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Venous malformations (VM) are slow-flow vascular lesions that result from morphogenesis errors. Composed of dysfunctional veins lacking smooth muscle cells, VMs grow slowly and may initially be managed by observation. Treatment is indicated for lesions that cause functional impairment. One treatment option is laser therapy, which is very effective due to its tissue penetration and relative selectivity. The Nd:YAG laser has been shown to treat small and moderately sized mucosal vascular lesions; in this case, it was applied in a staged approach for treatment of a massive venous malformation of the tongue. A 66-year-old male presented with a venous malformation involving the tongue and lower lip, the lesion estimated to be 10 x 14 x 15 cm in size. The Nd:YAG laser was used in three separate stages to photocoagulate the venous lesion. Follow-up for recurrence is ongoing, but currently shows complete malformation ablation with a 36.2% volume reduction after treatment. The Nd:YAG laser is an effective tool in the treatment of large vascular lesions. Successful laser application in a staged approach for the treatment of a venous malformation of this size and complexity further supports its clinical value. Monitoring for possible recurrence of the venous malformation should be ongoing. [ABSTRACT FROM AUTHOR]

ABSTRACT: Background: Intranodal hemangiomas are rare benign vascular tumors of the lymph nodes, often misdiagnosed as malignant lymphadenopathies due to their clinical and radiological features. This case report and systematic review aim to elucidate the epidemiology, clinical profile, and therapeutic interventions for intranodal hemangioma, enhancing diagnostic accuracy and management. Methods: A systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches were conducted in PubMed, Scopus, Web of Science, and Google Scholar up to March 15, 2024, using keywords like "intranodal hemangioma," "primary intranodal hemangioma," "nodal hemangioma," "vascular tumor," and "lymph node hemangioma." Inclusion criteria included studies on clinical presentation, diagnostic strategies, and therapeutic interventions for intranodal hemangioma in patients of all ages. Excluded were reviews, opinion pieces, non-English studies, cases of hemangioendothelioma, sclerosing hemangioma, and animal studies. Key aspects analyzed included demographics, clinical presentation, management strategies, and histopathological findings. Results: Our case involves a 3-year-old female with a submandibular mass, initially diagnosed with hemangioma. After no response to beta-blocker therapy and conflicting imaging results, surgical excision was performed. Histopathology confirmed intranodal hemangioma. From 233 records, 19 publications (36 patients, including our case) met the inclusion criteria. The review shows common clinical presentations as painless, slowly enlarging lymph nodes, primarily in the cervical and axillary regions. Diagnostic challenges often require imaging and biopsy to differentiate from malignancies. Conclusion: Recognizing intranodal hemangiomas, particularly in childhood, is crucial to prevent misdiagnosis and inappropriate treatment. The rarity of these tumors in childhood and their clinical presentation similar to malignancies in older patients make diagnosis challenging without widespread awareness. Surgical excision remains the mainstay of both diagnosis and treatment, often leading to favorable outcomes. [ABSTRACT FROM AUTHOR]

Simple Summary: This study looked at the complications that can occur following nephrectomies for kidney cancer with and without a so-called tumor thrombus, a tumor that has spread into the large vein called the inferior vena cava. So far, insufficient data are available on this topic. We developed a new way to classify the different types of complications, which, along with the established Clavien–Dindo classification, helped to better analyze these complications. We reviewed the data of 88 patients and found that the 44 patients with a tumor thrombus experienced significantly more and more severe complications than the 44 patients without a tumor thrombus. These results highlight the importance of having these complicated surgeries performed in specialized hospitals by experienced urologists and skilled staff. This practice could lead to earlier detection, better prevention, and more effective treatment of any complications arising after such surgeries. Consequently, the research findings could improve clinical practice and optimize treatment outcomes for patients undergoing nephrectomies. Background/Objectives: Radical nephrectomy (RN) with inferior vena cava thrombectomy (IVCT) is indicated for the curative management of renal cell carcinoma (RCC) with tumor thrombus (TT). In the literature, any direct comparison of complications between RNs with or without IVCT is lacking. The objective of this study was to analyze and compare complications after RNs with or without IVCT. Methods: A retrospective evaluation of the complications recorded in RCC patients who underwent RN with (TT group, n = 44) or without (non-TT group, n = 44) IVCT between 2009 and 2021 was conducted. The non-TT group was identified via propensity-score matched-pair analysis. Postoperative complications up until discharge or postoperative day 30, whichever came first, were classified using the Clavien–Dindo classification (CDC). Complications were categorized into cardiovascular, pulmonary, bleeding, gastrointestinal, neurological/psychiatric, wound, urinary tract, dysglycemia, and other groups. Statistical analyses using descriptive statistics included the chi2 and Mann–Whitney U tests. Results: All CDC-grade postoperative complications were more frequent in the TT than in the non-TT group regarding the number of patients affected (93% vs. 73%), as well as per patient (median: 3 vs. 1; p < 0.001). Complications in CDC grade ≥ 3 were rare and comparable between groups. Cardiovascular, gastrointestinal, neurological/psychiatric, and bleeding complications occurred significantly more often in the TT group. However, its small study population and retrospective character limit this study. Conclusions: Significantly more patients undergoing an RN-IVCT experience more frequent postoperative complications than patients with an RN but without IVCT. Surgeons performing the procedures should be experienced, and hospital staff should be trained in the early recognition and treatment of complications. [ABSTRACT FROM AUTHOR]

Background and purpose: Imaging with conventional MRI plays a pivotal role in characterization of skull base bone-intrinsic lesions, yet some lesions are very challenging. The purpose of this study is to evaluate the role of diffusion and perfusion by T2* dynamic susceptibility contrast (DSC) in characterization of such lesions. Results: Lesions showed mostly correlated with approach: Chordomas had low perfusion and intermediate to high perfusion, while chondrosarcoma had ADC value > 1.6 × 10–3/cm2 and more perfused. Metastases had variable ADC values usually intermediate with high perfusion. Plasmacytomas had similar features yet with characteristic conventional morphology and single number. Lymphoma (primary bony) had high perfusion and lowest diffusion ADC (= 04–0.7 × 10–3/cm2). Giant cell tumors and hemangiopericytomas had lowest perfusion. Conclusion: The proposed imaging approach showed very good results and high accuracy in differentiation of skull base bony lesions. [ABSTRACT FROM AUTHOR]

The article focuses on the rare condition of synovial hemangioma of the knee, highlighting its diagnostic challenges and clinical management. Topics include its presentation as a benign vascular lesion causing joint symptoms, the role of imaging techniques like Magnetic resonance imaging (MRI) for accurate diagnosis, and distinguishing it from other synovial membrane-originating masses.

Background: Leiomyosarcoma (LMS) of vascular origin is a rare entity of soft tissue sarcomas. Although they arise mostly from retroperitoneal major vascular structures, some encountered cases may arise from the smaller vascular structures of the thigh as the femoral vein. Due to their origin from the vascular structures, they represent a diagnostic challenge as they may be misdiagnosed as deep vein thrombosis (DVT). Case presentation: We present a case of a 45-year-old woman with left femoral and iliac vein LMS that was previously described in the vascular ultrasound (US) report as extensive DVT involving the femoral and external iliac veins. The patient did not improve after receiving the prescribed anticoagulants. Seven months later, the patient underwent computerized tomography (CT) with contrast, revealing a soft tissue mass in the anatomical site of the left common femoral and external iliac veins. The patient underwent both US-guided tru-cut biopsy and incisional biopsy from the iliac lymph nodes which revealed leiomyosarcoma. Materials and methods: The patient underwent both vascular ultrasound and magnetic resonance imaging of the pelvis and the left thigh at the time of the first presentation. Seven months later, she underwent contrast-enhanced CT of the abdomen and pelvis. Outcome: The patient was referred to the oncology department to receive the appropriate chemotherapy protocol as the tumor was inoperable. Conclusions: Although leiomyosarcoma of vascular origin is a rare entity of neoplasms, it is usually underestimated. A high index of suspicion would help the clinician to suspect such a neoplasm and save time for early diagnosis and management. Special caution should be taken for patients with venous thrombosis not improving on anticoagulants. When there is suspicion, other modalities such as computerized tomography and magnetic resonance imaging help confirm the diagnosis. [ABSTRACT FROM AUTHOR]

