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PRRX1-fused mesenchymal neoplasm is a recently identified, rare subcutaneous soft tissue neoplasm that is characterized by fusion of PRRX1 (exon 1) with NCOA1 (exon 13) in the majority of reported cases. Although initially considered to be fibroblastic, a possibility of neural or neuroectodermal differentiation has been suggested in a subset of cases. We report a 26-year-old female with a 4.0 cm painless mass located in the subcutis of the left thigh. Microscopically, the tumor was well-circumscribed and multinodular and was composed of relatively monomorphic ovoid to spindle cells arranged in loose fascicles, trabeculae, and cords within alternating myxoid and fibrous matrix, and vascularized stroma. Mitotic figures were scarce and necrosis was not observed. By immunohistochemistry, the neoplastic cells demonstrated focal co-expression of S100 protein and SOX10 and were negative for epithelial membrane antigen, smooth muscle actin, desmin, CD34, STAT6, HMB45, Melan-A, and MUC4. The expression of Rb1 was retained. Targeted RNA-sequencing identified a novel transcript fusion of PRRX1 (exon 1)::NCOA1 (exon 15), which was further confirmed by reverse transcription polymerase chain reaction and Sanger sequencing. The tumor was narrowly excised and no tumor recurrence or metastasis was identified after 13 months of follow-up. In summary, we report a new case of PRRX1-fused mesenchymal neoplasm, expanding the molecular genetic spectrum and providing further support for possible neural or neuroectodermal differentiation of this emerging soft tissue tumor entity., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Managing infectious complications after kidney transplantation (KT) remains a major challenge. Infections are the leading non-cardiovascular cause of death among kidney transplant recipients (KTr). The urinary tract is particularly vulnerable to infections in this group, leading to high levels of morbidity and mortality, as well as significant economic costs., Case Presentation: This case report presents the first documented instance of extensive thigh pyomyositis resulting from cystic fistulae in an 84-year-old KTr. The patient was referred to our hospital with acute onset fever, pain in the inner thighs and pyuria. A CT scan revealed bilateral pyomyositis of the thighs, characterized by multiple abscesses in the adductor muscles and hydroaerobic levels. Additionally, cystic fistulae complicated by pubic symphysis osteitis were identified., Conclusion: In KTr, lower limb pyomyositis resulting from a urinary tract infection is an extremely rare and significantly worsens the overall prognosis for these patients., (© 2024. The Author(s).)

Abstract: Atypical spindle cell/pleomorphic lipomatous tumor is categorized as a benign lipomatous tumor, but various MRI findings pose accurate diagnostic challenges. In our case, both MRI and PET/CT scans indicated the possibility of atypical lipomatous tumor/well-differentiated liposarcoma or dedifferentiated liposarcoma. Needle biopsy suggested benign to low-grade malignancy; hence, we opted for the wide resection. The final diagnosis of atypical spindle cell/pleomorphic lipomatous tumor was confirmed through histopathology analysis, including immunohistochemistry and fluorescence in situ hybridization. Since achieving an accurate diagnosis solely through imaging can be challenging, histopathology remains essential., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To investigate the characteristics and treatment modalities of malignant tumors originating from the sublingual gland, as well as evaluate the therapeutic outcomes following free flap reconstruction., Methods: A retrospective statistical analysis was conducted on the clinical data of nine patients diagnosed with malignant neoplasms tumor of the sublingual gland., Results: Nine case of malignant tumors originated from the sublingual glandular tissue, encompassing eight adenoid cystic carcinoma (ACC) and a single case of bipartite differentiated carcinoma-a hybrid of epithelial-myoepithelial carcinoma and adenoid cystic carcinoma. Among the nine patients, four anterolateral thigh flaps were used (three of which were thin flaps), and five forearm flaps were also empoyed. The size of flaps varied, with the lengths ranging from 4 cm to 9 cm, and the widths ranging from 2.5 cm to 6 cm. The vessels chosen for anastomosis were the superior thyroid artery in seven cases, the facial artery in one case, and the lingual artery in one case. Among the eight patients who underwent dissection of cervical lymph nodes, metastasis were found in one case. Two patients underwent adjuvant radiotherapy. Upon postoperative follow-up, there was no recurrence in any of the nine patients ., Conclusion: The anterolateral thigh perforator flap thinning technique can be employed for postoperative reconstruction of malignant sublingual gland tumors., Competing Interests: Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

The assessment of skin changes in the context of possible child abuse is an important task in forensic medicine. This requires knowledge of pigmentation variants and pigmentation disorders such as congenital dermal melanocytosis, which includes Mongolian spots. Particularly in the case of atypical localization and dark pigmented skin, the differentiation from hematomas can be challenging. A case of two Nigerian siblings with extensive and atypically localized Mongolian spots is reported. The 1.5-year-old girl showed Mongolian spots on her back and the right side of her trunk. The 8-year-old boy showed Mongolian spots only on the back of his thighs. The authors are not aware of any case in which so called Mongolian spots were present exclusively on the back of the thighs and this case is all the more noteworthy as the back of the thigh is a common localization of blunt force trauma., (© 2024. The Author(s).)

