quercetin). This evidence sustains that the composites generated in this investigation improve the shelf life of Persian limes and conserve their nutraceutical content.

Influence of parallel pins on the angle deviation for placement of dental implants: an in vitro study.

The aim of the present study was to analyze and compare the angle deviation of two, four and six adjacent dental implants placed with and without straight parallel pins. MATERIALS AND METHODS: Two hundred and forty (240) dental implants were selected and randomly allocated into the following study groups: Two dental implants placed with straight parallel pins (Ref.: 144-100, BioHorizons, Birmingham, AL, USA) (n = 10) (2PP); Two dental implants placed without parallel pins (n = 10) (2withoutPP); Four dental implants placed with straight parallel pins hT(n = 10) (4PP); Four dental implants placed without parallel pins (n = 10) (4withoutPP); Six dental implants placed with straight parallel pins (n = 10) (6PP) and Six dental implants placed without parallel pins (n = 10) (6withoutPP). The dental implants randomly assigned to groups 2PP and 2withoutPP were placed into standardized polyurethane models of partially edentulous upper jaws in tooth positions 2.4 and 2.6, the dental implants randomly assigned to groups 4PP and 4withoutPP were placed into standardized polyurethane models of fully edentulous upper jaws in tooth positions 1.6, 1.4, 2.4 and 2.6, and the dental implants randomly assigned to groups 6PP and 6withoutPP were placed into standardized polyurethane models of fully edentulous upper jaws in tooth positions 1.6, 1.4, 1.2, 2.2, 2.4 and 2.6. Afterwards, postoperative CBCT scans and digital impressions were aligned in a 3D implant-planning software to compare the angle deviation (°) of two, four and six adjacent dental implants placed with and without straight parallel pins using the General Linear Model statistical analysis. RESULTS: Statistically significant differences were found between the angle deviation of 2 dental implants placed with straight parallel pins (p < 0.0001) and between the angle deviation of 4 dental implants placed with straight parallel pins (p = 0.0024); however, no statistically significant differences were found in the angle deviation of 6 dental implants placed with straight parallel pins (p = 0.9967). CONCLUSION: The use of a straight parallelization pin results in lower angle deviation between two and four adjacent dental implants; however, it is not effective for a larger number of dental implants.

LT and SOX9 expression are associated with gene sets that distinguish Merkel cell polyomavirus (MCPyV)-positive and MCPyV-negative Merkel cell carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive malignant neuroendocrine tumour. There are two subsets of MCC, one related to Merkel cell polyomavirus (MCPyV) and the other to ultraviolet radiation (UVR). MCPyV-positive and MCPyV-negative MCCs have been considered to be different tumours, as the former harbour few DNA mutations and are not related to UVR, and the latter usually arise in sun-exposed areas and may be found in conjunction with other keratinocytic tumours, mostly squamous cell carcinomas. Two viral oncoproteins, large T antigen (LT; coded by MCPyV_gp3) and small T antigen (sT; coded by MCPyV_gp4), promote different carcinogenic pathways. OBJECTIVES: To determine which genes are differentially expressed in MCPyV-positive and MCPyV-negative MCC; to describe the mutational burden and the most frequently mutated genes in both MCC subtypes; and to identify the clinical and molecular factors that may be related to patient survival. METHODS: Ninety-two patients with a diagnosis of MCC were identified from the medical databases of participating centres. To study gene expression, a customized panel of 172 genes was developed. Gene expression profiling was performed with nCounter technology. For mutational studies, a customized panel of 26 genes was designed. Somatic single nucleotide variants (SNVs) were identified following the GATK Best Practices workflow for somatic mutations. RESULTS: The expression of LT enabled the series to be divided into two groups (LT positive, n = 55; LT negative, n = 37). Genes differentially expressed in LT-negative patients were related to epithelial differentiation, especially SOX9, or proliferation and the cell cycle (MYC, CDK6), among others. Congruently, LT displayed lower expression in SOX9-positive patients, and differentially expressed genes in SOX9-positive patients were related to epithelial/squamous differentiation. In LT-positive patients, the mean SNV frequency was 4.3; in LT-negative patients it was 10 (P = 0.03). On multivariate survival analysis, the expression of SNAI1 [hazard ratio (HR) 1.046, 95% confidence interval (CI) 1.007-1.086; P = 0.02] and CDK6 (HR 1.049, 95% CI 1.020-1.080; P = 0.001) were identified as risk factors. CONCLUSIONS: Tumours with weak LT expression tend to co-express genes related to squamous differentiation and the cell cycle, and to have a higher mutational burden. These findings are congruent with those of earlier studies.

