[Skin manifestations of the external male genitals]. / Hauterscheinungen des männlichen Genitals.

The urological examination includes the inspection of the external male genitals. Harmless normal variants, such as heterotopic sebaceous glands and pearly penile papules must be differentiated from malignant and infectious manifestations. Lichen sclerosus et atrophicus is a frequent connective tissue disease that can lead to functional impairments and an associated high level of suffering for those affected. Both conservative and invasive treatment options are available. Sexually transmitted diseases, such as syphilis, are gaining increasing importance in routine clinical and daily practice due to the increasing incidence in recent years. An early diagnosis and treatment of malignant neoplasms, such as Queyrat's erythroplasia can be carried out by routine inspection of the genital skin.

Platelet-Rich stroma from Crohn's disease patients for treatment of perianal fistula shows a higher myeloid cell profile compared to non-IBD controls.

BACKGROUND: New cell-based therapies are under investigation to improve perianal fistulizing Crohn's disease (pCD) healing. Autologous stromal vascular fraction combined with platelet-rich plasma (referred to as platelet-rich stroma [PRS]) is a new adipose-derived stromal therapy. The effect of Crohn's disease (CD) on adipose tissue, and adipose-derived therapies, is largely unknown. We characterized the cellular composition of subcutaneous lipoaspirate and PRS of pCD patients and non-Inflammatory Bowel Disease (IBD) controls. METHODS: Consecutive pCD patients (≥18 years) and non-IBD controls, who underwent liposuction for the purpose of autologous PRS therapy, were included (October 2020 and March 2021). Mechanically fractionated lipoaspirate and the combined PRS product were analyzed for cell surface marker expression using fluorescence-activated cell sorting analysis. RESULTS: Twenty-three patients (37.8 [IQR 30.7-45.0] years; 9 [39.1 %] male; 11CD patients) were included. Similar total number of cells were found in CD and non-IBD lipoaspirate (CD 8.23 ± 1.62*105 cells/mL versus non-IBD 12.20 ± 3.39*105). Presence of stromal cells, endothelial like cells, immune cells, T-cells, myeloid cells and M2/M1 macrophage ratio were similar in CD and non-IBD lipoaspirate. In PRS samples, more cells/mL were seen in CD patients (P = 0.030). Myeloid cells were more abundant in CD PRS samples (P = 0.007), and appeared to have a higher regulatory M2/M1 ratio. Interdonor variation was observed between lipoaspirate and PRS samples. CONCLUSIONS: The composition of CD and non-IBD lipoaspirate were found to be similar and interdonor variation was observed. However, PRS from CD patients showed more myeloid cells with a regulatory phenotype. Crohn's disease does not appear to alter the immunological composition of adipose-derived products.

Diffusion-Weighted Imaging to Assess HPV-Positive versus HPV-Negative Oropharyngeal Squamous Cell Carcinoma: The Importance of b-Values.

BACKGROUND AND PURPOSE: Controversy exists as to whether ADC histograms are capable to distinguish human papillomavirus-positive (HPV+) from human papillomavirus-negative (HPV-) oropharyngeal squamous cell carcinoma. We investigated how the choice of b-values influences the capability of ADC histograms to distinguish between the two tumor types. MATERIALS AND METHODS: Thirty-four consecutive patients with histologically proved primary oropharyngeal squamous cell carcinoma (11 HPV+ and 23 HPV-) underwent 3T MR imaging with a single-shot EPI DWI sequence with 6 b-values (0, 50, 100, 500, 750, 1000 s/mm2). Monoexponentially calculated perfusion-sensitive (including b=0 s/mm2) and perfusion-insensitive/true diffusion ADC maps (with b ≥ 100 s/mm2 as the lowest b-value) were generated using Matlab. The choice of b-values included 2 b-values (ADCb0-1000, ADCb100-1000, ADCb500-1000, ADCb750-1000) and 3-6 b-values (ADCb0-750-1000, ADCb0-500-750-1000, ADCb0-50-100-1000, ADCb0-50-100-750-1000, ADCb0-50-100-500-750-1000). Readers blinded to the HPV- status contoured all tumors. ROIs were then copied onto ADC maps, and their histograms were compared. RESULTS: ADC histogram metrics in HPV+ and HPV- oropharyngeal squamous cell carcinoma changed significantly depending on the b-values. The mean ADC was lower, and skewness was higher in HPV+ than in HPV- oropharyngeal squamous cell carcinoma only for ADCb0-1000, ADCb0-750-1000, and ADCb0-500-750-1000 (P < .05), allowing distinction between the 2 tumor types. Kurtosis was significantly higher in HPV+ versus HPV- oropharyngeal squamous cell carcinoma for all b-value combinations except 2 perfusion-insensitive maps (ADCb500-1000 and ADCb750-1000). Among all b-value combinations, kurtosis on ADCb0-1000 had the highest diagnostic performance to distinguish HPV+ from HPV- oropharyngeal squamous cell carcinoma (area under the curve = 0.893; sensitivity = 100%, specificity = 82.6%). Acquiring multiple b-values for ADC calculation did not improve the distinction between HPV+ and HPV- oropharyngeal squamous cell carcinoma. CONCLUSIONS: The choice of b-values significantly affects ADC histogram metrics in oropharyngeal squamous cell carcinoma. Distinguishing HPV+ from HPV- oropharyngeal squamous cell carcinoma is best possible on the ADCb0-1000 map.