Vascular anomalies (VAs) are a heterogeneous group of vascular malformations and tumors that are frequently encountered in daily practice. While most are benign, malignant vascular tumors exist, as do other nonvascular soft-tissue malignancies that may masquerade as a benign VA. The subtleties of arriving at an accurate diagnosis can be intimidating, as new entities are described and names of conditions have been modified. Therefore, the goal of this article is to describe the classic imaging findings of VAs so that the interventional radiologist can confidently and competently contribute to management decisions, with special attention to ominous imaging features that should prompt biopsy or further imaging workup. [ABSTRACT FROM AUTHOR]

Wilms tumor (WT) is the most common kidney tumor in pediatric patients. Intravascular extension of WT above the level of the renal veins is a rare manifestation that complicates surgical management. Patients with intravascular extension are frequently asymptomatic at diagnosis, and tumor thrombus extension is usually diagnosed by imaging. Neoadjuvant chemotherapy is indicated for thrombus extension above the level of the hepatic veins and often leads to thrombus regression, obviating the need for cardiopulmonary bypass in cases of cardiac thrombus at diagnosis. In cases of tumor extension to the retrohepatic cava, neoadjuvant therapy is not strictly indicated, but it may facilitate the regression of tumor thrombi, making resection safer. Hepatic vascular isolation and cardiopulmonary bypass increase the risk of bleeding and other complications when utilized for tumor thrombectomy. Fortunately, WT patients with vena caval with or with intracardiac extension have similar overall and event-free survival when compared to patients with WT without intravascular extension when thrombectomy is successfully performed. Still, patients with metastatic disease at presentation or unfavorable histology suffer relatively poor outcomes. Dedicated pediatric surgical oncology and pediatric cardiothoracic surgery teams, in conjunction with multimodal therapy directed by a multidisciplinary team, are preferred for optimized outcomes in this patient population. [ABSTRACT FROM AUTHOR]

Simple Summary: Endometrial endometrioid carcinoma (EEC) is generally considered to have a good prognosis. However, a subset of patients die of their disease. For this reason, it is essential to reveal and define all prognostic factors that can guide optimal treatment. Because lymphovascular space invasion (LVSI) is now related to staging, and blood vascular invasion seems to be more important than lymphovascular invasion, it could be important to encourage pathologists to distinguish between the two. The morphologic pattern of myometrial invasion may also be related to biological behavior. Myometrial invasion with an infiltrative pattern is associated with advanced-stage LVSI and recurrence. A specific pattern of myometrial invasion characterized by microcystic, elongated, and fragmented (MELF) glands is known to be associated with LVSI and lymph node metastases. This study investigated the relationship between these three closely related parameters, particularly the importance of blood vessel invasion (BVI), lymphatic vessel invasion (LVI), and MELF invasion as prognostic factors for endometrial cancer. This may be of great importance for the therapeutic approach in EEC. We studied 115 cases of EEC diagnosed on hysterectomy specimens. Double immunohistochemical staining (D2-40/CD31) was performed in all 115 cases to show LVI and BVI on the same slide. MELF pattern invasion was present in 24/115 (21%) cases. MELF-positive tumors had a higher frequency of LVI than MELF-negative tumors (58% and 23%, respectively); the frequency of BVI was twice as high in MELF-positive tumors in comparison to MELF-negative tumors (25% and 12%, respectively). These differences were significant (p ˂ 0.0001). All tumors with positive BVI also had a concomitant LVI. The presence of MELF invasion had no impact on overall survival, confirming previous studies. 5-year survival rates were almost equal in cases with negative LVSI and cases with positive isolated LVI (98% vs. 97%). However, in cases where BVI was also present, the 5-year survival rate was significantly lower, 63% (p ˂ 0.0001). Furthermore, BVI proved to be an independent prognostic factor for overall survival, disease-free survival, and recurrence in the multivariate analysis. In conclusion, MELF pattern invasion is a good predictor of lymphatic and blood vessel invasion but has no prognostic value. Our results suggest that BVI in EEC has greater clinical value than isolated LVI or myometrial invasion patterns, and the therapeutic approach should be guided by BVI presence. Therefore, we hope this study will promote the routine evaluation of BVI in the context of EEC diagnostic procedures. [ABSTRACT FROM AUTHOR]

Simple Summary: Discovering hypervascular incidentalomas (HVIs) during intra-arterial therapies (IATs) for hepatocellular carcinoma (HCC) is a common condition, but guidelines lack precise management suggestions. This study examines whether to include HVIs in IAT for HCC patients awaiting liver transplantation. A retrospective study analyzed liver-transplanted HCC patients who received TACE or TARE before LT from 2014 to 2018. The study compared HCC detection rates between pre-interventional imaging and per-interventional CBCT and investigated correlations between HVIs and poor prognosis criteria. Results showed higher nodule detection with CBCT and no significant correlations between HVIs and poor prognosis criteria, tumor recurrence, or mortality. Kaplan–Meier analysis found no significant impact of HVIs on recurrence-free, recurrence-related, or overall survival. These data may indicate that the treatment plan during IAT should not be impacted or modified in response to HVI detection in patients awaiting LT. Background: Current guidelines do not indicate any comprehensive management of hepatic hypervascular incidentalomas (HVIs) discovered in hepatocellular carcinoma (HCC) patients during intra-arterial therapies (IATs). This study aims to evaluate the prognostic value of HVIs detected on per-interventional cone beam computed tomography (CBCT) during IAT for HCC in patients waiting for liver transplantation (LT). Material and methods: In this retrospective single-institutional study, all liver-transplanted HCC patients between January 2014 and December 2018 who received transarterial chemoembolization (TACE) or radioembolization (TARE) before LT were included. The number of ≥10 mm HCCs diagnosed on contrast-enhanced pre-interventional imaging (PII) was compared with that detected on per-interventional CBCT with a nonparametric Wilcoxon test. The correlation between the presence of an HVI and histopathological criteria associated with poor prognosis (HPP) on liver explants was investigated using the chi-square test. Tumor recurrence (TR) and TR-related mortality were investigated using the chi-square test. Recurrence-free survival (RFS), TR-related survival (TRRS), and overall survival (OS) were assessed according to the presence of HVI using Kaplan–Meier analysis. Results: Among 63 included patients (average age: 59 ± 7 years, H/F = 50/13), 36 presented HVIs on per-interventional CBCT. The overall nodule detection rate of per-interventional CBCT was superior to that of PII (median at 3 [Q1:2, Q3:5] vs. 2 [Q1:1, Q3:3], respectively, p < 0.001). No significant correlation was shown between the presence of HVI and HPP (p = 0.34), TR (p = 0.095), and TR-related mortality (0.22). Kaplan–Meier analysis did not show a significant impact of the presence of HVI on RFS (p = 0.07), TRRS (0.48), or OS (p = 0.14). Conclusions: These results may indicate that the treatment plan during IAT should not be impacted or modified in response to HVI detection. [ABSTRACT FROM AUTHOR]