Background: Limb-sparing resections of thigh soft tissue sarcomas (STSs) can result in adverse outcomes. Identifying preoperative predictors for wound healing complications, tumor recurrence, and mortality is crucial for informed reconstructive decision-making. We hypothesized that preoperative measurements of thigh and tumor dimensions could serve as reliable indicators for postoperative complications, recurrence, and death., Patients and Methods: In this retrospective cohort study conducted from March 2016 to December 2021, we analyzed patients undergoing thigh STS excisions followed by reconstruction. Preoperative magnetic resonance imaging or computed tomography scans provided necessary thigh and tumor dimensions. Univariate and multivariate regression assessed relationships between these dimensions and postoperative outcomes, including complications, recurrence, and death., Results: Upon the analysis of 123 thighs, we found thigh width to be highly predictive of postoperative complications, even surpassing body mass index (BMI) and retaining significance in multivariate regression [odds ratio (OR) 1.19; 95% CI 1.03-1.39; p = 0.03]. Sarcoma-to-thigh width and thickness ratios predicted STS recurrence, with the thickness ratio retaining significance in multivariate regression (OR 1.03; 95% CI 1.001-1.05; p = 0.041). Notably, greater thigh thickness was independently protective against mortality in multivariate analysis (OR 0.80; 95% CI 0.65-0.98; p = 0.030)., Conclusions: Thigh width outperformed BMI in association with postoperative complications. This may create an opportunity for intervention, where weight loss can play a role during the neoadjuvant therapy period to potentially reduce complications. Sarcoma-to-thigh width and thickness ratios, particularly the latter, hold substantial predictive value in terms of STS recurrence. Moreover, thigh thickness is an independent predictor of survival., (© 2024. Society of Surgical Oncology.)

Hemosiderotic/aneurysmal variant of dermatofibroma (DF) is infrequent and may be misdiagnosed with malignant lesions. We report the case of a giant (7.6cm) subcutaneous hemosiderotic/aneurysmal DF (H/ADF) of the thigh in a 53-year-old female patient. Internal arterial and venous hypervascularity was seen by spectral Doppler ultrasound. Magnetic resonance image showed a discrete homogeneous hypointense in T1-weighted images (WI) and T2-WI mass, with hyperintense areas in fat-suppressed T2-WI. The histology revealed a monotonous fusocelular proliferation without atypia, positive for CD163, factor XIIIa and CD10. Widely distributed hemosiderin pigment and two blood-filled pseudovascular spaces lacking endothelial lining were present. H/ADF was diagnosed. The mass was removed but surgical margins were affected. The patient did not present local relapse or distant metastasis. H/ADF are unusual cutaneous soft tissue tumours that can be clinically, radiologically and histopathologically confused with malignant lesions such as melanomas, vascular lesions or sarcomas, especially in giant cases., (Copyright © 2024 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: To investigate the associations of thigh muscle and fat volumes with structural abnormalities on MRI related to knee osteoarthritis., Materials and Methods: MRI studies of the thighs and knees from 100 individuals were randomly selected from the Osteoarthritis Initiative Cohort. Whole Organ MR Scoring (WORMS) and effusion-synovitis scoring were performed in all knee MRI. Thigh muscles, intermuscular fat, and subcutaneous fat were manually segmented in 15 consecutive MR thigh images. Radiographic Kellgren-Lawrence grades (KLG) were also obtained in all knee radiographs. Independent t-tests were used to investigate the associations between thigh muscle and fat volumes, and sex. Mixed-effects analyses were obtained to investigate the associations between thigh muscle and fat volumes, KLG, WOMAC pain score, cartilage and bone marrow WORMS, as well as effusion-synovitis scores., Results: Women had higher subcutaneous fat volume than men (616.82 vs. 229.13 cm 3 , p < 0.01) and men had higher muscle volumes than women (p < 0.01). Quadriceps (coef = -2.15, p = 0.01) and vastus medialis (coef = -1.84, p = 0.03) volumes were negatively associated with the WORMS cartilage scores. Intermuscular fat volume (coef = 0.48, p = 0.01) was positively associated with WORMS bone marrow edema-like lesion (BMEL) scores. The quadriceps (coef = -0.99, p < 0.01) and hamstring (coef = -0.59, p = 0.01) volumes were negatively associated with WORMS BMEL scores. No evidence of an association was found between thigh muscle and fat volumes with KLG and effusion-synovitis grading (p > 0.05)., Conclusion: Increased quadriceps and hamstring volumes were negatively associated with cartilage lesion and BMEL scores while no evidence of an association was found between thigh muscle and fat volumes, and radiographic knee osteoarthritis or effusion-synovitis grading., (© 2024. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Abstract: Superficial CD34 positive fibroblastic tumor (SCPFT) is a recently described soft tissue tumor of intermediate grade with predilection for subcutaneous and superficial fascial planes. Morphological diagnosis of these tumors can be challenging because of overlapping features with other benign and malignant soft tissue tumors of fibroblastic and myofibroblastic origin. A middle-aged man came with a subcutaneous mass in the left thigh. Microscopic examination showed features of an SCPFT comprised of a circumscribed spindle cell tumor with focal striking pleomorphism and bizarre hyperchromatic nuclei. Immunohistochemically (IHC), the tumor cells showed diffuse positivity for CD34 and focally for cytokeratin. Diagnosis of SCPFT on the basis of morphology alone can be challenging. A constellation of clinicoradiological profiles, IHC features, and sometimes molecular studies clinch the definitive diagnosis which can be helpful in avoiding overtreatment and chemotherapy. Here we highlight the diagnostic challenges and pitfalls in a case of SCPFT., (Copyright © 2023 Copyright: © 2023 Indian Journal of Pathology and Microbiology.)