Merkel cell carcinoma (MCC) is an aggressive form of skin tumour. There are two subtypes of MCC: one of them is related to a virus called Merkel cell polyomavirus (MCPyV); the other one is related to persistent exposure to sunlight. The aim of this research was to find differences between these subtypes in their molecular behaviour (the genes that are expressed and the mutations that may be found). To do this, we carried out two studies, one to investigate gene expression (the process cells use to convert the instructions in our DNA into a functional product such as a protein) and one to look at gene mutations (changes in the DNA sequence). We found that the tumours that were not related to MCPyV expressed genes related to epithelial differentiation (the process by which unspecialized cells gain features characteristics of epithelial cells, which, among other things, make up the outer surface of the body), which means that the origin of both MCC subtypes may be different. We also found that MCPyV-related tumours had fewer mutations. Our findings are important because they help us to understand the biology of the MCC subtypes and could help with the development of new treatments for people diagnosed with skin tumours.

Indicators to evaluate quality of care in head and neck cancer in Spain.

PURPOSE: This study aimed to develop a set of criteria and indicators to evaluate the quality of care of patients with head and neck cancer (HNC). METHODS: A systematic literature review was conducted to identify valuable criteria/indicators for the assessment of the quality of care in HNC. With the aid of a technical group, a scientific committee of oncologists specialised in HNC used selected criteria to propose indicators that were evaluated with a two-round Delphi method. Indicators on which consensus was achieved were then prioritised by the scientific committee to develop a final set of indicators. RESULTS: We proposed a list of 50 indicators used in the literature or developed by us to be evaluated with a Delphi method. There was consensus on the appropriateness of 47 indicators in the first round; the remaining 3 achieved consensus in the second round. The 50 indicators were scored to prioritise them, leading to a final selection of 29 indicators related to structure (3), process (22), or outcome (4) and covering diagnosis, treatment, follow-up, and health outcomes in patients with HNC. Easy-to-use index cards were developed for each indicator, with their criterion, definition, formula for use in real-world clinical practice, rationale, and acceptable level of attainment. CONCLUSIONS: We have developed a set of 29 evidence-based and expert-supported indicators for evaluating the quality of care in HNC, covering diagnosis, treatment, follow-up, and health outcomes.

Management of periocular keratinocyte carcinomas with Mohs micrographic surgery and predictors of complex reconstruction: a retrospective study.

BACKGROUND: Skin cancer is the most frequent cancer worldwide and the most frequent periocular tumor. Keratinocyte Carcinomas (KC) located in periorificial areas, such as periocular tumors, are considered high-risk tumors. Mohs Micrographic Surgery (MMS) is considered the first line for the treatment of high-risk KC, providing a lower recurrence rate than conventional wide excision. OBJECTIVE: To describe the clinical-pathological features of periocular KC treated with MMS in a tertiary university center in Chile. METHODS: A single-center, retrospective study of patients with KC located on the periocular area, that underwent MMS between 2017‒2022. MMS details were recorded. RESULTS: One hundred thirteen patients with periocular carcinomas were included. The mean age was 59 ± 13 years; 52% were women. The most frequent location was the medial canthus (53%), followed by the lower eyelid (30.1%). The most frequent BCC histology was the nodular variant (59.3%). Regarding MMS, the average number of stages was 1.5 ± 0.7, and 54% of the cases required only 1 stage to achieve clear margins. To date, no recurrence has been reported. Tumors larger than 8.5 mm in largest diameter or 43.5 mm2 were more likely to require complex reconstruction. STUDY LIMITATIONS: Retrospective design and a relatively low number of patients in the SCC group. Possible selection bias, as larger or more complex cases, may have been referred to oculoplastic surgeons directly. CONCLUSION: The present study confirms the role of MMS for the treatment of periocular KCs. Periocular KCs larger than 8.5 mm might require complex reconstruction. These results can be used to counsel patients during pre-surgical visits.

