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Kasabach–Merritt syndrome (KMS) is a coagulopathy characterized by thrombocytopenia and hypofibrinogenemia associated with large vascular tumors. KMS can lead to disseminated intravascular coagulation which, if not treated promptly, can be life-threatening. There are few reported cases of this syndrome and its evolution in pregnant women. [ABSTRACT FROM AUTHOR]

Objective : To evaluate the curative effect of Potassium titanyl phosphate (KTP) laser and pingyangmycin injection for adult laryngeal hemangiomas (ALH). Methods : This was a retrospective study conducted on patients treated with either KTP laser or pingyangmycin injection to assess the efficacy of both treatment and compare the effects on different types of adult laryngeal hemangioma. Results : The ordinal logistic regression test showed the surgery methods had no effect on the therapeutic results. On the contrary, the shape of ALH and the interaction between surgical procedures and ALH shape affected the prognosis of the ALH. This meant that the shape of ALH might be a confounding factor affecting the therapeutic effect of ALH. Thus, the Cochran–Mantel–Haenszel test which is a stratification analysis was used to assess the interaction between surgical procedures and ALH shape. Then better results were achieved using the KTP laser for the plane and raised types of ALH. Conclusions : The selection of surgical procedures (the KTP laser or pingyangmycin injection approaches) affects the treatment of ALH. For plane and raised ALH, the KTP laser may be recommended. [ABSTRACT FROM AUTHOR]

Although hemangiomas are common in the head and neck area, they are uncommon in the temporal bone. External auditory canal (EAC) hemangiomas are a relatively uncommon otologic condition. In the English literature, only 29 cases of temporal bone hemangioma have been reported. We also present the case of a 32-year-old male patient who presented with an 8-month history of decreased hearing, tinnitus, and aural fullness on the right side. Otoscopic examination of the right ear revealed a smooth, oval, well-circumscribed, reddish, pulsatile, nontender mass, which was about 1 cm in diameter and occupied two-thirds of the EAC. It was soft in consistency, compressible, and appeared to be arising from the right posterior-superior portion of the bony canal wall; the tympanic membrane seemed to be uninvolved. The patient had mild conductive hearing loss of the right ear with an average air-bone gap of 20 dB; the left side was normal. High-resolution contrast-enhanced computed tomographic scanning of the temporal bone showed well-defined, rounded, homogenously enhancing lesions at the posterior-superior aspect of right EAC measuring 0.7 × 0.8 cm. Angiography was performed and there was evidence of vascular blush in the region of the right EAC. Three blood vessels were identified and embolized. The mass was completely removed by the endaural approach, and a retroauricular skin graft to restore cutaneous integrity was not required. Histopathology indicated a capillary hemangioma without cytological atypia or mitotic activity. There was no recurrence 1 year after the surgery. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioma is a very rare lesion arising from vascular structures. We encountered a case in very atypical location i.e. on the anterior maxillary wall. The underwent excision of tumour twice elsewhere and presented in our hospital with reccurence of mass. Patient was managed in our hospital with good cosmetic outcome with no disease recurrence till date. [ABSTRACT FROM AUTHOR]

Spinal involvement of infantile hemangiomas is rare with the predilection to involve the epidural space. A proper diagnosis might be challenging due to the atypical location and variable/inconsistent use of the International Society for the Study of Vascular Anomalies (ISSVA) classification by radiologists, pathologists, and clinicians. A proper diagnosis of epidural infantile hemangioma is key due to the different aggressiveness of the treatment options with inconstant literature regarding the best available treatment. Herein, we present a case of a massive epidural infantile hemangioma successfully treated with only beta-blocker. We discuss the clinical, MRI, CT, ultrasound, and histological features of this lesion as we review the literature with the objective of addressing some of the confusion surrounding the subject. [ABSTRACT FROM AUTHOR]

Background: Intramuscular hemangioma is a vascular malformation occurring in muscle tissues. It is most common in skeletal muscles of limbs, especially lower limbs in childhood. The intercostal intramuscular hemangioma with sternal invasion is very rare. Case presentation: The 47-year-old female patient was hospitalized due to "suddenly chest pain for 4 days". Chest contrast-enhanced CT indicated bony changes in the sternum, accompanied by soft tissue shadows in the posterior sternum and diaphragmatic angle. After admission, physical examination showed: peristernal tenderness, percussion pain, other no obvious positive signs. PET-CT examination indicated that there was a high possibility of benign sternal changes. No cancer cells were found by sternal puncture. Surgical biopsy was performed and pathological findings were consistent with intramuscular hemangioma. Conclusion: Intramuscular hemangioma with bone invasion is very rare, imaging examination is difficult to indicate obvious evidence, preoperative diagnosis is difficult, complete surgical resection is very important, more personalized treatment should be provided according to the overall situation of the patient. [ABSTRACT FROM AUTHOR]

Infantile hemangioma (IH) is a vascular anomaly observed in newborns, with potential severe complications if left undetected. Consequently, researchers have turned to artificial intelligence (AI) and digital imaging (DI) methods for detection, segmentation, and assessing the treatment response in IH cases. This paper conducts a systematic literature review (SLR) following the Kitchenham framework to scrutinize the utilization of AI and digital imaging techniques in IH applications. A total of 21 research articles spanning from 2014 to April 2024 were carefully selected and analyzed to address four key research questions: the issues solved in IH using AI and DI, the most-used AI and DI techniques, the best-performing technique in detecting IH, and the limitations and future directions in the various fields of IH. After an extensive review of the selected articles, it was found that 10 of the 21 articles focused on detecting IH, and 15 articles utilized AI. However, the best-performing technique in detecting IH employed DI. Additionally, the SLR offers insights and recommendations into future directions for IH applications. [ABSTRACT FROM AUTHOR]

Key Clinical Message: A 26‐year‐old patient with swelling on the lingual surface of the mandible in the incisors area was referred to the Faculty of Dentistry at Mashhad University of Medical Sciences. After conducting clinical, radiographic, and pathological examinations, the patient was diagnosed with capillary hemangioma. This study explores the clinical manifestation and unusual behavior of intraoral hemangioma. Hemangioma is a tumor that occurs due to the proliferation of endothelial cells in 4%–5% of infants and affects the head and neck region in almost 70% of cases. The tumor in the mouth can appear as a soft, smooth, or lobulated mass with or without a base and varying in size from a few millimeters to a few centimeters. This article reports a case of hemangioma with unusual clinical features in a 26‐year‐old male patient. [ABSTRACT FROM AUTHOR]