Muscle mass depletion is associated with mortality and morbidity in various conditions including sepsis. However, few studies have evaluated muscle mass using point-of-care ultrasound in patients with sepsis. This study aimed to evaluate the association between thigh muscle mass, evaluated using point-of-care ultrasound with panoramic view in patients with sepsis in the emergency department, and mortality. From March 2021 to October 2022, this prospective observational study used sepsis registry. Adult patients who were diagnosed with sepsis at the emergency department and who underwent point-of-care ultrasounds for lower extremities were included. The thigh muscle mass was evaluated by the cross-sectional area of the quadriceps femoris (CSA-QF) on point-of-care ultrasound using panoramic view. The primary outcome was 28 day mortality. Multivariable Cox proportional hazard model was performed. Of 112 included patients with sepsis, mean CSA-QF was significantly lower in the non-surviving group than surviving group (49.6 [34.3-56.5] vs. 63.2 [46.9-79.6] cm 2 , p = 0.002). Each cm 2 increase of mean CSA-QF was independently associated with decreased 28 day mortality (adjusted hazard ratio 0.961, 95% CI 0.928-0.995, p = 0.026) after adjustment for potential confounders. The result of other measurements of CSA-QF were similar. The muscle mass of the quadriceps femoris evaluated using point-of-care ultrasound with panoramic view was associated with mortality in patients with sepsis. It might be a promising tool for determining risk factors for mortality in sepsis patients in the early stages of emergency department., (© 2024. The Author(s).)

Competing Interests: The authors declare no conflicts of interest.

Malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue sarcoma with a poor prognosis, affecting most commonly the extremities. The lungs constitute the most frequent location for distant metastases. Half of all MPNSTs arise in patients with neurofibromatosis type 1, while approximately 10% are radiation induced and the rest are sporadic.The authors present a pregnant woman in her 40s with a sporadic MPNST of the lower limb and with lung metastases at diagnosis. Treatment consisted of interilioabdominal amputation, followed by adjuvant chemotherapy. Partial response and disease stabilisation were achieved with chemotherapy.Surgical resection with negative margins is the only potentially curative therapy, while radiation therapy and chemotherapy might be useful in the neoadjuvant or adjuvant setting, but their advantage in survival is not demonstrated. In the reported case, chemotherapy permitted the achievement of partial response and stabilisation of the disease., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

The current case presentation highlights the potential of cemiplimab, a programmed cell death protein-1 (PD-1) inhibitor, as first-line treatment for periocular metastatic cutaneous squamous cell carcinoma (SCC) without requiring curative surgery or radiotherapy. A 64-year-old male presented with a progressing 4.5 × 3.0 cm left upper eyelid lesion initially diagnosed as psoriasis. Work-up revealed cutaneous SCC with tumor invasion into extraconal fat and lacrimal gland, and metastasis to the left parotid lymph node. The patient also presented with a suspicious lesion on his left medial thigh found to be a second primary on pathology. To avoid orbital exenteration and treat the multifocal disease, the patient was started on intravenous cemiplimab immunotherapy. Following six doses, repeated FGD-PET-CT revealed a complete response of the left eyelid lesion and residual low-grade hypermetabolic activity of the left medial thigh lesion. Biopsy confirmed chronic inflammation and fibrosis with no signs of malignancy. This unique case with dual primary cutaneous SCC provides support for cemiplimab in treating locally invasive periocular SCC, and potentially abrogating the need for highly morbid exenteration procedures to preserve binocular vision.