Simple Summary: Hepatocellular carcinoma (HCC) is a life-threatening disease of the liver. Patients who also have a blockage of the portal vein, which takes blood into the liver, are at particular risk for death. This condition is known as portal vein tumor thrombosis (PVTT). Proton beam therapy (PBT) is an excellent treatment option for tumors because it allows the tumor to be irradiated while avoiding radiation effects on normal tissue. In this study, we found that the long-term outcomes in patients with HCC with advanced PVTT (Vp3 or Vp4) were improved by treatment with PBT. In particular, the median survival time after was >20 months in patients treated with PBT for cure of the disease. These results are better than those with other therapies and suggest that PBT gives a survival benefit in these cases. There were also very few adverse events, indicating that PBT is a safe method. Hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) has a poor prognosis and is generally not indicated for surgery. Proton beam therapy (PBT) may offer an alternative treatment. In this study, long-term outcomes were examined in 116 patients (median age 66 years, 100 males) with HCC with advanced PVTT (Vp3 or Vp4) who received PBT from April 2008 to March 2018. Of these patients, 63 received PBT as definitive treatment and 53 as palliative treatment. The representative dose was 72.6 Gy (RBE) in 22 fractions. Eight patients died in follow-up, including 72 due to tumor progression. The 5-year overall survival (OS) rate was 18.0% (95% CI 9.8–26.2%) and the 5-year local control (LC) rate was 86.1% (74.9–97.3%). In multivariate analyses, performance status and treatment strategy were significantly associated with OS. The median follow-up period for survivors with definitive treatment was 33.5 (2–129) months, and the 5-year OS rate was 25.1% (12.9–37.3%) in these cases. The median survival time after definitive irradiation was >20 months. The 5-year OS rate was 9.1% (0–19.7%) for palliative irradiation. These results compare favorably with those of other therapies and suggest that PBT is a useful option for cases of HCC with advanced PVTT that cannot undergo surgery, with an expected survival benefit and good local control. Determining the optimal indication for this treatment is a future challenge. [ABSTRACT FROM AUTHOR]

Simple Summary: This study aimed to explore the relationship of the pN stage of thyroid cancer (TC) and ultrasound hypoechogenicity with encapsulation and vascular invasion. A retrospective study involving 678 TC patients revealed that a greater pN stage was correlated with encapsulation and vascular invasion as well as with positive hypoechogenicity. These findings have significant prognostic and clinical implications for managing TC. Discovering these correlations confirms the importance of the TNM scale in stratifying TC patients based on disease severity. The results suggest that the hypoechoic patterns observed on thyroid ultrasound may serve as indicators of aggressive tumour behaviour and increased invasion risk. This analysis highlights diverse invasion patterns among TC patients, which may have significant clinical implications for diagnostic processes and treatment strategy selection. Further research is necessary to validate these findings and explore new biomarkers and imaging methods to refine TC staging systems and improve TC patient care. In this retrospective study, the relationship between the pN stage of TC and the ultrasound hypoechogenicity of tumour encapsulation and vascular invasion was investigated. The data of a total of 678 TC patients were analysed. The goal of this study was to assess the significance of the pTNM score and preoperative ultrasound features in predicting cancer prognosis and guiding therapeutic decisions in patients with TC. The main research methods included a retrospective analysis of patient data, mainly the pTNM score and presence of tumour encapsulation and vascular invasion obtained from histopathological results and preoperative ultrasound imaging. Patients with well-differentiated TCs (papillary and follicular) were extracted from TC patients to better unify the results because of similar clinical strategies for these TCs. Significant associations were observed between advanced pN stage and the presence of encapsulation and vessel invasion. The majority of pN1a patients exhibited encapsulation (77.71%; p < 0.0001) and vascular invasion (75.30%; p < 0.0001), as did the majority of pN1b patients (100%; p < 0.0001 and 100%; p < 0.0001, respectively). Less than half of the patients with hypoeghogenic patterns presented with encapsulation (43.30%; p < 0.0001) and vascular invasion (43.52%; p < 0.0001), while the vast majority of patients without hypoechogenicity did not present with encapsulation (90.97%; p < 0.0001) or vascular invasion (90.97%; p < 0.0001). Hypoechogenicity was found to be indicative of aggressive tumour behaviour. The results of this study underscore the importance of accurate N staging in TC and suggests the potential use of ultrasound features in predicting tumour behaviour. Further research is needed to confirm these findings and explore additional prognostic markers to streamline TC management strategies and improve patient outcomes. [ABSTRACT FROM AUTHOR]

Background: Masson's tumor, commonly referred to as intravascular papillary endothelial hyperplasia (IPEH), is an uncommon growth of endothelial cells within a vessel wall that is frequently assumed to indicate an abnormal resolution of thrombosis. IPEH is most typically found in the extremities however it is rare for IPEH to appear as a neck tumor. The issue with IPEH is that it could clinically, radiologically, and pathologically imitate some malignant neoplasms such as angiosarcomas creating a diagnostic challenge. Case Report: We describe a 21-year-old male patient who presented with right anterolateral neck swelling for 12 months. Ultrasound revealed a 9.0 × 8.0 cm well-defined echogenic hyper-vascular lesion. The contrast computed tomography (CT) scan of the neck revealed an oval, well-defined subcutaneous mass, measuring 9 × 4.5 cm, situated over and separable from the right sternocleidomastoid muscle with no significant enhancement in the post-contract study. T1-weighted and T2-weighted MRI revealed a 10 × 9 × 7 cm well-defined subcutaneous lobulated lesion superficial to the sternocleidomastoid expanding upward to the Rt. side of the cheek and below to the suprasternal region, eliciting an intermediate signal in T1 and a heterogenous bright signal (mostly fluid) in T2 with low signal foci within the mass. The decision had been reached to entirely excise the lesion surgically with safety margins for histological evaluation. Histological examination indicated thrombosed variable-sized ectatic vascular spaces with papillary formations related to the thrombus, covered with a single layer of flat endothelium, and no features suggestive of malignancy. There was no recurrence at 18 months follow-up post-surgery. Conclusion: Masson's tumor is a benign intravascular disease with an unclear origin and no confirmed inheritance pattern. Presentation of Masson's tumor as a neck mass is incredibly uncommon. Masson's tumor lacks a distinct or distinguishing clinical and radiological appearance. Histopathologic examination is the sole definitive way for diagnosing the disease and the only tool for distinguishing it from angiosarcoma. Surgical excision is the best treatment for IPEH. Recurrence is extremely rare. [ABSTRACT FROM AUTHOR]

* Context.--Recent molecular discoveries have led to improved understanding of tumor biology and the development of new diagnostic assays. Objective.--To review primarily 3 examples of liver tumors and to briefly illustrate how recent molecular discoveries have altered clinical liver pathology practice. Data Sources.--First, we will discuss fibrolamellar carcinoma, which will be the main focus of discussion, as an example for new diagnostic tests that have been developed as a result of molecular discoveries. Additional information on the role of molecular diagnostics in hepatocellular adenoma and hepatocellular carcinoma will be provided. Second, we will use the example of epithelioid hemangioendothelioma as an example of how new diagnostic tools, based on molecular discoveries, may support improved prognostication. Finally, we will use the example of intrahepatic cholangiocarcinoma as an example of a liver tumor where new molecular discoveries have identified tractable therapeutic targets and led to new effective therapies. This portion of the manuscript will also include a description of the anatomic and molecular differences between intrahepatic, hilar, and extrahepatic cholangiocarcinoma. Conclusions.--Fueled by molecular discoveries, new and better diagnostic tests and therapeutic targets have improved clinical care in patients affected by liver tumors. [ABSTRACT FROM AUTHOR]

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Vascular anomalies are localized congenital morphogenetic defects in the blood vessels. They represent a broad spectrum of disorders, ranging from a simple "birthmark" to life-threatening entities. Most (60%) occur in the head and neck region, and each anomaly has a different etiology and clinical behavior; therefore, an accurate diagnosis is crucial for appropriate evaluation and management. This study aims to briefly elaborate on the vascular anomalies, their etiology, and clinical features and assess the role of sclerosing agents in treating venous malformations. The International Society for the Study of Vascular Anomalies (ISSVA) classifies vascular anomalies into vascular tumors and malformations, with subdivisions of each. This classification is accepted worldwide and has become the official system for classifying vascular anomalies. Hemangiomas are the most common type of vascular tumors, while venous malformations are the most common type of vascular malformations. Venous malformations can be treated conservatively without surgical disfigurement. Sclerotherapy is the first-line treatment for venous malformations, as it is an easy-to-perform procedure with minimal equipment needed. [ABSTRACT FROM AUTHOR]