Background: Next‐generation sequencing has greatly increased our understanding of vascular birthmarks. Many port‐wine birthmarks are due to somatic mutations in GNAQ/GNA11 exon 183, but other genomic causes have been identified. Most congenital hemangiomas are due to somatic mutations in GNAQ/GNA11 at exon 209. Although genomically distinct, clinical overlap of congenital hemangiomas and port‐wine birthmarks has occasionally been described. Objective: We report a case series of a unique segmentally distributed vascular anomaly with overlapping characteristics of port‐wine birthmarks and congenital hemangiomas with other distinctive features including ulceration, atrophy, and scarring. Methods: This was a multicenter study with retrospective identification of patients via a detailed review of medical records. We also reviewed previously published cases. Results: The clinical, histological, radiological, and genomic characteristics of 19 new and 13 previously reported cases characterized by segmental distribution, sharply demarcated borders, with variable thickening are presented. All cases had central atrophy with or without episodic ulceration. Those with genomic studies (13 out of 32) had somatic activating missense mutations in GNA11 or GNAQ codon 209. Conclusions: We describe the features and propose a descriptive name segmental congenital vascular anomaly with atrophy, ulceration, and scarring (SeCVAUS) for this condition. [ABSTRACT FROM AUTHOR]

Pediatric dermatologists are frequently consulted to evaluate children for cutaneous signs of systemic disorders. Numerical thresholds of significance have been described in the dermatologic literature for various skin findings where the likelihood of an associated extracutaneous abnormality or known genetic syndrome increases significantly. Knowledge of these numerical thresholds facilitates diagnosis and management, which improves clinical outcomes and avoids severe complications. This review highlights the clinical presentation, complications, evaluation, and numerical significance, when applicable, for the following skin findings: infantile hemangiomas, capillary malformations, café‐au‐lait macules, hypopigmented macules, juvenile xanthogranulomas, pilomatricomas, and angiofibromas. [ABSTRACT FROM AUTHOR]

Background: Infantile hemangiomas (IHs), the most prevalent vascular tumors in infancy, are generally understood to be absent at birth, appearing in the initial weeks of life during their proliferative stage. While the classic presentation is recognizable, the precursor lesion of IHs may be misinterpreted as other entities, including vascular malformations. Methods: A retrospective, single‐center study was conducted, examining neonates with photographed IH precursor lesions on day of life (DOL) 1 and matured classical IHs. The study spanned from 2017 to 2023. Results: The case series is comprised of nine neonates all exhibiting precursor lesions on DOL 1. A comparative display of photographs featuring precursor lesions and classic IH is presented. Further tabulated information for each case includes IH locations, subsequent treatment modalities, and further diagnostic workup if necessary. Conclusions: Improving recognition of precursor lesions increases diagnostic accuracy, decreasing unnecessary workup. This, in turn, allows dermatologists to confidently employ close follow‐up management strategies. Additionally, in cases of extensive involvement, recognition of the precursor lesion allows for expedited investigation for syndromes such as PHACE (posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects, and eye abnormalities) and LUMBAR (lower body IH, urogential anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies). [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: Uveal melanoma (UM) is an aggressive cancer emerging from mutations in the GNAQ and GNA11 genes. These mutations are also linked to other conditions with distinct morphological features (skin capillary malformations, hemangiomas, Sturge–Weber syndrome, choroidal nevi, blue nevi, hepatic small vessel neoplasm, Blitz nevi, iris and ciliary body melanocytoma, primary central nervous system melanocytic neoplasms, and aldosterone-producing adenomas). By examining the molecular pathways influenced by these mutations, we provide insights into potential targeted therapies for UM and related disorders. Additionally, we address the involvement of SOX10-positive perivascular cells in the complex pathophysiology of GNAQ/GNA11-related conditions. Uveal melanoma (UM), recognized as the most prevalent primary intraocular malignancy in adults, is primarily driven by mutations in the GNAQ and GNA11 genes. These genetic alterations are also implicated in other conditions, which exhibit distinct morphological characteristics. In this article, we investigate the role of GNAQ and GNA11 mutations across varied disorders (e.g., UM, skin blue nevi, and hemangiomas), emphasizing the shared pathogenic mechanisms that connect them despite their differing clinical manifestations. By investigating the molecular pathways affected by these mutations, we provide insights into the potential for targeted therapies that could address not only UM but also other disorders associated with GNAQ/GNA11 mutations. Moreover, we discuss the role of SOX10-positive perivascular cells that may be implicated in the complex pathophysiology of GNAQ/GNA11-related entities. Understanding the common molecular foundation of these conditions opens new ways for research and treatment opportunities, potentially leading to more effective, personalized therapeutic strategies. [ABSTRACT FROM AUTHOR]

A 6-week-old boy is brought to the hospital for fussiness and abdominal distension. He was febrile on presentation and was admitted to the hospital for further evaluation. On subsequent examinations, he continued to demonstrate abdominal distension and tenderness to palpation. Ultrasonography of the abdomen was performed and revealed a heterogeneous liver mass. With further diagnostics, a diagnosis was made and treatment initiated, with the infant experiencing resolution of his symptoms. Our panel of experts first discuss the management of an infant with abdominal distension, then discuss the evaluation of a liver mass in an infant, including oncologic, vascular, and infectious etiologies. [ABSTRACT FROM AUTHOR]

The article presents a case study of a healthy female newborn, born at 40 weeks and 3 days to a 33-year-old mother, who was diagnosed with a congenital hemangioma presenting as a right-sided lateral neck mass. Topics discussed include the differential diagnosis of congenital lateral neck masses, the characteristics and progression of congenital and infantile hemangiomas, and the importance of using ultrasound with Doppler for proper imaging and assessment of such lesions.