Five-Fraction Proton Therapy for the Treatment of Skull Base Chordomas and Chondrosarcomas: Early Results of a Prospective Series and Description of a Clinical Trial.

(1) Background: Our purpose is to describe the design of a phase II clinical trial on 5-fraction proton therapy for chordomas and chondrosarcomas of the skull base and to present early results in terms of local control and clinical tolerance of the first prospective series. (2) Methods: A dose of 37.5 GyRBE in five fractions was proposed for chordomas and 35 GyRBE in five fractions for chondrosarcomas. The established inclusion criteria are age ≥ 18 years, Karnofsky Performance Status ≥ 70%, clinical target volume up to 50 cc, and compliance with dose restrictions to the critical organs. Pencil beam scanning was used for treatment planning, employing four to six beams. (3) Results: A total of 11 patients (6 chordomas and 5 chondrosarcomas) were included. The median follow-up was 12 months (9-15 months) with 100% local control. Acute grade I-II headache (64%), grade I asthenia and alopecia (45%), grade I nausea (27%), and grade I dysphagia (18%) were described. Late toxicity was present in two patients with grade 3 temporal lobe necrosis. (4) Conclusions: Hypofractionated proton therapy is showing encouraging preliminary results. However, to fully assess the efficacy of this therapeutic approach, future trials with adequate sample sizes and extended follow-ups are necessary.

Expanding access to microneurosurgery in low-resource settings: Feasibility of a low-cost exoscope in transforaminal lumbar interbody fusion.

Objectives: Less than a quarter of the world population has access to microneurosurgical care within a range of 2 h. We introduce a simplified exoscopic visualization system for low-resource settings. Materials and Methods: We purchased a 48 megapixels microscope camera with a c-mount lens and a ring light at a total cost of US$ 125. Sixteen patients with lumbar degenerative disk disease were divided into an exoscope group and a microscope group. In each group, we performed four open and four minimally invasive transforaminal lumbar interbody fusions (TLIF). We conducted a questionnaire-based assessment of the user experience. Results: The exoscope achieved similar outcomes with comparable blood loss and operating time as the microscope. It provided similar image quality and magnification. Yet, it lacked stereoscopic perception and the adjustability of the camera position was cumbersome. Most users strongly agreed the exoscope would significantly improve surgical teaching. Over 75% reported that they would recommend the exoscope to colleagues and all users saw its great potential for low-resource environments. Conclusion: Our low-budget exoscope is safe and feasible for TLIF and purchasable at a fraction of the cost of conventional microscopes. It may thus help expand access to neurosurgical care and training worldwide.

Papillary dermal elastolysis histopathology mimicking folliculotropic mycosis fungoides.

Papillary dermal elastolysis is a rare acquired disease of the elastic tissue that mainly affects elderly women with a clinical presentation of small firm papules in the neck, the supraclavicular areas and the upper back. Histopathologically, it is characteristic to find a complete or almost complete absence of elastic fibers in the papillary dermis with stains such as orcein or Verhoeff-Van Gieson. We present the case of an adult female patient presenting a clinical picture of years of evolution of elastic skin-colored papules on her neck, occasionally pruritic. Two biopsies were performed. In one of them an inflammatory infiltrate affecting the hair follicles was observed, and she was diagnosed with mycosis fungoides. The other biopsy showed a total absence of elastic fibers in the papillary dermis and was diagnosed as elastolysis of the papillary dermis. In early stages of papillary dermal elastolysis, a perivascular and periadnexal lymphocytic inflammatory infiltrate has been described, as is the case described above. It is important for dermatopathologist to know this atypical but possible presentation, as it may require a differential diagnosis with other entities such as follicular mycosis fungoides.

Effect of the computer-aided static navigation technique on the accuracy of bicortical mini-implants placement site for maxillary skeletal expansion appliances: an in vitro study.