Soft tissue sarcomas are heterogenous group of tumours and the most common site are lower limbs. Primary vascular leiomyosarcomas (LMS) are rare and mostly has venous origin. The diagnosis is made by typical radiological and pathological features. Over the years, treatment for extremity vascular sarcoma has changed from amputation to limb salvage surgery with same oncological outcomes. Here, we report a case study of a 45-year-old gentleman suffering from primary femoral vein leiomyosarcoma and managed successfully with resection of tumor and autologous venous reconstruction. Autologous venous grafts remain an excellent option for vascular reconstruction in resource-constrained setup of low- and middle-income countries. [ABSTRACT FROM AUTHOR]

A case of non‐thrombotic intravascular pathology of the external jugular vein. The patient presented with a 5‐week history of a pain‐less lump on the right side of the neck. There was no history of trauma or associated signs or symptoms. Ultrasound imaging showed an intravenous mass that was excised and histopathologically diagnosed as an intravascular lobular capillary haemangioma (IVLCH). The rare tumour most commonly effects the neck, face, and upper extremities. Ultrasound is often the first‐line imaging in such cases, and IVLCH should be considered in the differential under specific circumstances. [ABSTRACT FROM AUTHOR]

Simple Summary: This study provides a thorough review of robot-assisted partial nephrectomy (RAPN) in managing renal hilar masses. It focuses on the evaluation of RAPN's effectiveness and the exploration of varied surgical methods for these complex tumors. The research underscores the reliability of RAPN and emerging surgical techniques in addressing these challenges with a manageable risk of complications. The findings are poised to significantly contribute to the medical community's understanding and management of renal hilar masses, especially in terms of effectively balancing treatment efficacy and complication risks. Background: Robot-assisted partial nephrectomy (RAPN) is increasingly being employed in the management of renal cell carcinoma (RCC) and it is expanding in the field of complex renal tumors. The aim of this systematic review was to consolidate and assess the results of RAPN when dealing with entirely central hilar masses and to examine the various methods used to address the surgical difficulties associated with them. Methods: A thorough literature search in September 2023 across various databases focused on RAPN for renal hilar masses, adhering to PRISMA guidelines. The primary goal was to evaluate RAPN's surgical and functional outcomes, with a secondary aim of examining different surgical techniques. Out of 1250 records, 13 full-text manuscripts were reviewed. Results: Evidence is growing in favor of RAPN for renal hilar masses. Despite a predominance of retrospective studies and a lack of long-term data, RAPN shows positive surgical outcomes and preserves renal function without compromising cancer treatment effectiveness. Innovative suturing and clamping methods are emerging in surgical management. Conclusions: RAPN is a promising technique for managing renal hilar masses in RCC, offering effective surgical outcomes and renal function preservation. The study highlights the need for more long-term data and prospective studies to further validate these findings. [ABSTRACT FROM AUTHOR]

Simple Summary: Surgical candidacy in the treatment of pancreatic cancer is complex. There are multiple factors influencing if a patient is a good candidate for surgery with or without preceding treatment with chemotherapy. This review summarizes the importance of both anatomical (tumor size and its relation to important blood vessels to the liver and bowel) and biological (aggressiveness of the tumor) considerations. Pancreatic ductal adenocarcinoma (PDAC) remains associated with poor outcomes with a 5-year survival of 12% across all stages of the disease. These poor outcomes are driven by a delay in diagnosis and an early propensity for systemic dissemination of the disease. Recently, aggressive surgical approaches involving complex vascular resections and reconstructions have become more common, thus allowing more locally advanced tumors to be resected. Unfortunately, however, even after the completion of surgery and systemic therapy, approximately 40% of patients experience early recurrence of disease. To determine resectability, many institutions utilize anatomical staging systems based on the presence and extent of vascular involvement of major abdominal vessels around the pancreas. However, these classification systems are based on anatomical considerations only and do not factor in the burden of systemic disease. By integrating the biological criteria, we possibly could avoid futile resections often associated with significant morbidity. Especially patients with anatomically resectable disease who have a heavy burden of radiologically undetected systemic disease most likely do not derive a survival benefit from resection. On the contrary, we could offer complex resections to those who have locally advanced or oligometastatic disease but have favorable systemic biology and are most likely to benefit from resection. This review summarizes the current literature on defining anatomical and biological resectability in patients with pancreatic cancer. [ABSTRACT FROM AUTHOR]

Background: Glomus tumors are benign mesenchymal tumors of the neuromyoarterial glomus bodies. They are generally localized at the subungual area of the fingers. Extradigital or non-subungual glomus tumors are less common. Their diagnosis can be challenging because of their lower incidence, nonspecific symptoms, and the fact that they can be missed during the physical examination. The aim of this case series is to provide an overview of non-subungual glomus tumors, including the patients' epidemiology characteristics, tumor localization and size, their varied imaging manifestations in ultrasound and magnetic resonance imaging, differential diagnosis and histopathological features. Our report included 64 patients with the diagnosis of non-subungual glomus tumor in the Pathology Department at the La Paz University Hospital in Madrid from January 1966 to September 2022. There were 44 men and 20 women (mean age, 55.8 years; range, 8–85 years). The tumors had a mean size of 1 cm and were located in the lower limbs in most cases (59.3%). In 17 cases (27%) a diagnostic imaging test was performed (US, MRI or both). The radiological appearance of a glomus tumor on ultrasound was in most cases a circumscribed hypoechoic oval nodule located in the subcutaneous layer. Spectral Doppler can demonstrate both venous and arterial intralesional flow and in some cases a "Vascular stalk sign,'' seen in 4/9 cases. MRI assists in providing a more detailed characterization of these lesions, which present as T2-weighted hyperintense nodules with avid enhancement. Short conclusion: Glomus tumors are considered rare neoplasms, accounting for a small percentage of all tumors. Our report brings together the most up-to-date information available regarding the imaging findings and differential diagnosis of this entity. Although ultrasound images are not specific, they are crucial for early diagnosis, provide precise location information, and serve as a guide for tumor excision. MRI study helps to better characterize these tumors. The diagnosis is mostly clinical, with confirmation in the histopathologic study. [ABSTRACT FROM AUTHOR]

Simple Summary: Congenital tumors are an uncommon finding on prenatal ultrasound and in the first 3 months of life, and they are (almost) always subjected to magnetic resonance imaging. Although they are usually easy to recognize as pathological masses, differential diagnosis is not easy and includes both benign and malignant conditions. Teratomas are the most frequent group of inborn neoplasms, followed by cardiac rhabdomyomas. In this paper, the authors show a series of cases in order to provide tips to identify the more common masses and to keep in mind that the most unusual tumor may occur as congenital and that no diagnosis should be rejected a priori. The article is intended to raise awareness and draw attention to this little-known group of cancers and facilitate the diagnostic process. Congenital tumors are rare and, owing to this rarity, there is limited information on many of them. A total of 839 fetal and postnatal MRI studies performed in the first 3 months of life were retrospectively reviewed. They were performed with the use of 1.5 T scanners. Seventy-six tumors were diagnosed based on fetal MRI between 20 and 37 gestational weeks, and 27 were found after birth, from 1 day of age to 3 months of life. Teratomas were the most common tumors in our dataset, mainly in the sacrococcygeal region (SCT), followed by cardiac rhabdomyomas and subependymal giant cell astrocytomas (SEGA) associated with TSC, and neuroblastomas. The group of less common tumors consisted of infantile fibrosarcomas, malignant rhabdoid tumors, mesoblastic nephromas and Wilms tumor, craniopharyngiomas, brain stem gliomas, desmoplastic infantile astrocytoma, choroid plexus carcinoma, glioblastoma, hemangiopericytoma, rhabdomyosarcoma, melanoma, mesenchymal hamartomas of the chest wall and the liver, and juvenile xanthogranuloma, with special consideration of blue rubber bleb nevus syndrome. MRI plays a significant role in further and better characterization of congenital tumors, leading to a correct diagnosis in many cases, which is crucial for pregnancy and neonatal management and psychological preparation of the parents. No diagnosis is impossible and can be absolutely excluded. [ABSTRACT FROM AUTHOR]