Background: Preferentially expressed antigen in melanoma (PRAME) has been extensively studied in cutaneous melanocytic tumors and has proven valuable as a diagnostic adjunct in routine dermatopathology practice. However, its expression in cutaneous vascular neoplasms, particularly angiosarcomas (AS), remains largely unexplored. Methods: To further explore PRAME expression in cutaneous AS, 18 cases of post‐irradiation and 13 cases of primary cutaneous AS were evaluated for PRAME. For comparison, sections from 11 deep soft tissue/visceral AS, 10 Kaposi sarcomas, 8 microvenular hemangiomas, 7 infantile hemangiomas, 8 atypical vascular lesions, 6 epithelioid hemangioendotheliomas, 6 pyogenic granulomas, 6 papillary endothelial hyperplasias, 6 epithelioid hemangiomas, 3 capillovenous malformations, 3 hobnail hemangiomas, 2 spindle cell hemangiomas, 2 pseudomyogenic hemangioendotheliomas, and 2 composite hemangioendotheliomas were also retrieved. Results: Overall, 22 of 31 (70.9%; 12 post‐irradiation and 10 primary) cutaneous AS were positive for PRAME. In contrast, only 1 of 11 (9.1%) deep soft tissue/visceral AS showed diffuse and strong PRAME nuclear staining. All other tumor types were negative for PRAME, except for 5 of 7 (71.4%) infantile hemangiomas, which demonstrated rare (<5%; four cases) and 1+ (5–25%; one case) nuclear staining. Conclusions: In this study, we have demonstrated frequent nuclear PRAME expression in cutaneous AS. PRAME immunohistochemistry may serve as a valuable additional marker in selected clinical settings. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety, efficacy, and long-term outcomes of transarterial chemoembolization (TACE) with bleomycin-Lipiodol for giant liver hemangiomas. Materials and Methods: Single-center retrospective study from 1998 to January 2020, including patients with giant liver hemangiomas treated with bleomycin-Lipiodol TACE and followed up >36 months. The exclusion criteria were defined as patients who had been treated but had no available follow-up above 3 years and patients who had previously been treated with any other treatment method. Clinical success was defined as the disappearance of symptoms and radiological success (responded vs. non-responded groups) as a more than 50% decrease in the volume of the giant hemangioma in follow-up CT or MRI compared to the baseline images. Results: A total of 121 patients were included. The mean maximum diameter of the hemangiomas decreased from 122 (range: 40–300) to 73 mm (range: 15–240), and the mean volume reduced from 984.4 (range: 30–7312) to 286.6 cm3 (range: 1–3835). There were 106 patients in the responded group, while only 15 patients were in the non-responded group. No significant difference was found in size and volume change percentages across these two groups based on gender, age, lesion size, lesion volume, lesion number, and second TACE. When the follow-up period was stratified in 5-year periods, the maximum volume decrease was observed in the first 5-year period and then remained constant up to > 15 years. Conclusion: TACE with bleomycin-Lipiodol is safe, reducing the size and volume of giant liver hemangiomas with stable results in the long-term follow-up. [ABSTRACT FROM AUTHOR]

Infantile haemangiomas (IHs) affect 3–10% of infants, 10% of whom need topical or systemic beta-blocker therapy. Propranolol is the first choice for IHs with a high risk of complications. Since more than half of IHs leave a permanent mark, to reduce outcomes, it is essential to start oral propranolol (2–3 mg/kg/day in 2 doses/day) within the 5th month of life (i.e., during the proliferative phase) and to complete the therapy cycle for at least 6 months. This review aims to summarise the epidemiology, clinical presentation, diagnosis, and treatment of IHs and to highlight the importance of proper referral to specialised hub centres. Patients with vascular anomalies, particularly those suspected of having IH, should be referred to a specialised centre for accurate diagnosis, management by a multidisciplinary team, and timely treatment. IHs may pose life-threatening, functional, and aesthetic risks or may ulcerate. Segmental infantile haemangioma of the face/neck and the lumbosacral regions can be associated with various malformations. To ensure timely specialist evaluation and treatment to reduce the potential risk of complications, it is essential to identify high-risk IHs rapidly. The Infantile Haemangioma Referral Score (IHReS) scale is an important tool to assist primary care paediatricians and general dermatologists. [ABSTRACT FROM AUTHOR]

Background: Intramuscular hemangioma is a vascular malformation occurring in muscle tissues. It is most common in skeletal muscles of limbs, especially lower limbs in childhood. The intercostal intramuscular hemangioma with sternal invasion is very rare. Case presentation: The 47-year-old female patient was hospitalized due to "suddenly chest pain for 4 days". Chest contrast-enhanced CT indicated bony changes in the sternum, accompanied by soft tissue shadows in the posterior sternum and diaphragmatic angle. After admission, physical examination showed: peristernal tenderness, percussion pain, other no obvious positive signs. PET-CT examination indicated that there was a high possibility of benign sternal changes. No cancer cells were found by sternal puncture. Surgical biopsy was performed and pathological findings were consistent with intramuscular hemangioma. Conclusion: Intramuscular hemangioma with bone invasion is very rare, imaging examination is difficult to indicate obvious evidence, preoperative diagnosis is difficult, complete surgical resection is very important, more personalized treatment should be provided according to the overall situation of the patient. [ABSTRACT FROM AUTHOR]

Vascular anomalies can be broadly classified as vascular tumors or vascular malformations, depending on the degree of endothelial involvement. These lesions can be subtle and challenging to diagnose in individuals with darker skin tones, which can lead to a delay in treatment. Improving diagnostic accuracy of these lesions may prevent progression and potential adverse outcomes when left untreated. Here, we present a review of common vascular anomalies with a focus on diagnostic considerations in individuals with skin of color. [ABSTRACT FROM AUTHOR]

Venous hemangiomas within the central nervous system (CNS) represent a rare pathological entity described by sporadic case reports so far. Comprehensive insights into their histological and imaging features, pathogenesis, natural course, and therapeutic modalities are lacking. This review article presents two patients with contrast-enhancing cerebellar lesions near the tentorium cerebelli lacking edema or diffusion restriction. Despite meticulous preoperative neuroradiological examination, diagnostic classification remained inconclusive. Confronted with both—progressive size and diagnostic uncertainty—surgical intervention was undertaken, resulting in uneventful and complete resection of the lesions. Histopathological analyses subsequently revealed a venous hemangioma in each case. In the literature, the term "hemangioma" is often misapplied and inaccurately used to describe a broad spectrum of vascular anomalies. Therefore, a precise identification is essential since the particular type of vascular anomaly affects its natural course and the treatment options available. We aim to contribute to the understanding of this diagnostically intricate entity by presenting the two cases and by providing a detailed overview of radiological and histopathological features of venous hemangiomas. [ABSTRACT FROM AUTHOR]