The objective of the present study was to evaluate and compare the effect of the computer-aided static navigation technique on the accuracy of the maxillary skeletal expansion (MSE) appliances. MATERIAL AND METHODS: Forty orthodontic self-drilling mini-implants were placed in ten anatomically based standardized polyurethane models of a completely edentulous upper maxilla, manufactured using a 3D impression procedure. The four orthodontic self-drilling mini-implants for anchoring the MSE appliance were digitally planned on 3D planning software, based on preoperative cone-beam computed tomography (CBCT) scan and a 3D extraoral surface scan. Afterwards, the surgical templates were virtually planned and manufactured using stereolithography. Subsequently, the orthodontic self-drilling mini-implants were placed an postoperative CBCT scans were performed. Finally, coronal entry-point, apical end-point and angular deviations were calculated using a t-test for independent samples or a non-parametric Signed Rank test. RESULTS: Statistically significant differences were not shown at coronal entry-point (p = 0.13), apical end-point (p = 0.41) and angular deviations (p = 0.27) between the planned and performed orthodontic self-drilling mini-implants. CONCLUSIONS: Computer-aided static navigation technique enables accurate orthodontic mini-implant placement for the MSE appliances.

Biopsy type does not affect the number of stages during Mohs micrographic surgery: a retrospective study.

Mohs micrographic surgery (MMS) is the treatment of choice for high-risk basal cell carcinoma (BCC). However, there are no evidence-based recommendations regarding which biopsy type is more appropriate to obtain tumour samples prior to MMS. Shave or punch biopsies are performed depending on the clinical characteristics of the tumour, surgeon experience and local protocols. However, biopsy type might result in difficult histopathological interpretation and influence the practical implementation of MMS. We performed a retrospective study on 208 consecutive BCCs treated with MMS. Of the 208 BCC biopsies, 42 (20.2%) were obtained by the shave method and 166 (79.8%) via punch. Those obtained with the shave technique had a mean of 1.64 stages vs. 1.69 stages with the punch technique (P = 0.130). These findings suggest biopsy type does not affect Mohs surgery performance. The biopsy type of choice is the one deemed adequate for each specific case to obtain a diagnosis and tumour subtyping.

Cutaneous Metastasis of Alveolar Rhabdomyosarcoma in a Child.

ABSTRACT: Rhabdomyosarcoma (RMS) is one of the most common soft tissue sarcomas in children. This lesion is classically included in the generic group of "small round blue cell tumors" along with other entities that share similar microscopic features. Although the head and neck region is a frequent site for primary tumors, cutaneous metastases of RMS involving this anatomical location are rare in the pediatric population. We report a case of a 12-year old girl previously diagnosed with a primary alveolar RMS involving the left maxillary sinus, presenting with a metastatic lesion on the skin of the left temple area. Along with a brief review of the previous case reports on the topic, we highlight the initial immunohistochemistry panel useful for diagnosing this tumor.

Median raphe cysts: A clinico-pathologic and immunohistochemical study of 52 cases.

BACKGROUND: Median raphe cysts (MRC) are epithelial-lined cystic lesions of the genital area that do not communicate with the urethra or the overlying epidermis. Immunohistochemically, MRC show positivity for cytokeratin (CK) 5-6, CK 7, carcinoembryonic antigen, p63 and uroplakin III (URO III). GATA3 and human milk fat globulin 1 (HMFG1) are immunohistochemical markers that have been not previously studied in MRC. METHODS: We conducted a study of 52 patients diagnosed with MRC in the Pathology Departments of eight hospitals between 1990 and 2016. The monoclonal antibodies used were CK5-6, CK7, CK20, URO III, p63, GATA3, and HMFG1. HMFG1 was studied in five cases of apocrine hidrocystomas and compared with five cases of MRC from our series. RESULTS: CK 5-6, CK7, and p63 expression showed strong positivity in the urothelial epithelium of 48 cases. CK20 was focally positive in areas of mucinous differentiation in three cases. GATA3 showed intense nuclear staining in 30 cases. HMFG1 was positive in three cases of MRC and in three cases of apocrine hidrocystoma. CONCLUSION: Positivity of GATA3 and CK7 in MRC supports the urothelial origin of these cysts. We found no differences in HMFG1 expression between MRC and apocrine hidrocystomas.