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning. [ABSTRACT FROM AUTHOR]

* Context.--Unicentric Castleman disease (UCD) is a dynamic entity with a wide spectrum of morphologic findings. UCD can be further subdivided into hyaline-vascular and mixed/plasmacytic variants. Hyaline-vascular UCD has both follicular and interfollicular (stromal) changes, and occasionally these lesions show a skewed representation of either the follicular or stromal compartments. Plasmacytosis is usually minimal in the hyalinevascular variant. The mixed/plasmacytic variant of UCD is composed of sheets of plasma cells often associated with a variable number of follicles with regressive changes. Objective.--To illustrate the differential diagnosis of UCD, as it is quite broad and includes lymphomas, plasma cell neoplasms, stromal neoplasms such as follicular dendritic cell sarcoma and vascular neoplasms, immunoglobulin G4-related disease, infections, and other rare lesions. An additional objective is to enhance awareness of the morphologic features of UCD in excisional and in small core-needle biopsy specimens, the latter of which may inadvertently target follicle- or stroma-rich areas, causing diagnostic challenges. Data Sources.--In this review, we provide readers a concise illustration of the morphologic spectrum of UCD that we have encountered in our practice and a brief discussion of entities in the differential diagnosis. Conclusions.--UCD exhibits a broad spectrum of morphologic changes, and awareness of these morphologic variations is key to avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

Introducion: Renal cell carcinoma (RCC) is one of the most prevalent malignant tumors. It extends up into the systemic veins and right atrium. Surgical extraction of such extensions is usually carried out using cardiopulmonary bypass (CPB) with moderate hypothermic (MH) being frequently applied in order to obtain a clear surgical field. However, due to obvious disadvantages of hypothermia, approaches with mild/normothermia (NT) during CPB have also been established. The current study aims to compare the outcomes of patients undergoing RCC tumor and extensions resection using MH versus NT. Material and Methods: This is a retrospective, non-randomized study. All patients who underwent RCC tumor and extensions resection for stage III or IV (Staehler) RCC in a single center between 2006 and 2020 were included. During surgery, MH or NT were applied. CPB was realized using aortic and bicaval cannulation. We compared the procedural times, transfusion requirements and postoperative outcomes, respectively between the MH and NT groups. Results: A total of 24 consecutive patients (n(NT) = 12, n(MH) = 12) were included in the study (median age NT 68.5 and MH 66.5). The study only showed a significant difference in heart-lung machine times (median CPB time NT 45.5 min and MH 110.0 min, p = 0.004). All other results, loss of drainage, administration of blood products, as well as the postoperative course and mortality were comparable in both groups. Conclusion: The results showed a high perioperative and long-term mortality. The perioperative course was similar after surgery with NT or MH. Therefore, NT which minimizes potential complications of MH should be preferred. [ABSTRACT FROM AUTHOR]

Intravascular papillary endothelial hyperplasia (IPEH), a rare benign endothelial vascular lesion related to thrombosis, generally develops in fingers, trunk, head, and neck. Nevertheless, it has been rarely reported in the salivary gland. In this article, we report a case of IPEH of the right submandibular gland in a 37-year-old female whose initial impression was sialadenitis secondary to sialolithiasis. To date, our case may be the first well-documented report of IPEH in the submandibular gland. The patient underwent ablation of the submandibular gland, and no evidence of recurrence was found during follow-up. [ABSTRACT FROM AUTHOR]

Intracranial hemangiopericytomas are rare vascular tumours, account for less than 1% of all primary tumours of the central nervous system. This tumour usually found outside of CNS i.e., lower extremities and retroperitoneum. Treatment is surgery followed by radiotherapy to decrease chances of recurrences. This is a case report of a young female diagnosed with intracranial hemangiopericytoma in 2021 at our hospital-Acharya Tulsi Regional Cancer Institute, Bikaner. [ABSTRACT FROM AUTHOR]

Objectives: Epithelioid hemangioendothelioma (EHE) is a vascular tumor of intermediate malignant potential, which presents as infiltrative lesions involving multiple organs. We reviewed our institutional experience with the cytologic diagnosis of this neoplasm including the performance of rapid on-site evaluation (ROSE). Material and Methods: From our institutional database, we identified 29 cytology specimens, obtained between 2012 and 2020, from 21 patients with biopsy confirmation of EHE. ROSE and final diagnosis were compared. All cytology slides were reviewed, and selected cytologic features were recorded. Results: The cohort included 29 specimens comprising 17 (59%) from liver, 6 (21%) from lung, 2 (7%) from lymph node, and 4 (14%) from other sites. At ROSE, 8/27 (30%) were reported inadequate, yet on review, all cases contained scattered cells typical of EHE in the touch imprint air-dried slides including two cases reported with a final diagnosis of non-diagnostic. All cases contained epithelioid and plasmacytoid cells with ovoid nuclei, fine chromatin, delicate (or biphasic) cytoplasm, and scattered cells with delicate, elongated cytoplasmic tails. The majority 26/29 (90%) of cases had multi-nucleated and multi-lobated nuclei. Intracytoplasmic lumens/blister cells were in 17/29 (59%), and a subset had erythrocytes therein (4/29, 14%). Metachromatic fibromyxoid or fibrotic stroma fragments were commonly seen (23/29, 79%). Mitoses and necrosis were absent in all cases. Of 11 tested cases, WWTR1::CAMTA1 and YAP1::TFE3 fusions were detected in nine and two cases, respectively. Conclusion: EHE has distinctive cytologic features which are often under-recognized during ROSE. [ABSTRACT FROM AUTHOR]

Simple Summary: Epithelioid haemangioendothelioma (EHE) is an ultra-rare malignant vascular tumour with a prevalence of 1 per 1,000,000. It develops from endothelial cells, which are the cells that line all blood vessels in the body. Therefore, it typically expresses endothelial cell markers. It can also be identified through analysis of the genes. Two genes, WWTR1 and CAMTA1, are broken and fused together in 90% of cases. Alternatively, in approximately 10% of cases, the genes that are broken and fused together are YAP1 and TFE3. We analysed an Australian cohort of EHE patients to look for associations between genetic changes and clinical characteristics. EHE cases are typically refractory to therapies, and no anticancer agents are reimbursed for EHE in Australia; therefore, this detailed study of EHE adds important information to advance our understanding and help aid the design of treatment regimens in the future. Background: Epithelioid haemangioendothelioma (EHE) is an ultra-rare malignant vascular tumour with a prevalence of 1 per 1,000,000. It is typically molecularly characterised by a WWTR1::CAMTA1 gene fusion in approximately 90% of cases, or a YAP1::TFE3 gene fusion in approximately 10% of cases. EHE cases are typically refractory to therapies, and no anticancer agents are reimbursed for EHE in Australia. Methods: We report a cohort of nine EHE cases with comprehensive histologic and molecular profiling from the Walter and Eliza Hall Institute of Medical Research Stafford Fox Rare Cancer Program (WEHI-SFRCP) collated via nation-wide referral to the Australian Rare Cancer (ARC) Portal. The diagnoses of EHE were confirmed by histopathological and immunohistochemical (IHC) examination. Molecular profiling was performed using the TruSight Oncology 500 assay, the TruSight RNA fusion panel, whole genome sequencing (WGS), or whole exome sequencing (WES). Results: Molecular analysis of RNA, DNA or both was possible in seven of nine cases. The WWTR1::CAMTA1 fusion was identified in five cases. The YAP1::TFE3 fusion was identified in one case, demonstrating unique morphology compared to cases with the more common WWTR1::CAMTA1 fusion. All tumours expressed typical endothelial markers CD31, ERG, and CD34 and were negative for pan-cytokeratin. Cases with a WWTR1::CAMTA1 fusion displayed high expression of CAMTA1 and the single case with a YAP1::TFE3 fusion displayed high expression of TFE3. Survival was highly variable and unrelated to molecular profile. Conclusions: This cohort of EHE cases provides molecular and histopathological characterisation and matching clinical information that emphasises the molecular patterns and variable clinical outcomes and adds to our knowledge of this ultra-rare cancer. Such information from multiple studies will advance our understanding, potentially improving treatment options. [ABSTRACT FROM AUTHOR]