Cross-Reactivity to Mutated Viral Immune Targets Can Influence CD8+ T Cell Functionality: An Alternative Viral Adaptation Strategy.

Loss of T cell immunogenicity due to mutations in virally encoded epitopes is a well-described adaptation strategy to limit host anti-viral immunity. Another described, but less understood, adaptation strategy involves the selection of mutations within epitopes that retain immune recognition, suggesting a benefit for the virus despite continued immune pressure (termed non-classical adaptation). To understand this adaptation strategy, we utilized a single cell transcriptomic approach to identify features of the HIV-specific CD8+ T cell responses targeting non-adapted (NAE) and adapted (AE) forms of epitopes containing a non-classical adaptation. T cell receptor (TCR) repertoire and transcriptome were obtained from antigen-specific CD8+ T cells of chronic (n=7) and acute (n=4) HIV-infected subjects identified by either HLA class I tetramers or upregulation of activation markers following peptide stimulation. CD8+ T cells were predominantly dual tetramer+, confirming a large proportion of cross-reactive TCR clonotypes capable of recognizing the NAE and AE form. However, single-reactive CD8+ T cells were identified in acute HIV-infected subjects only, providing the potential for the selection of T cell clones over time. The transcriptomic profile of CD8+ T cells was dependent on the autologous virus: subjects whose virus encoded the NAE form of the epitope (and who transitioned to the AE form at a later timepoint) exhibited an 'effective' immune response, as indicated by expression of transcripts associated with polyfunctionality, cytotoxicity and apoptosis (largely driven by the genes GZMB, IFNÉ£, CCL3, CCL4 and CCL5). These data suggest that viral adaptation at a single amino acid residue can provide an alternative strategy for viral survival by modulating the transcriptome of CD8+ T cells and potentially selecting for less effective T cell clones from the acute to chronic phase.

CD127+ CD94+ innate lymphoid cells expressing granulysin and perforin are expanded in patients with Crohn's disease.

Phenotypic definition of helper ILC1 and NK cells is problematic due to overlapping markers. Recently we showed the identification of cytotoxic ILC3s characterized by expression of CD94. Here we analyse CD127+ ILCs and NK cells in intestinal lamina propria from healthy donors and Crohn's disease patients and identify two populations of CD127+CD94+ ILCs, designated population A and B, that can be distinguished on the expression of CD117, CD18 and cytotoxic molecules. Population B expresses granulysin, a cytotoxic molecule linked to bacterial lysis and/or chemotaxis of monocytes. Granulysin protein is secreted by population B cells upon stimulation with IL-15. Activation of population B in the presence of TGF-ß strongly reduces the expression of cytotoxic effector molecules of population B. Strikingly, samples from individuals that suffer from active Crohn's disease display enhanced frequencies of granulysin-expressing effector CD127+CD94+ ILCs in comparison to controls. Thus this study identifies group 1 ILC populations which accumulate in inflamed intestinal tissue of Crohn's disease patients and may play a role in the pathology of the disease.

Non-donor specific anti-human leukocyte antigen (HLA) antibodies are not associated with poor outcome in hematopoietic stem cell transplant recipients.