Background Confident diagnosis of hepatic hemangioma on imaging can avoid biopsy in early infancy and helps guide conservative management. Purpose This article aims to determine if dynamic contrast-enhanced magnetic resonance imaging (MRI) can be used to differentiate liver hemangioma from other lesions in infants ≤ 100 days and to determine association of MRI features with hepatic lesions. Methods MRI performed for liver lesions were retrospectively reviewed to note imaging characteristics and the MRI diagnosis. Final diagnosis was assigned based on pathology in available cases and by corroborative standard of reference including overall clinical features, lab findings, and follow-up. Results Of 30 infants (18 boys, 12 girls; average age 42.2 days) included, 18 had solitary and 12 had multifocal lesions. Diagnoses in total 33 lesions included hemangiomas (23), hepatoblastoma (6), arteriovenous malformation (2), neuroblastoma metastases (1), and infarction (1). MRI and final diagnosis matched in 94% lesions with almost perfect agreement (kappa 0.86) for reader 1, and matched in 88% lesions with substantial agreement (kappa 0.71) for reader 2. Interobserver agreement for MRI diagnosis was substantial (kappa 0.62). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of MRI in differentiating hemangioma from other lesions were 100, 90, 96, 100, and 97%, respectively. Centripetal (16/23) or flash (5/23) filling were only seen with hemangioma. There was no significant difference in alpha-fetoprotein elevation (p 0.08), average size (p 0.35), multifocality (p 0.38), and intralesional hemorrhage (p 1) between hemangioma and hepatoblastoma. Conclusion Centripetal filling on dynamic imaging and absence of washout are characteristic MRI features of hepatic hemangioma that can help to differentiate it from other lesions in early infancy. [ABSTRACT FROM AUTHOR]

In this case report, we describe a 21-year-old man with recurrent hemangiomas in his left eye socket and nasal cavity. Traditional surgeries were unsuccessful, so we used Bevacizumab, a drug that inhibits blood vessel growth. This approach significantly reduced the tumor size and stopped frequent nosebleeds. Over two years, the tumor remained controlled without major side effects, suggesting Bevacizumab as a promising non-surgical treatment for recurrent hemangiomas. [ABSTRACT FROM AUTHOR]

This narrative review delves into the intricacies of hemangiomas and vascular malformations (VMs), exploring their characteristics, diagnostic approaches, and therapeutic interventions. Hemangiomas, characterized by an abnormal growth of blood vessels, and VMs, arising from developmental abnormalities in blood vessels, present multifaceted challenges in clinical practice. The review navigates through the clinical manifestations and classifications of these vascular anomalies, emphasizing the importance of accurate diagnosis based on clinical evaluation, imaging techniques, and histopathological assessment. Variations in presentation, ranging from asymptomatic lesions to severe complications impacting vital organs, underscore the necessity for comprehensive understanding and tailored management strategies. Moreover, this review examines current therapeutic modalities encompassing medical, interventional, and surgical interventions. It synthesizes the evolving landscape of treatment options and highlighting the benefits, limitations, and potential adverse effects associated with each approach. In addition, the review underscores the significance of a multidisciplinary approach, integrating expertise from dermatology, radiology, and surgery, to optimize patient care and outcomes. The challenges posed by these vascular anomalies necessitate ongoing research endeavors aimed at refining diagnostic criteria and advancing therapeutic innovations. This review amalgamates existing knowledge and providing clinicians and researchers with a comprehensive understanding of hemangiomas and VMs, thereby fostering continued exploration and innovation in this complex field.

Focal liver lesions are frequently encountered during imaging studies, and hemangiomas represent the most common solid liver lesion. Liver hemangiomas usually show characteristic imaging features that enable characterization without the need for biopsy or follow-up. On the other hand, there are many benign and malignant liver lesions that may show one or more imaging features resembling hemangiomas that radiologists must be aware of. In this article we will review the typical imaging features of liver hemangiomas and will show a series of potential liver hemangiomas' mimickers, giving radiologists some hints for improving differential diagnoses. Critical relevance statement: The knowledge of imaging features of potential liver hemangiomas mimickers is fundamental to avoid misinterpretation. Key Points: Liver hemangiomas typically show imaging features that enable avoiding a biopsy. Many benign and malignant liver lesions show imaging features resembling hemangiomas. Radiologists must know the potentially misleading imaging features of hemangiomas' mimickers. [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.

Objective: To explore the diagnostic value of real-time shear wave elastography (SWE) in hepatic hemangioma (ANGI) and hepatocellular carcinoma (HCC). Methods: A total of 88 patients diagnosed with angiosarcoma (ANGI) or hepatocellular carcinoma (HCC) in our department from February 2019 to February 2020 were included in this study. These patients were assigned to two groups based on pathological diagnosis: the ANGI group (n = 42) and the HCC group (n = 46). All patients underwent SWE examination, and the clinical efficacy was analyzed by comparing the average value of Young's modulus of the two groups and plotting the receiver operator characteristic (ROC) curve. Results: There was no significant difference in lesion diameter between ANGI and HCC patients (P > 0.05). The ANGI group exhibited a significantly higher average Young's modulus for both the lesions and the adjacent liver tissues compared to the HCC group (P < 0.001); The area under the curve (AUC) of the average value of Young’s modulus of the lesion to ANGI and HCC was 0.875 (95% CI = 0.795-0.955). Conclusion: SWE demonstrates high diagnostic accuracy in distinguishing ANGI from HCC, providing valuable clinical evidence for differentiating between the two diseases. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioma (EH) is an uncommon, benign vascular tumor of mesenchymal origin. It mainly presents as a tumor with a lytic appearance and septations. However, no case reports have documented the predominantly sclerotic appearance of EH. Here, we present the imaging findings, including X-ray, CT, MRI, and histopathological findings of a 24-year-old female with EH. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 38-week term newborn with a giant maxillary mass, highlighting the challenges of diagnosing and managing congenital epulis. Topics discussed include the rarity and benign nature of oral tumors in neonates, the importance of a multidisciplinary approach for surgical intervention, and the necessity for careful histopathological evaluation due to the potential for misdiagnosis.