Intravenous leiomyomatosis (IVL) is a rare condition characterized by a tumor that originates within a pelvic vein wall or the uterine smooth muscle, with extension into the right heart (referred to as intracardiac leiomyomatosis) in approximately 10% of all IVL cases. Usually, computed tomography (CT) or magnetic resonance imaging (MRI) is performed for diagnostic imaging of IVL. Notably, this neoplasm presents with characteristic ultrasonographic findings. In this report, we present the case of a 49‐year‐old woman with IVL, which extended into the right heart. Echocardiography combined with abdominal ultrasonography was useful to outline the course of the tumor from the right heart to the uterus. Our findings suggest that in addition to CT or MRI, ultrasonography shows high diagnostic value in cases of IVL, and ultrasonography combined with CT or MRI can further improve the preoperative diagnosis rate of IVL. [ABSTRACT FROM AUTHOR]

The article focuses on infantile hemangiomas (IHs), discussing their risk factors, pathogenesis, diagnosis, and management. IHs are common vascular tumors in infants, and while many resolve without complications, understanding their complexities, including genetic predisposition and environmental factors, is crucial for proper diagnosis and treatment.

Glomus tumours are painful superficial tumours, and ultrasonography is an extremely useful and noninvasive diagnostic technique for superficial organs. In this study, we retrospectively examined glomus tumours using ultrasonography. Among 18 patients histopathologically diagnosed with glomus tumours via ultrasonography, we observed five different development sites: subungual areas or those surrounding the nail bed (12), other areas on the finger surface (3), abdominal wall (1), upper arm (1), and forearm (1). The ultrasonographic images revealed significant differences in tumour size, indicating that tumours on other body surfaces tended to be smaller than those on patients' fingers (p < 0.01). The depth/width ratios of tumours on the other body surfaces were significantly higher than those on the fingers (p < 0.05). The tumours showed a regular shape (72.2%) and clear border (100%). Furthermore, most tumours were low-echo tumours with a diameter of up to 15 mm, clear margins, and no lateral shadows. Abundant blood flow and vessels in and out of the tumours were also observed. In conclusion, our study describes the ultrasonographic characteristics of glomus tumours and reveals that they cannot be ruled out when diagnosing small painful subcutaneous tumours. [ABSTRACT FROM AUTHOR]

Background: Intravenous lobular capillary hemangioma (IVLCH) of the neck is a kind of rare benign tumor of vein. Purpose: In this paper, we report two female patients who were hospitalized because of neck masses. Results: The tumors in the neck veins of our patients were white oval masses with pedicle, clearly defined and of different sizes. Their immunohistochemical staining results showed CD31 (+), CD34 (+), SMA (+), ERG (+). The pathological diagnosis was intravenous lobular capillary hemangioma. Conclusions: Due to the location, morphology and immunohistochemistry, This lesion needs to be distinguished from other intravascular lesions such as thrombus, hemangiosarcoma and papillary endothelial hyperplasia. [ABSTRACT FROM AUTHOR]

Simple Summary: Epithelioid haemangioendothelioma is a rare type of cancer with an unpredictable disease course and very few treatment options. To better understand how this type of cancer develops and to uncover possible lines of treatment, it is critical to have experimental approaches to study this cancer. Here, we describe and compare the model systems that are currently available to study this disease. The research undertaken and the discoveries made using each of these experimental models is presented, and the advantages and disadvantages of each model are discussed. Epithelioid haemangioendothelioma (EHE) is a rare sarcoma of the vascular endothelium with an unpredictable disease course. EHE tumours can remain indolent for long period of time but may suddenly evolve into an aggressive disease with widespread metastases and a poor prognosis. Two mutually exclusive chromosomal translocations define EHE tumours, each involving one of the transcription co-factors TAZ and YAP. The TAZ-CAMTA1 fusion protein results from a t(1;3) translocation and is present in 90% of EHE tumours. The remaining 10% of EHE cases harbour a t(X;11) translocation, resulting in the YAP1-TFE3 (YT) fusion protein. Until recently, the lack of representative EHE models made it challenging to study the mechanisms by which these fusion proteins promote tumorigenesis. Here, we describe and compare the recently developed experimental approaches that are currently available for studying this cancer. After summarising the key findings obtained with each experimental approach, we discuss the advantages and limitations of these different model systems. Our survey of the current literature shows how each experimental approach can be utilised in different ways to improve our understanding of EHE initiation and progression. Ultimately, this should lead to better treatment options for patients. [ABSTRACT FROM AUTHOR]

Uterine hemangiopericytoma is extremely rare. This article describes a case of uterine hemangiopericytoma. The tumor involved the parauterine vein; extended into the inferior vena cava, right cardiac cavity, and pulmonary artery; and metastasized to the lungs. It was irregular in shape and exhibited the string‐of‐beads sign on echocardiography, and it was tightly attached to the right ventricular surface and pulmonary artery wall. The patient underwent tumor resection without adjuvant treatment. A pelvic nodule was found 3 months postoperatively and was considered a recurrent lesion. [ABSTRACT FROM AUTHOR]

Context: Congenital vascular anomalies are classically subdivided into vascular tumors and vascular malformations. The role of propranolol in the regression of infantile hemangioma (IH), a vascular tumor, is well established. Aims: This study aimed to analyze the therapeutic efficacy and complications associated with oral propranolol and adjuvant therapy in the treatment of vascular anomalies. Settings and Design: A prospective interventional study was undertaken over 10 years duration extending from 2012 to 2022 at a tertiary care teaching institute. Materials and Methods: All children with cutaneous hemangiomas and lymphatic and venous malformations under 12 years of age, except those with contraindications for administration of propranolol, were included in the study. Results: Out of 382 patients, there were 159 males and 223 females (male: female = 1:1.4). The majority (53.66%) were between ≥3 months and 1 year. There were 481 lesions in 382 patients. There were 348 patients with IH, and 11 were congenital hemangiomas (CHs). There were 23 patients with vascular malformations; lymphatic malformation (n = 19) and venous malformation (n = 4) were present. The size of the lesions ranged from 5 mm to 20 cm; 50.73% were 2-5 cm in size. Ulceration (>5 mm) was the most common complication present in 20/382 (5.24%) patients. Complications related to oral propranolol were seen in 23 (6.02%) patients. Drugs were given for a mean period of 10 months (range from 5 months to 2 years). At the end of the study, 282 (81.03%) out of 348 patients with IH showed an excellent response; 4 (36.36%) patients in the case of CH (n = 11) and 5 (21.74%) patients with vascular malformation (n = 23) showed excellent response. Conclusion: The study validates the use of propranolol hydrochloride as the first-line agent for the treatment of IHs and congenital hemangiomas. It may have an additive role in lymphatic malformations, and venous malformations, as a part of a multimodality treatment approach for vascular malformations. [ABSTRACT FROM AUTHOR]