Testing for anti-human leukocyte antigen (HLA) antibodies has now become standard practice in allogeneic hematopoietic stem cell transplantation (HSCT), and anti-HLA antibodies (both donor specific and non-donor specific) are being identified and have many potential consequences. Most studies suggest that donor-specific HLA antibodies lead to adverse outcomes, though little is reported on non-donor specific anti-HLA antibodies. We present the results of a retrospective cohort analysis of 157 patients who received HSCT at the University of Rochester over a period of four years. We identified 45 patients (28.7%) who had detectable anti-HLA antibodies, while only one patient (0.6%) had donor-specific anti-HLA antibodies. Patients with prior pregnancies and multiple transfusions were at increased risk to develop antibodies. In our cohort, the presence of non-donor specific anti-HLA antibodies did not significantly impact overall survival, progression free survival, graft failure, or transplant-related mortality.

Human Tissue-Resident Mucosal-Associated Invariant T (MAIT) Cells in Renal Fibrosis and CKD.

BACKGROUND: Mucosal-associated invariant T (MAIT) cells represent a specialized lymphocyte population associated with chronic inflammatory disorders. Little is known, however, about MAIT cells in diseases of the kidney, including CKD. METHODS: To evaluate MAIT cells in human native kidneys with tubulointerstitial fibrosis, the hallmark of CKD, we used multicolor flow cytometry to identify, enumerate, and phenotype such cells from human kidney tissue biopsy samples, and immunofluorescence microscopy to localize these cells. We cocultured MAIT cells and human primary proximal tubular epithelial cells (PTECs) under hypoxic (1% oxygen) conditions to enable examination of mechanistic tubulointerstitial interactions. RESULTS: We identified MAIT cells (CD3+ TCR Vα7.2+ CD161hi) in healthy and diseased kidney tissues, detecting expression of tissue-resident markers (CD103/CD69) on MAIT cells in both states. Tissue samples from kidneys with tubulointerstitial fibrosis had significantly elevated numbers of MAIT cells compared with either nonfibrotic samples from diseased kidneys or tissue samples from healthy kidneys. Furthermore, CD69 expression levels, also an established marker of lymphocyte activation, were significantly increased on MAIT cells from fibrotic tissue samples. Immunofluorescent analyses of fibrotic kidney tissue identified MAIT cells accumulating adjacent to PTECs. Notably, MAIT cells activated in the presence of human PTECs under hypoxic conditions (modeling the fibrotic microenvironment) displayed significantly upregulated expression of CD69 and cytotoxic molecules perforin and granzyme B; we also observed a corresponding significant increase in PTEC necrosis in these cocultures. CONCLUSIONS: Our findings indicate that human tissue-resident MAIT cells in the kidney may contribute to the fibrotic process of CKD via complex interactions with PTECs.

A two-step risk assessment method for radiofrequency ablations of spine metastases.

BACKGROUND: Spine metastases (MTS) can be treated via Radiofrequency Ablation (RFA) electrodes. To bring these electrodes into vertebral MTS, pathways have to be created. This can be done via transpedicular hammering or drilling. However, this is challenging due to spatial constraints, and because MTS can alter bone density considerably. METHOD: In this work a two-step method is presented that intends to offer cognitive and physical assistance. Step 1 comprises two visualization methods that depict safety margins between and in risk structures. For Step 2, the correlation between Hounsfield Units (HUs) and drilling forces was analyzed to support manual and robot-assisted RFAs. RESULTS: In-depth descriptions of two clinical cases and detailed feedback from the local clinic of neuroradiology are used to present the capabilities of the proposed method. Furthermore, a stiffness criterion is presented to predict drilling force changes from the local distribution and homogeneity of HUs with an inaccuracy of less than 1 mm. CONCLUSIONS: The combination of visualization and drilling force prediction shows potential to support manual and robot-assisted spine RFAs. However, limitations have to be addressed in the future. For example, it has to be carefully evaluated to which extent the proposed method can speed up the planning process and increase intervention safety.

Materials processing using radio-frequency ion-sources: Ion-beam sputter-deposition and surface treatment.

The capabilities of ion-beam techniques for thin-film processing, i.e., for materials deposition by ion-beam sputtering and surface treatment, are reviewed. The basic interaction mechanisms between ions and solids are summarized and related to materials processing by ion sources. Typical geometries of ion sources, targets, and samples are discussed for corresponding experimental apparatus. The versatility of ion-beam techniques in the preparation of thin films and multilayer structures is illustrated by several examples: ion-beam sputter-deposition of various binary oxide materials (including crystalline MgO, NiO, ZnO, SnxOy, and CuxOy) as well as combinatorial growth of materials libraries of amorphous ternary oxides. Furthermore, controlled ion-beam etching of surfaces is discussed.