While ulceration is one of the most common infantile hemangioma (IH) complications, severe bleeding is a rare consequence, with a paucity of patients reported. We report a 5‐month‐old girl with a very large, mixed, partial segmental IH of the upper chest wall who, despite medical intervention, developed severe ulceration and multiple episodes of life‐threatening bleeding that ultimately led to hemorrhagic shock. Experience in our patient and a review of six previous reports shows that severe bleeding is a risk when ulceration extends directly into an arterial feeding vessel that is often visible clinically. Other potential predictors for severe bleeding include large to very large IH size with extension of the tumor into underlying structures, segmental or partial segmental patterning, mixed and bulky morphology, and white discoloration as a sign of impending or worsening ulceration. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Ovarian hemangioma, though rare and asymptomatic, can mimic malignant ovarian tumors, thus it is necessary for comprehensive histopathological and immunohistochemical evaluation for accurate diagnosis and appropriate management. [ABSTRACT FROM AUTHOR]

Background: Cavernous hemangioma of the small intestine is rarein adults. Obscure Overt Gastrointestinal bleeding (OOGIB) caused by smallbowel hemangioma is extremely rare. According to the size of the affected vessels, hemangiomas are histologically classified into cavernous, capillary, and mixed-type tumors, with the cavernous type being the most common and racemose being very rare in the clinic.Owing to the rarity of gastrointestinal hemangiomas and the lack of specific manifestations and diagnostic methodsthe source of GI bleeding could not be initially identified despite the use of numerous diagnostic modalities. Case Summary: We report the case of a 40-year-old female presenting with painless melena and postprandial bloating. Laboratory investigations revealed recurrent and prominent anemia. DBE (Double-balloon endoscopy) revealed subepithelial lesion in the distal jejunum. Segmental resection of the lesion in the jejunum was performed, and cavernous hemangioma was diagnosed based on histopathological analysis. Segmental resection of the ileal lesion was performed surgically, and the final pathologicalresults revealed a diagnosis of cavernous hemangioma. At present the patient is symptom free and doing fine. Conclusion: The current report will increase our understanding of the diagnosis and treatment of gastrointestinal hemangiomas and provide a review of the related literature. [ABSTRACT FROM AUTHOR]

Intra-osseous hemangiomas are uncommon tumors that can present diagnostic and treatment dilemmas. Bone hemangiomas with papillary and glomeruloid growth patterns are exceptionally rare. We present an example of an intra-osseous hemangioma of the rib displaying aggressive features on both radiology and histology. Morphologically, prominent papillary and glomeruloid architectural patterns were observed, in addition to features of cavernous and capillary hemangiomas. Extensive extra-osseous soft tissue involvement was seen. Awareness of the diverse histological features and locally aggressive behavior of bone hemangiomas is important in avoiding over-interpretation as a malignant lesion. [ABSTRACT FROM AUTHOR]

Anastomosing hemangioma of the ovary is a rare vascular tumor that predominantly affects middle-aged women. Despite its benign nature, its histological appearance can mimic aggressive vascular lesions, posing diagnostic challenges. This review aims to provide an overview of this uncommon entity. The PubMed and Scopus databases were searched for relevant articles published in English. Information on all retrieved cases was extracted and reviewed in detail. We found 33 cases with relevant details of anastomosing heamangioma of the ovary. Despite the small number of cases we found, our study demonstrated the importance of an accurate hystopathological evaluation. Although the preliminary imaging and initial microscopic features may appear alarming, careful microscopic examination reveals benign behavior. There is a need to raise awareness of this unusual and rare entity to improve morphologic recognition and avoid misdiagnosis that could lead to unnecessary treatment or patient anxiety. [ABSTRACT FROM AUTHOR]

Purpose: Superficial Infantile hemangioma (SIH) is the most common type of IH. Some studies have shown the efficacy of 755-nm long pulse alexandrite laser (LPAL) and topical 2% carteolol hydrochloride (C-HCL) eye drops for the treatment of SIH. This article retrospectively analyzes the safety and efficacy of 755-nm LPAL combined with 2% C-HCL eye drops for treating thicker SIH, and explores the optimal treatment time for SIH. Materials and Methods: This study included 2– 5 mm thick SIH patients who received co-treatment of 755-nm LPAL and 2% C-HCL eye drops. The SIH patients were divided into 3 groups based on their age and IH growth curve: ≤ 1 month (≤ 1M), 1– 3 months (excluding 1 month; 1– 3M), and 3– 12 months (excluding 3 months; 3− 12M). Results: There was no difference in efficacy between the ≤ 1M and the 1– 3M group, and were both better than the 3– 12M group. Furthermore, there was no difference in the average number of treatments between the ≤ 1M and 1– 3M groups and were both less than the 3– 12M group. There was no significant difference in the incidence of adverse reactions between the groups. Compared with the ≤ 1M and 1– 3M groups, the 3– 12M group indicated more permanent skin lesions after the treatment. Conclusion: It was revealed that co-treatment with 755-nm LPAL and 2% C-HCL eye drops is safe and effective against thicker SIH. Compared with the 3– 12M group, ≤ 3 months can achieve better efficacy, requires a shorter treatment time, less likely to leave permanent skin lesions such as scars. Moreover, patients with no proliferation can be observed to 1 month. [ABSTRACT FROM AUTHOR]

Background: Infantile haemangiomas (IHs) sometimes require treatment with propranolol. Sleep disturbances are the most frequently reported side effects. Monitoring adverse drug events necessitates repeated hospital visits, which can be challenging during a pandemic. Objectives: To explore the effectiveness of a new electronic questionnaire in identifying sleep disturbances related to treatment with propranolol and potential confounding factors. To evaluate the response rate to the questionnaire. To report the proportion of patients on propranolol with sleep disturbances. Methods: In an observational, prospective cohort study, caregivers provided clinical information during ambulatory visits and via an electronic questionnaire after an 8-week treatment course with propranolol and at the time of treatment interruption. Adverse drug reaction reporting forms were assessed for causality. Results: The questionnaire response rate was 91%, and the completion rate was 100%. A total of 59% of patients experienced sleep disturbances during propranolol treatment, which were considered adverse reactions. Sleep disorders were frequent during sleep regression phases and in subjects who fell asleep during physical contact with caregivers or bed-sharing with parents. Conclusion: The application of this questionnaire allows for identifying adverse sleep events associated with propranolol in IHs and potential confounders. Counselling on sleep hygiene is recommended before treatment onset. [ABSTRACT FROM AUTHOR]