Simple Summary: Deep myometrial infiltration, clinical risk score, histological type, and lymphovascular space invasion are important clinical variables that have significant management implications for endometrial cancer patients. Determination of these factors using pure T2-weighted MRI is time-consuming, and the accuracy of this relies on the experience of the clinicians. Combining clinical information and radiomic features from MRI, we developed machine learning classification models to predict these clinical variables. Based on a training dataset, an automatic selection classification model with an optimized hyperparameters method was adopted to find the optimal classifiers. The accuracy of the model predictions was evaluated using an independent external testing dataset. The results suggest that an integrated model (combining clinical and radiomic features) achieved a reasonable accuracy for endometrial cancer clinical variable prediction. The application of these models in clinical practice could potentially lead to cost reductions and personalized treatment. Purpose: To predict deep myometrial infiltration (DMI), clinical risk category, histological type, and lymphovascular space invasion (LVSI) in women with endometrial cancer using machine learning classification methods based on clinical and image signatures from T2-weighted MR images. Methods: A training dataset containing 413 patients and an independent testing dataset consisting of 82 cases were employed in this retrospective study. Manual segmentation of the whole tumor volume on sagittal T2-weighted MRI was performed. Clinical and radiomic features were extracted to predict: (i) DMI of endometrial cancer patients, (ii) endometrial cancer clinical high-risk level, (iii) histological subtype of tumor, and (iv) presence of LVSI. A classification model with different automatically selected hyperparameter values was created. The area under the curve (AUC) of the receiver operating characteristic (ROC) curve, F1 score, average recall, and average precision were calculated to evaluate different models. Results: Based on the independent external testing dataset, the AUCs for DMI, high-risk endometrial cancer, endometrial histological type, and LVSI classification were 0.79, 0.82, 0.91, and 0.85, respectively. The corresponding 95% confidence intervals (CI) of the AUCs were [0.69, 0.89], [0.75, 0.91], [0.83, 0.97], and [0.77, 0.93], respectively. Conclusion: It is possible to classify endometrial cancer DMI, risk, histology type, and LVSI using different machine learning methods. [ABSTRACT FROM AUTHOR]

Glomus tumour (GT) is a rare, benign perivascular lesion which arises from the glomus body. GT is most common in the subungual area of fingers. The classical clinical features are pain, cold intolerance, and pinpoint tenderness which are nonspecific. A 48-year-old lady presented with an 8-year history of swelling in the subungual region of her left ring finger and chronic pain with severe exacerbations to touch and exposure to cold. A small, solitary, bluish purple swelling of 0.5 × 0.5 cm was present in the subungual region of the left ring finger with pinpoint tenderness. Magnetic resonance imaging and colour Doppler were suggestive of GT. The lesion was removed en bloc using the subungual approach. The biopsy confirmed the diagnosis of GT. There was a marked reduction in the level of pain and discomfort postoperatively. A subungual small GT can be a rare but treatable cause of chronic finger pain. Most ideal treatment is complete excision. [ABSTRACT FROM AUTHOR]

Eccrine angiokeratomatous hamartoma is a variant of eccrine angiomatous hamartoma. Histopathologically, it shows both features of eccrine angiomatous hamartoma with components of angiokeratoma. Eccrine angiokeratomatous hamartoma is extremely rare. Eccrine angiokeratomatous hamartoma in our case co-existed with intravascular papillary endothelial hyperplasia. This is the first reported case. [ABSTRACT FROM AUTHOR]

Objectives: To differentiate OCVM from other orbital lesions using structural MRI. Methods: This IRB-approved a historical-prospective cohort single-center analysis of a prospective cohort that included consecutive adult patients presenting with an orbital lesion undergoing a 3T MRI before surgery from December 2015 to May 2021. Two readers blinded to all data read all MRIs assessing structural MRI characteristics. A univariate analysis followed by a stepwise multivariate analysis identified structural MRI features showing the highest sensitivity and specificity when diagnosing OCVM. Results: One hundred ninety-one patients with 30/191 (16%) OCVM and 161/191 (84%) other orbital lesions were included. OCVM were significantly more likely to present with a higher signal intensity than that of the cortex on T2WI: 26/29 (89.7%) versus 28/160 (17.5%), p < 0.001, or with a chemical shift artifact (CSA): 26/29 (89.7%) versus 16/155 (10.3%), p < 0.001, or to present with a single starting point of enhancement, as compared to other orbital lesions: 18/29 (62.1%) versus 4/159 (2.5%), p = 0.001. The step-wise analysis identified 2 signatures increasing performances. Signature 1 combined a higher signal intensity than that of the cortex on T2WI and a CSA. Signature 2 included these two features and the presence of a single starting point of enhancement. Sensitivity, specificity, and accuracy were 0.83, 0.94, and 0.92 for signature 1 and 0.97, 0.93, and 0.93 for signature 2, respectively. Conclusion: Structural MRI yields high sensitivity and specificity when diagnosing OCVM. Key Points: • Structural MRI shows high sensitivity and specificity when diagnosing orbital cavernous venous malformation. • We identified two signatures combining structural MRI features which might be used easily in routine clinical practice. • The combination of higher signal intensity of the lesion as compared to the cortex on T2WI and of a chemical shift artifact yields a sensitivity and specificity of 0.83 and 0.94 for the diagnosis of orbital cavernous venous malformation, respectively. [ABSTRACT FROM AUTHOR]

Simple Summary: A solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) of the central nervous system (CNS) represents a rare meningeal tumor with the propensity to recur almost invariably and to metastasize extracranially. Given the rarity of the disease, there are no prospective trials by which to guide its management, and indications for radiotherapy are unclear. The NRG Oncology and European Organization for Research and Treatment of Cancer (EORTC) cooperative groups recently completed the first prospective trials to evaluate risk-adapted radiotherapeutic strategies for meningiomas, based on tumor grade and extent of resection. Using a similar approach, we created three risk categories using two large national US datasets. Our risk categories were highly prognostic of overall and cause-specific survival. Furthermore, our risk categories predicted the survival benefit associated with radiotherapy, which was limited to the high-risk group and, potentially, the intermediate-risk group. Our data suggest that a risk-adapted approach may be employed for the management of SFT/HPC of the CNS. These risk categories may be used in future retrospective and/or prospective studies. Introduction: Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) of the central nervous system (CNS) is a rare meningeal tumor. Given the absence of prospective or randomized data, there are no standard indications for radiotherapy. Recently, the NRG Oncology and EORTC cooperative groups successfully accrued and completed the first prospective trials evaluating risk-adapted adjuvant radiotherapy strategies for meningiomas. Using a similar framework, we sought to develop prognostic risk categories that may predict the survival benefit associated with radiotherapy, using two large national datasets. Methods: We queried the National Cancer Database (NCDB) and the Surveillance, Epidemiology, and End Results (SEER) databases for all newly diagnosed cases of SFT/HPC within the CNS. Risk categories were created, as follows: low risk—grade 1, with any extent of resection (EOR) and grade 2, with gross–total resection; intermediate risk—grade 2, with biopsy/subtotal resection; high risk—grade 3 with any EOR. The Kaplan–Meier method and Cox proportional hazards regressions were used to determine the association of risk categories with overall and cause-specific survival. We then determined the association of radiotherapy with overall survival in the NCDB, stratified by risk group. Results: We identified 866 and 683 patients from the NCDB and SEER databases who were evaluated, respectively. In the NCDB, the 75% survival times for low- (n = 312), intermediate- (n = 239), and high-risk (n = 315) patients were not reached, 86 months (HR 1.60 (95% CI 1.01–2.55)), and 55 months (HR 2.56 (95% CI 1.68–3.89)), respectively. Our risk categories were validated for overall and cause-specific survival in the SEER dataset. Radiotherapy was associated with improved survival in the high- (HR 0.46 (0.29–0.74)) and intermediate-risk groups (HR 0.52 (0.27–0.99)) but not in the low-risk group (HR 1.26 (0.60–2.65)). The association of radiotherapy with overall survival remained significant in the multivariable analysis for the high-risk group (HR 0.55 (0.34–0.89)) but not for the intermediate-risk group (HR 0.74 (0.38–1.47)). Similar results were observed in a time-dependent landmark sensitivity analysis. Conclusion: Risk stratification based on grade and EOR is prognostic of overall and cause-specific survival for SFT/HPCs of the CNS and performs better than any individual clinical factor. These risk categories appear to predict the survival benefit from radiotherapy, which is limited to the high-risk group and, potentially, the intermediate-risk group. These data may serve as the basis for a prospective study evaluating the management of meningeal SFT/HPCs. [ABSTRACT FROM AUTHOR]