Impact of Data Presentation on Physician Performance Utilizing Artificial Intelligence-Based Computer-Aided Diagnosis and Decision Support Systems.

Ultrasound (US) is a valuable imaging modality used to detect primary breast malignancy. However, radiologists have a limited ability to distinguish between benign and malignant lesions on US, leading to false-positive and false-negative results, which limit the positive predictive value of lesions sent for biopsy (PPV3) and specificity. A recent study demonstrated that incorporating an AI-based decision support (DS) system into US image analysis could help improve US diagnostic performance. While the DS system is promising, its efficacy in terms of its impact also needs to be measured when integrated into existing clinical workflows. The current study evaluates workflow schemas for DS integration and its impact on diagnostic accuracy. The impact on two different reading methodologies, sequential and independent, was assessed. This study demonstrates significant accuracy differences between the two workflow schemas as measured by area under the receiver operating curve (AUC), as well as inter-operator variability differences as measured by Kendall's tau-b. This evaluation has practical implications on the utilization of such technologies in diagnostic environments as compared to previous studies.

[Precision pulse capsulotomy : The new capsulorhexis?] / Präzisionspulskapsulotomie : Die neue Kapsulorhexis?

The manual capsulorhexis created by Neuhann is still the standard procedure for opening the anterior capsule for cataract surgery. A limitation is the inaccuracy in the size and placement of the opening due to manual execution. In addition to the femtosecond laser a possible improvement in the standardization of capsulorhexis is provided by the Zepto procedure (precision pulse capsulotomy, PPC). In this case a 5.25 mm rhexis is created in a standardized fashion with a flexible suction adapter in which a nitinol ring is located. Whether the strength of PPC is comparable or better than that of the manual technique and how it behaves in terms of capsule shrinkage has not yet been finally clarified.

MRI with DWI for the Detection of Posttreatment Head and Neck Squamous Cell Carcinoma: Why Morphologic MRI Criteria Matter.

BACKGROUND AND PURPOSE: Although diffusion-weighted imaging combined with morphologic MRI (DWIMRI) is used to detect posttreatment recurrent and second primary head and neck squamous cell carcinoma, the diagnostic criteria used so far have not been clarified. We hypothesized that precise MRI criteria based on signal intensity patterns on T2 and contrast-enhanced T1 complement DWI and therefore improve the diagnostic performance of DWIMRI. MATERIALS AND METHODS: We analyzed 1.5T MRI examinations of 100 consecutive patients treated with radiation therapy with or without additional surgery for head and neck squamous cell carcinoma. MRI examinations included morphologic sequences and DWI (b=0 and b=1000 s/mm2). Histology and follow-up served as the standard of reference. Two experienced readers, blinded to clinical/histologic/follow-up data, evaluated images according to clearly defined criteria for the diagnosis of recurrent head and neck squamous cell carcinoma/second primary head and neck squamous cell carcinoma occurring after treatment, post-radiation therapy inflammatory edema, and late fibrosis. DWI analysis included qualitative (visual) and quantitative evaluation with an ADC threshold. RESULTS: Recurrent head and neck squamous cell carcinoma/second primary head and neck squamous cell carcinoma occurring after treatment was present in 36 patients, whereas 64 patients had post-radiation therapy lesions only. The Cohen κ for differentiating tumor from post-radiation therapy lesions with MRI and qualitative DWIMRI was 0.822 and 0.881, respectively. Mean ADCmean in recurrent head and neck squamous cell carcinoma/second primary head and neck squamous cell carcinoma occurring after treatment (1.097 ± 0.295 × 10-3 mm2/s) was significantly lower (P < .05) than in post-radiation therapy inflammatory edema (1.754 ± 0.343 × 10-3 mm2/s); however, it was similar to that in late fibrosis (0.987 ± 0.264 × 10-3 mm2/s, P > .05). Although ADCs were similar in tumors and late fibrosis, morphologic MRI criteria facilitated distinction between the 2 conditions. The sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios (95% CI) of DWIMRI with ADCmean < 1.22 × 10-3 mm2/s and precise MRI criteria were 92.1% (83.5-100.0), 95.4% (90.3-100.0), 92.1% (83.5-100.0), 95.4% (90.2-100.0), 19.9 (6.58-60.5), and 0.08 (0.03-0.24), respectively, indicating a good diagnostic performance to rule in and rule out disease. CONCLUSIONS: Adding precise morphologic MRI criteria to quantitative DWI enables reproducible and accurate detection of recurrent head and neck squamous cell carcinoma/second primary head and neck squamous cell carcinoma occurring after treatment.