Simple Summary: This review paper deals with incidentally found focal liver lesions (IFLLs) in otherwise healthy subjects, which is a frequent occurrence in daily practice. The clinical presentation and the imaging aspects play an important role in deciding whether and what further evaluation is required. In low-risk patients (i.e., those without a history of malignant or chronic liver disease or related symptoms, younger than 40 years old), more than 95% of IFLLs are benign. Shear Wave liver Elastography of the surrounding liver parenchyma should be considered to exclude liver cirrhosis and for further risk stratification. If an IFLL in a low-risk patient has a typical appearance on a B-mode ultrasound of a benign lesion, no further imaging is needed. Contrast-Enhanced Ultrasound (CEUS) should be considered as the first-line contrast imaging modality to differentiate benign from malignant IFLLs. In high-risk patients (i.e., with chronic liver disease or an oncological history), each IFLL should initially be considered as potentially malignant, and every effort should be made to confirm or exclude malignancy. An incidental focal liver lesion (IFLL) is defined as a hepatic lesion identified in a patient imaged for an unrelated reason. They are frequently encountered in daily practice, sometimes leading to unnecessary, invasive and potentially harmful follow-up investigations. The clinical presentation and the imaging aspects play an important role in deciding if, and what further evaluation, is needed. In low-risk patients (i.e., without a history of malignant or chronic liver disease or related symptoms), especially in those younger than 40 years old, more than 95% of IFLLs are likely benign. Shear Wave liver Elastography (SWE) of the surrounding liver parenchyma should be considered to exclude liver cirrhosis and for further risk stratification. If an IFLL in a low-risk patient has a typical appearance on B-mode ultrasound of a benign lesion (e.g., simple cyst, calcification, focal fatty change, typical hemangioma), no further imaging is needed. Contrast-Enhanced Ultrasound (CEUS) should be considered as the first-line contrast imaging modality to differentiate benign from malignant IFLLs, since it has a similar accuracy to contrast-enhanced (CE)-MRI. On CEUS, hypoenhancement of a lesion in the late vascular phase is characteristic for malignancy. CE-CT should be avoided for characterizing probable benign FLL and reserved for staging once a lesion is proven malignant. In high-risk patients (i.e., with chronic liver disease or an oncological history), each IFLL should initially be considered as potentially malignant, and every effort should be made to confirm or exclude malignancy. US-guided biopsy should be considered in those with unresectable malignant lesions, particularly if the diagnosis remains unclear, or when a specific tissue diagnosis is needed. [ABSTRACT FROM AUTHOR]

To explore the advantages and disadvantages of 3D Slicer reconstruction and 3D printing localization combined with transcranial neuroendoscope in the surgical treatment of deep cerebral micro cavernous hemangiomas. Method The clinical data of patients with deep cerebral micro cavernous hemangiomas treated by our hospital from June 2022 to February 2023 using 3D Slicer reconstruction and 3D printing localization technology combined with transcranial endoscopic surgery were retrospectively analyzed. A total of 5 cases with complete data were collected, including 2 males and 3 females, aged 9–59 years. All 5 patients had deep supratentorial cavernous hemangiomas with a diameter of less than 1.5 cm, and had clinical symptoms such as headache or epilepsy, and had been diagnosed by CT or MRI. Repeated bleeding from small cavernous hemangiomas in the deep brain can lead to clinical symptoms such as recurrent headache and epilepsy, and is required surgical treatment. However, cavernous hemangiomas often have smaller lesions and are difficult to locate in the deep part. Without neuronavigation, surgery can become extremely difficult. Our team's newly developed 3D Slicer reconstruction and 3D printing localization technology which could provide new options for surgical treatment of small cavernous hemangiomas or other small lesions in the deep brain, but its accuracy and safety still need to be verified by further clinical research. [ABSTRACT FROM AUTHOR]

Introduction: Intrauterine transfusion is the treatment for fetal anemia resulting from maternal alloimmunization, infections (parvovirus B19 and cytomegalovirus), single demise of a monochorionic twin, chorioangioma, and other rare conditions. Fetal analgesia is mandatory to reduce movement and pain perception during the procedure. This study aims to evaluate perinatal outcomes for such procedures, following the routine use of fetal analgesia in our clinical practice. Materials and methods: Retrospective analysis of cases from 2009 to 2022, including all confirmed fetal anemia with fetal blood sampling. After fetal analgesia, Rh-negative concentrated red blood cells were transfused, with ultrasonographic follow-up 24 h and 1 week later. In case of suspected brain lesion, magnetic resonance imaging was performed. Elective delivery was considered in case of persistent anemia after 34 weeks. Post-natal follow-up and comprehensive obstetric and perinatal outcomes data were collected. Results: Altogether 59 anemic fetuses were included, with 34 (57.6%) being hydropic. The causes of anemia were maternal alloimmunization (22, 37.3%), infections (13, 22%), monochorionicity (10, 16.9%), rare conditions (9, 15.3%), and two chorioangiomas (3.4%). The median gestational age at the procedure was 25.2 weeks (18-32 weeks), with no related preterm premature rupture of membranes (<48 h), or side effects from fetal analgesia. Gestational age at delivery was 33weeks (26-41 weeks), with survival rate of 90%. There were four fetal demises, two termination of pregnancies, and eight neonatal deaths due to persistent severe anemia after preterm delivery. The main contributors to adverse outcome were the type of anemia, and the management with a preterm delivery. Conclusion: Intrauterine transfusion of red blood cells under analgesia is safe, with low incidence of obstetric complication. [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]

Background: Photodynamic therapy (PDT) is a minimally invasive therapy that was gradually established as a first-line treatment for vascular abnormalities. Its action depends on the appropriate wavelength of light and photosensitizer to produce toxic oxygen species and cause cell death. Objective: Several new clinical improvements and trends in PDT have been described in recent years. The aim of this review is to provide an overview of the current data from clinical trials. Methods: In this review, we introduce and generalize the wavelength, duration, dose, strength, and photosensitizer of PDT for the treatment of vascular abnormalities, such as circumscribed choroidal hemangiomas (CCH), choroidal neovascularization (CNV) and capillary malformation (CM). Results: The systematic review findings indicate that the application of PDT is a safe effective method to treat CCH, CNV and CM. However, PDT also has early onset side effects and late onset side effects. Conclusions: Based on the discussion of the effectiveness of PDT, we conclude that PDT has great potential for clinical use, although PDT has possible side effects. [ABSTRACT FROM AUTHOR]

Buccal cavernous hemangiomas are uncommon vascular lesions, particularly in adult patients. We present a case of a 23-year-old male with a progressive left cheek swelling over three years. Clinical examination and radiographic imaging revealed a solid, multilobulated mass in the left buccal and masticator spaces. Surgical excision was performed, and histopathological analysis confirmed the diagnosis of a cavernous hemangioma. This case underscores the importance of recognizing and appropriately managing rare vascular lesions in adult patients. [ABSTRACT FROM AUTHOR]