Simple Summary: Intracranial solitary fibrous tumours (iSFTs) are exceptional mesenchymal tumours with a high relapse rate. We aimed to analyse the clinical outcome at each stage of the disease. We carried out a multicentre retrospective study including 88 patients from 16 French centres. Gross tumour resection was found to be a factor for good prognosis and significantly reduced local recurrence without impacting overall survival. High-grade tumours were a factor for poorer PFS and LRFS. More than 40% of our patients experienced local recurrence and were mostly treated with surgery and radiotherapy. The first relapse is a turning point in iSFT evolution, with reduced recurrence latency over the course of the disease. The management of repeated recurrence and disseminated diseases is challenging; these situations should be treated, if feasible, with local techniques considering the poor efficiency of systemic treatments. Background: Intracranial solitary fibrous tumour (iSFT) is an exceptional mesenchymal tumour with high recurrence rates. We aimed to analyse the clinical outcomes of newly diagnosed and recurrent iSFTs. Methods: We carried out a French retrospective multicentre (n = 16) study of histologically proven iSFT cases. Univariate and multivariate Cox models were used to estimate the prognosis value of the age, location, size, WHO grade, and surgical extent on overall survival (OS), progression-free survival (PFS), and local recurrence-free survival (LRFS). Results: Eighty-eight patients were included with a median age of 54.5 years. New iSFT cases were treated with gross tumour resection (GTR) (n = 75) or subtotal resection (STR) (n = 9) and postoperative radiotherapy (PORT) (n = 32, 57%). The median follow-up time was 7 years. The median OS, PFS, and LRFS were 13 years, 7 years, and 7 years, respectively. Forty-two patients experienced recurrence. Extracranial metastasis occurred in 16 patients. Median OS and PFS after the first recurrence were 6 years and 15.4 months, respectively. A higher histological grade was a prognosis factor for PFS (p = 0.04) and LRFS (p = 0.03). GTR influenced LRFS (p = 0.03). Conclusion: GTR provided benefits as a first treatment for iSFTs. However, approximately 40% of patients experienced relapse, which remains a challenging state. [ABSTRACT FROM AUTHOR]

BACKGROUND: The presence of lymph-vascular space invasion is a powerful predictor of lymph node metastasis. However, most studies do not distinguish lymph vessel invasion (LVI) and blood vessel invasion (BVI). The aim of this study was to distinguish the role of LVI and BVI in lymphatic metastasis and recurrence in patients with endometrial cancer. METHODS: We examined 171 patients with endometrial cancer. Immunohistochemical double staining was used to distinguish lymphatic invasion and vascular invasion. First, the relationship between lymphatic/vascular invasion and clinicopathological features and lymphatic metastasis was studied. Then, the expression of D2-40/LVI and CD31/BVI in patients with recurrence was analyzed. RESULTS: Pathological grading (G3) and D2-40/LVI were independent high-risk factors for lymph node metastasis of endometrial cancer. The area under the receiver operating characteristic curve values for predicting lymphatic metastasis using pathological grading (G3) or D2-40/LVI alone were .642 and .680, respectively, and the area under the curve value for the combined detection of pathological grading (G3) and D2-40/LVI was .726, which was greater than the values obtained for the abovementioned independent variables. Among the 15 recurrent patients, 5 (33.3%) were D2-40/LVI positive, 2 (13.3%) were CD31/BVI positive, and 8 (53.3%) were both D2-40/LVI and CD31/BVI positive. CONCLUSION: D2-40/LVI combined with G3 can effectively predict lymph node metastasis of endometrial carcinoma. [ABSTRACT FROM AUTHOR]

Background: We report a case of recurrent primary intraventricular synovial sarcoma of the brain with no extracranial primary, initially reported as a haemangiopericytoma. We believe this is the first reported case of primary intraventricular synovial sarcoma at this site.Case Presentation: A 27-year-old male presented to hospital with a new onset of seizures. Imaging revealed a left ventricular trigone mass with surrounding oedema. He underwent a left occipito-temporal craniotomy and resection with the histology reported as haemangiopericytoma. Resection was followed by adjuvant radiation treatment. Seven years later follow-up imaging revealed a 4 mm contrast enhancing lesion in the previous surgical bed. The patient underwent resection. Histological analysis of the recurrence revealed a spindle cell tumour with a SS18 gene rearrangement consistent with synovial sarcoma. Retrospective fluorescent in-situ hybridisation analysis of original histology also revealed a SS18 gene rearrangement consistent with a diagnosis of synovial sarcoma.Conclusion: Synovial sarcoma should be included as part of the differential diagnosis for patients presenting with intraventricular spindle cell tumours in the brain. [ABSTRACT FROM AUTHOR]

Objectives: Congenital vascular lesions commonly present in the head and neck, and most are managed conservatively. Location and rapid growth, however, may necessitate surgical intervention. Endoscopic endonasal surgery (EES) in the pediatric population has emerged as a viable option in treating sinonasal and skull base lesions. Utilizing these techniques in newborns carries unique challenges. The objective of this report is to describe the successful use of direct intralesional embolization followed by endoscopic endonasal resection of a venous malformation in a postnatal patient. Methods: We reviewed the case reported and reviewed the pertinent literature. Results: A 6-week-old infant was found to have a large right-sided sinonasal lesion confirmed as a venous malformation. Rapid growth, impending orbital compromise, and potential long-term craniofacial abnormalities demanded the need for urgent surgical intervention. Risk of bleeding was mitigated with direct intralesional embolization. Immediately afterward, the patient underwent endoscopic endonasal resection of the lesion. EES in the very young presents multiple challenges both anatomically and behaviorally. A multidisciplinary approach lead to a successful outcome. Conclusion: We report a case of a 6-week-old infant, the youngest reported patient to the authors' knowledge, who successfully underwent direct intralesional embolization followed by endoscopic endonasal resection of a sinonasal vascular malformation. This report highlights the challenges of this technique in the very young and demonstrates it as a viable treatment strategy for sinonasal vascular anomalies in this population. [ABSTRACT FROM AUTHOR]

Simple Summary: Intravascular lymphoma is a neoplasm with tumor cells localized exclusively within blood vessel lumina. Most cases are of B-cell origin; only rare cases of NK or T-cell lineage. Unlike intravascular large B-cell lymphoma, which is a well-recognized entity in the WHO classification, intravascular NK/T-cell lymphoma is not considered a specific entity and there is still a debate about whether it is closer to aggressive NK leukemia or to extranodal NK/T-cell lymphoma. Our aim was to summarize the clinical, pathological, and molecular data on intravascular NK/T-cell lymphoma, which is a challenge both in terms of diagnosis and treatment. Recent molecular studies improved our understanding of the mechanism of lymphomagenesis, showing that multiple genetic events associated with EBV infection are required for the pathogenesis of this aggressive lymphoma. Promising therapeutic results may be offered by immune checkpoint inhibitors due to the high PD-L1 expression, which is possibly related to EBV infection. Intravascular lymphoma is a form of lymphoid malignancy characterized by neoplastic cells growing almost exclusively within the lumina of small- to medium-sized blood vessels. Most cases are of B-cell origin with rare cases of natural killer or T-cell lineage. Extranodal sites are affected, mainly the skin and central nervous system, although any organ may be involved. Intravascular NK/T-cell lymphoma deserves special attention because of its clinicopathologic features and the need for adequate immunophenotyping combined with clonality test for a proper diagnosis. Moreover, intravascular NK/T-cell lymphoma is strongly linked to Epstein–Barr virus (EBV), which is considered to play a role in tumorigenesis and to be responsible for the aggressive behavior of the disease. In this paper, we review the current knowledge on this rare lymphoma and, in particular, the most recent advances about its molecular landscape. The main distinguishing features with other EBV-related entities, such as extranodal NK/T-cell lymphoma, EBV-positive primary nodal T/NK-cell lymphoma, and aggressive NK-cell leukemia, are discussed to help pathologists obtain the correct diagnosis and consequently develop an adequate and prompt therapy response. [ABSTRACT FROM AUTHOR]