Identification and Quantitation of Leukocyte Populations in Human Kidney Tissue by Multi-parameter Flow Cytometry.

Inflammatory immune cells play direct pathological roles in cases of acute kidney injury (AKI) and chronic kidney disease (CKD). However, the identification and characterization of distinct populations of leukocytes in human kidney biopsies have been confounded by the limitations of immunohistochemical (IHC)-based techniques used to detect them. This methodology is not amenable to the combinations of multiple markers necessary to unequivocally define discrete immune cell populations. We have developed a multi-parameter, flow cytometric-based approach that addresses the need for panels of cell-specific markers in the identification of immune cell populations, allowing both the accurate detection and quantitation of leukocyte subpopulations from a single, clinical kidney biopsy specimen. In this approach, fresh human kidney tissue is dissociated into a single cell suspension followed by antibody-labeling and flow cytometric-based acquisition and analysis. This novel technique provides a major step forward in identifying and enumerating immune cell subpopulations in human kidney disease and is a powerful platform to complement traditional histopathological examinations of clinical kidney biopsies.

Apparent Diffusion Coefficient Histograms of Human Papillomavirus-Positive and Human Papillomavirus-Negative Head and Neck Squamous Cell Carcinoma: Assessment of Tumor Heterogeneity and Comparison with Histopathology.

BACKGROUND AND PURPOSE: Head and neck squamous cell carcinoma associated with human papillomavirus infection represents a distinct tumor entity. We hypothesized that diffusion phenotypes based on the histogram analysis of ADC values reflect distinct degrees of tumor heterogeneity in human papillomavirus-positive and human papillomavirus-negative head and neck squamous cell carcinomas. MATERIALS AND METHODS: One hundred five consecutive patients (mean age, 64 years; range, 45-87 years) with primary oropharyngeal (n = 52) and oral cavity (n = 53) head and neck squamous cell carcinoma underwent MR imaging with anatomic and diffusion-weighted sequences (b = 0, b = 1000 s/mm2, monoexponential ADC calculation). The collected tumor voxels from the contoured ROIs provided histograms from which position, dispersion, and form parameters were computed. Histogram data were correlated with histopathology, p16-immunohistochemistry, and polymerase chain reaction for human papillomavirus DNA. RESULTS: There were 21 human papillomavirus-positive and 84 human papillomavirus-negative head and neck squamous cell carcinomas. At histopathology, human papillomavirus-positive cancers were more often nonkeratinizing (13/21, 62%) than human papillomavirus-negative cancers (19/84, 23%; P = .001), and their mitotic index was higher (71% versus 49%; P = .005). ROI-based mean and median ADCs were significantly lower in human papillomavirus-positive (1014 ± 178 × 10-6 mm2/s and 970 ± 187 × 10-6 mm2/s, respectively) than in human papillomavirus-negative tumors (1184 ± 168 × 10-6 mm2/s and 1161 ± 175 × 10-6 mm2/s, respectively; P < .001), whereas excess kurtosis and skewness were significantly higher in human papillomavirus-positive (1.934 ± 1.386 and 0.923 ± 0.510, respectively) than in human papillomavirus-negative tumors (0.643 ± 0.982 and 0.399 ± 0.516, respectively; P < .001). Human papillomavirus-negative head and neck squamous cell carcinoma had symmetric normally distributed ADC histograms, which corresponded histologically to heterogeneous tumors with variable cellularity, high stromal component, keratin pearls, and necrosis. Human papillomavirus-positive head and neck squamous cell carcinomas had leptokurtic skewed right histograms, which corresponded to homogeneous tumors with back-to-back densely packed cells, scant stromal component, and scattered comedonecrosis. CONCLUSIONS: Diffusion phenotypes of human papillomavirus-positive and human papillomavirus-negative head and neck squamous cell carcinomas show significant differences, which reflect their distinct degree of tumor heterogeneity.

Interferon-γ production by tubulointerstitial human CD56bright natural killer cells contributes to renal fibrosis and chronic kidney disease progression.