Simple Summary: Patients with chronic liver disease and cirrhosis are at risk of developing liver cancer (HCC). Regular ultrasound screening for HCC is recommended for these patients so that HCC can be found early. However, ultrasound is not always effective at picking up small cancers, especially in patients who are overweight or obese. Other tests including CT and MRI are expensive. Our study looked at a shorter version of an MRI scan (abbreviated MRI, or aMRI) in thirty patients who had recently had an ultrasound with poor views of the liver. All thirty patients tolerated the aMRI scan well. In these patients, the aMRI scan found one HCC and five other liver abnormalities which had not been picked up on ultrasound. Experts evaluated the aMRI scans and felt they were of good quality. Our study shows that aMRI is possible and useful in patients undergoing screening for HCC, especially those who have had poor views on an ultrasound. Background: Biannual ultrasound (US) is recommended for hepatocellular carcinoma (HCC) surveillance in patients with cirrhosis. However, US has limited sensitivity for early-stage HCC, particularly in overweight cohorts, where hepatic visualisation is often inadequate. Currently there are no robust imaging surveillance strategies in patients with inadequate US visualisation. We investigated the ability of non-contrast, abbreviated magnetic resonance imaging (aMRI) to adequately visualise the liver for HCC surveillance in patients with previously inadequate US. Methods: Patients undergoing US surveillance, where liver visualisation was inadequate (LI-RADS VIS-B and VIS-C), were prospectively recruited. Patients underwent non-contrast T2-weighted and diffusion-weighted aMRI. The images were reviewed and reported by an expert liver radiologist. Three independent, blinded radiologists assessed the aMRI visualisation quality using a binary score assessing five parameters (parenchymal definition, vascular definition, coverage of the liver, uniformity of liver appearance and signal-to-noise ratio). Results: Thirty patients completed the aMRI protocol. The majority (90%) had underlying cirrhosis and were overweight (93.3%), with 50% obese and 20% severely obese. A total of 93.3% of the aMRI scans were of satisfactory quality. Six patients (20%) had hepatic abnormalities detected with aMRI that were not seen on their US: one HCC, one haemangioma and three clinically insignificant lesions. For the aMRI visualisation quality assessment, the coverage of the liver, vascular definition and parenchymal definition were consistently rated to be of sufficient quality by all three radiologists. Conclusions: Non-contrast aMRI provided good visualisation of the liver and detection of abnormalities in patients with inadequate US. aMRI should be further explored in a larger, prospective study as an alternative surveillance strategy in patients with inadequate US. [ABSTRACT FROM AUTHOR]

An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences. [ABSTRACT FROM AUTHOR]

Recently, FN1 fusions to receptor tyrosine kinase genes have been identified in soft tissue tumors with calcified chondroid matrix named calcifying chondroid mesenchymal neoplasms (CCMNs). We collected 33 cases of CCMN from the French network for soft tissue and bone tumors. We performed wholeexome RNA sequencing, expression analysis, and genome-wide DNA methylation profiling in 33, 30, and 20 cases of CCMN compared with a control group of tumors, including noncalcified tenosynovial giant cell tumor (TGCT). Among them, 15 cases showed morphologic overlap with soft tissue chondroma, 8 cases with tophaceous pseudogout, and 10 cases with chondroid TGCT. RNA-sequencing revealed a fusion of FN1 in 76% of cases (25/33) with different 5' partners, including most frequently FGFR2 (14 cases), TEK or FGFR1. Among CCMN associated with FGFR1 fusions, 2 cases had overexpression of FGF23 without tumor-induced osteomalacia. Four CCMN had PDGFRA::USP8 fusions; 3 of which had histologic features of TGCT and were located in the hip, foot, and temporomandibular joint (TMJ). All cases with FN1::TEK fusion were located at TMJ and had histologic features of TGCT with or without chondroid matrix. They formed a distinct cluster on unsupervised clustering analyses based on whole transcriptome and genome-wide methylome data. Our study confirms the high prevalence of FN1 fusions in CCMN. In addition, through transcriptome and methylome analyses, we have identified a novel subgroup of tumors located at the TMJ, exhibiting TGCTlike features and FN1::TEK fusions. [ABSTRACT FROM AUTHOR]

Background: Synovial hemangiomas are rare benign vascular anomalies surrounded by a synovial lining and were first described by Bouchut in 1856. These neoplasms can develop in the intra-articular region, resulting in effusions and knee pain. However, their cause remains unknown. Prompt diagnosis and intervention are critical to prevent chondral damage. Histopathological examination is used to achieve the diagnosis, which is often delayed because of a lack of specific clinical signs. This report describes a unique case in which a painful infrapatellar mass was diagnosed as a synovial hemangioma. The absence of typical magnetic resonance imaging (MRI) findings highlights the importance of arthroscopic excision for diagnosis and symptom relief. Case presentation: A 20-year-old woman presented with persistent anterior left knee pain that became exacerbated when she climbed stairs. Despite previous pain management and physical therapy, she developed a painful lump beneath her patella that worsened over time. She had also undergone arthrocentesis, but this did not relieve her pain. Physical examination revealed a palpable, immobile 5-cm mass along the patellar tendon with limited knee flexion and extension and normal ligament stability. T1-weighted fat-saturated MRI of the left knee with gadolinium-based contrast revealed a lobulated intra-articular mass in Hoffa's fat pad that resembled a soft tissue chondroma. A biopsy of the mass was performed to provide histopathological evidence, confirming the benign nature of the mass. The subsequent excisional arthroscopy, combined with incision enlargement for mass removal, confirmed the histopathologic diagnosis of synovial hemangioma based on the presence of numerous dilated blood vessels and venous proliferation within sections of the synovium. Recovery was complete, and no residual tumor was detected on follow-up MRI after 1 year. Conclusion: This case study emphasizes the importance of arthroscopic excision over open surgery for patients with synovial hemangioma. The minimally invasive nature of arthroscopy combined with the well-encapsulated nature and location of the mass facilitates complete resection. [ABSTRACT FROM AUTHOR]