Objectives: To evaluate the relationship of thigh MRI (t-MRI) with manual muscle testing-8 (MMT-8), muscle enzymes and autoantibodies. To determine the causal and mediating factors resulting in poor recovery of MMT-8 in inflammatory myositis (IIM)., Methods: This was a single-centre retrospective study in IIM patients. t-MRI was semi-quantitatively scored for muscle oedema, fascial oedema, muscle atrophy and fatty infiltration. Spearman correlation of t-MRI scores was done with muscle enzymes at baseline, and MMT-8 at baseline and on follow-up. Causal mediation analysis was performed with age, sex, symptom duration, autoantibodies, diabetes and BMI as independent variables, follow-up MMT-8 as dependent and t-MRI scores as mediating variables., Results: Baseline evaluation was done on 59 and follow-up on 38 patients. Median follow-up of the cohort was 31 (10-57) months. Baseline MMT-8 negatively correlated with muscle oedema (r = -0755), fascial oedema (r = -0.443) and muscle atrophy (r = -0.343). Creatinine kinase (r = 0.422) and aspartate transaminase (r = 0.480) positively correlated with muscle oedema. Follow-up MMT-8 correlated negatively with baseline atrophy (r = -0.497) and fatty infiltration (r = -0.531). On follow-up, MMT-8 males had positive total effect (estimate (95%CI)) via atrophy [2.93 (0.44, 4.89)] and fatty infiltration [2.08 (0.54, 3.71)]. Antisynthetase antibody had a positive total effect via fatty infiltration [4.50 (0.37, 7.59)]. Age had a negative total effect via atrophy [-0.09 (0.19, -0.01)] and fatty infiltration [-0.07 (-0.15, -0.01)]. Disease duration had a negative total effect via fatty infiltration [-0.18 (-0.27, -0.02)]., Conclusion: Baseline fatty infiltration and muscle atrophy resulting from older age, female sex, longer disease duration and absent anti-synthetase antibodies, partly mediate muscle recovery in IIM., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: The aim of this study was to evaluate the factors affecting local recurrence and survival in patients with soft-tissue sarcomas located in the thigh., Patients and Methods: This retrospective cross-sectional study evaluated 41 soft tissue sarcoma patients (21 males, 20 females; mean age: 57.9±13.7 years; range, 18 to 90 years) with thigh involvement between January 2010 and December 2020. All surgical intervention was performed by one surgeon with an experience of 15 years in orthopedic oncologic surgery. Epidemiological, radiological, histopathological, and metabolic features, as well as surgical and oncological treatments and prognoses, were assessed. The data was statistically analyzed to determine factors affecting local recurrence and survival in these cases, staged using Enneking and the American Joint Committee on Cancer classifications., Results: Liposarcomas were the most common type of tumor (39%), followed by undifferentiated pleomorphic sarcomas (32%). Tumors >10 cm were associated with decreased survival rates. High-grade tumors, tumor necrosis, Ki-67 index >20%, and positive surgical margins were also associated with lower survival rates. Metastatic patients had significantly lower survival rates. Local recurrence was significantly more frequent in patients with positive surgical margins. Survival rates were significantly lower in metastatic patients., Conclusion: There are many factors that affect local recurrence and survival of soft tissue sarcomas. The size of the mass, the presence of necrosis, a high Ki-67 index, positive surgical margins, and the presence of metastasis are the main factors that should be taken into consideration.

Eccrine acrospiroma is a rare benign tumor of the skin arising from the epithelial cells of eccrine sweat ducts. The clinical picture is characterized by its variability, so a detailed morphological study of the operative material is necessary to establish a diagnosis. Differential diagnosis must be carried out with hemangioma, melanoma, infected sebaceous cyst, metastatic skin lesion, and other tumors from elements of the sweat gland. In the article the authors presented the clinical and morphological analysis of own case from practice of large eccrine acrospiroma on the back surface of the left thigh which was diagnosed in a 56-year-old man.