Natural killer (NK) cells are a population of lymphoid cells that play a significant role in mediating innate immune responses. Studies in mice suggest a pathological role for NK cells in models of kidney disease. In this study, we characterized the NK cell subsets present in native kidneys of patients with tubulointerstitial fibrosis, the pathological hallmark of chronic kidney disease. Significantly higher numbers of total NK cells (CD3-CD56+) were detected in renal biopsies with tubulointerstitial fibrosis compared with diseased biopsies without fibrosis and healthy kidney tissue using multi-color flow cytometry. At a subset level, both the CD56dim NK cell subset and particularly the CD56bright NK cell subset were elevated in fibrotic kidney tissue. However, only CD56bright NK cells significantly correlated with the loss of kidney function. Expression of the tissue-retention and -activation molecule CD69 on CD56bright NK cells was significantly increased in fibrotic biopsy specimens compared with non-fibrotic kidney tissue, indicative of a pathogenic phenotype. Further flow cytometric phenotyping revealed selective co-expression of activating receptor CD335 (NKp46) and differentiation marker CD117 (c-kit) on CD56bright NK cells. Multi-color immunofluorescent staining of fibrotic kidney tissue localized the accumulation of NK cells within the tubulointerstitium, with CD56bright NK cells (NKp46+ CD117+) identified as the source of pro-inflammatory cytokine interferon-γ within the NK cell compartment. Thus, activated interferon-γ-producing CD56bright NK cells are positioned to play a key role in the fibrotic process and progression to chronic kidney disease.

Minimally invasive, multi-port approach to the lateral skull base: a first in vitro evaluation.

PURPOSE: The aim of the study was to validate a minimally invasive, multi-port approach to the internal auditory canal at the lateral skull base on a cadaver specimen. METHODS: Fiducials and a custom baseplate were fixed on a cadaver skull, and a computed tomography image was acquired. Three trajectories from the mastoid surface to the internal auditory canal were computed with a custom planning tool. A self-developed positioning system with a drill guide was attached to the baseplate. After referencing on a high precision coordinate measuring machine, the drill guide was aligned according to the planned trajectories. Drilling of three trajectories was performed with a medical stainless steel drill bit. RESULTS: The process of planning and drilling three trajectories to the internal auditory canal with the presented workflow and tools was successful. The mean drilling error of the system (Euclidian distance between the planned trajectory and centerline of the actual drilled canal) was [Formula: see text] mm at the entry point and [Formula: see text] mm at the target. The inaccuracy of the drill process itself and its physical limitations were identified as the main contributing factors. CONCLUSION: The presented system allows the planning and drilling of multiple minimally invasive canals at the lateral skull base. Further studies are required to reduce the drilling error and evaluate the clinical application of the system.

[Systemic sclerosis : What is currently available for treatment?] / Systemische Sklerose : Was ist gesichert in der Therapie?

Systemic sclerosis (scleroderma) is a rheumatologic disease characterised not only by inflammation/autoimmunity, but also by tissue fibrosis and vascular lesions. The therapeutic approach to patients is dictated by the organ involvement and includes treatment of vascular and fibrotic disease features beyond mere immunosuppression. Fibrotic features in particular, are still inadequately treated, whereas many drugs have been tested for vascular complications within recent years. In this review, the currently available treatment options for this rare disease are presented. Therapy options in systemic sclerosis have changed over the past 10 years and this trend will also continue in the future.

Synovial sarcoma of the hypopharynx in a pediatric patient: Case report.

INTRODUCTION: Synovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head & Neck. In the pediatric population, SS is an extremely rare head & neck malignancy. PRESENTATION OF CASE: We present a case of sixteen years old boy diagnosed with SS situated of the hypopharynx treated by surgical excision and post operative radio-chemotherapy. DISCUSSION: This anatomical location brings additional functional challenges (swallowing, phonation, respiration), especially in the pediatric population. Pre-operative and even post-operative histopathological diagnosis of SS remains difficult. Optimal treatment of Head & Neck SS has to balance functional and oncologic aspects. CONCLUSION: SS is an extremely rare head & neck malignancy in pediatric population. It has multifaceted challenges including pre and post-operative histopathological diagnosis and optimal modality of treatment. Clinical judgment, especially in the pediatric population, needs to balance tumor free margins and organ preservation in head and neck region.