Simple Summary: This study analyzes 61 rare tumors and lesions mimicking peripheral nerve tumors using data from the German Peripheral Nerve Tumor Registry. These cases exhibit significant heterogeneity with various morphological features and biological potentials, including malignant peripheral nerve tumors, perineuriomas, and hybrid nerve sheath tumors. This study underscores the challenges in preoperatively differentiating these rare entities from benign tumors using clinical and radiological methods alone. It highlights the importance of a multidisciplinary approach involving radiologists, neurologists, neuropathologists, and neurosurgeons for accurate diagnosis and effective treatment. The supplementary material offers valuable insights to help clinicians recognize these rare tumors early, improving patient outcomes. The most common peripheral nerve tumors are of a benign nature and include schwannoma or neurofibroma. In rare cases, other tumors or non-tumorous lesions can mimic peripheral nerve tumors clinically or radiologically. Based on data from the multicentric German Peripheral Nerve Tumor Registry (PNTR), which encompasses current information on 315 surgically treated patients from three high-volume centers, we present 61 cases of rare tumors and lesions that mimic tumors associated with peripheral nerves. This cohort displays considerable heterogeneity, featuring a broad spectrum of morphological features and biological potentials. Histopathological diagnoses include various intrinsic peripheral nerve tumors such as malignant peripheral nerve tumors (MPNSTs) (n = 13), perineurioma (n = 17), and hybrid nerve sheath tumors (HPNSTs, comprising schwannoma/perineurioma and schwannoma/neurofibroma) (n = 14), as well as atypical neurofibromatous neoplasm with unknown biological potential (ANNUBP) (n = 1). Additionally, the cohort encompasses extrinsic tumorous lesions like lymphoma (n = 3), lymphangioma (n = 2), hemangioma (n = 2), solitary fibrous tumor (n = 2), metastatic disease (n = 1), and single cases of other rare tumor entities (n = 6). An overview of the underlying pathology, imaging features, and clinical presentation is provided, with a brief description of each entity. A definitive preoperative differentiation between benign peripheral nerve tumors and rare intrinsic and extrinsic tumors is often not possible. Clinical examination and subtle imaging clues can at least indicate the possibility of a rare entity. The basic requirement is close cooperation between radiologists, neurologists, neuropathologists, and neurosurgeons at a specialized center to develop a multidisciplinary concept and offer the patient the best therapeutic approaches. [ABSTRACT FROM AUTHOR]

Simple Summary: Epithelioid vascular tumors are rare bone vascular lesions with a spectrum of overlapping cancer entities with varying degrees of malignant potential that remain controversial because of their rarity, unusual morphologic features, and unpredictable biologic behavior. The classification of vascular tumors of bone proposed by the World Health Organization in 2020 incorporated morphologic findings with available genetic evidence, including mainly angiosarcoma, epithelioid hemangioma, pseudomyogenic hemangioendothelioma, and epithelioid hemangioendothelioma. Here, we report our experience with eleven patients suffering from epithelioid hemangioma (EH) of the spine. As very few cases of EH of the spine have been reported in the literature, the evidence-based decision-making process for these patients can be very difficult. Thus, we propose a treatment flowchart based on our single center's experience. Epithelioid hemangioma is recognized by the World Health Organization as a distinct benign neoplasm; however, it is characterized by locally aggressive and rarely metastasizing behavior. Epithelioid vascular tumors are rare bony vascular lesions with varying degrees of malignant potential that remain controversial because of their rarity, unusual morphological features, and unpredictable biological behavior. The application of new molecular tools, such as massive parallel sequencing technologies, have provided new diagnostic markers and an opportunity to further refine the classification of bone vascular neoplasms. Very few cases of EH of the spine have been reported in the literature; therefore, it is difficult to make evidence-based therapeutic decisions for these patients. We report herein our experience with eleven patients suffering from EH of the spine. The study population included three males and eight females treated in our center from 2016 to the present; the average age was 44.8 years (range 14–75 years). The surgical, clinical, and radiographic data were retrospectively analyzed. The mean follow-up was 34.8 months. All patients presented lytic vertebral body lesions, six of them with pathological fracture. The majority of patients (80%) presented myelo-radicular compression. All patients were surgically treated, and preoperative embolization was performed in all cases. In light of the literature review and the clinical experience of our center, we can consider EH a locally aggressive tumor that requires surgical treatment in case of symptoms. Here, we propose a treatment algorithm that could be useful in the management of patients with this rare disease. [ABSTRACT FROM AUTHOR]

Introduction: Intramuscular hemangiomas of extraocular muscles are extremely rare tumors that usually present as retro-orbital masses causing proptosis. We describe a previously unreported presentation, in the form of an epibulbar mass; this easily accessible location allows direct imaging, complete surgical resection, and histopathological confirmation, providing a unique perspective. Case Presentation: A 69-year-old woman presented with a painless dark red mass in the lateral part of the right eye, which had been slowly enlarging over the last 18 months. Clinical features and imaging were suggestive of a benign vascular tumor of the conjunctiva. During surgical resection, the mass was observed to be enmeshed within the fibers of the lateral rectus muscle. Careful dissection from muscle fibers was needed for complete excision. Histopathology revealed the diagnosis of an intramuscular hemangioma of extraocular muscle. Conclusion: In this report, we describe the atypical anterior epibulbar presentation of intramuscular hemangioma of the lateral rectus muscle. We discuss the differential diagnoses and management of this rare tumor along with a review of existing literature. Careful surgical resection achieved complete resolution in this case without recurrence. [ABSTRACT FROM AUTHOR]

Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus. Their removal poses significant risk due to their vascularity and diffuse nature, and their genetic underpinnings remain incompletely understood. Our approach involved genetic analyses on 31 tissue samples of ECHs employing whole-exome sequencing and targeted deep sequencing. We explored downstream signaling pathways, gene expression changes, and resultant phenotypic shifts induced by these mutations, both in vitro and in vivo. In our cohort, 77.4% of samples had somatic missense variants in GNA14 , GNAQ , or GJA4. Transcriptomic analysis highlighted significant pathway upregulation, with the GNAQ c.626A>G (p.Gln209Arg) mutation elevating PI3K-AKT-mTOR and angiogenesis-related pathways, while GNA14 c.614A>T (p.Gln205Leu) mutation led to MAPK and angiogenesis-related pathway upregulation. Using a mouse xenograft model, we observed enlarged vessels from these mutations. Additionally, we initiated rapamycin treatment in a 14-year-old individual harboring the GNAQ c.626A>G (p.Gln209Arg) variant, resulting in gradual regression of cutaneous cavernous hemangiomas and improved motor strength, with minimal side effects. Understanding these mutations and their pathways provides a foundation for developing therapies for ECHs resistant to current therapies. Indeed, the administration of rapamycin in an individual within this study highlights the promise of targeted treatments in treating these complex lesions. [Display omitted] Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus, and their genetic underpinnings remain incompletely understood. Here, we report somatic mutations of GNA14 , GNAQ , and GJA4 in ECH lesions. Furthermore, we present the utilization of rapamycin in treating an individual with GNAQ mutations. [ABSTRACT FROM AUTHOR]