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Vertebral hemangiomas (VHs) are relatively common, symptomatic benign tumors of the spine with a reported estimated incidence up to 11%. They usually appear in the body of the vertebrae; however, they can extend into pedicles, laminae, and epidural space. They may cause pain, neurologic deficits. and fractures. In this retrospective, single-center study, we reviewed our cases with VH and we propose the novel classification system that evaluates these lesions per their views on magnetic resonance imaging and clinical findings under 4 main categories. Our novel classification system proposes that grade I lesions occupy less than 50% of the vertebral body, whereas grade II lesions occupy more than 50% and grade III lesions occupy the whole corpus. Grade IV lesions show an epidural and pedicular extension. We propose that grade I lesions may not be worthwhile for follow-up, whereas asymptomatic grade II (a) lesions to be worthy for a biannual imaging and symptomatic thoracolumbar grade II (b) and thoracolumbar grade III lesions to be considered for percutaneous vertebroplasty. We imply that decompression, posterior spinal instrumentation, and open vertebroplasty may be performed for thoracolumbar grade IV lesions. We further consider cervical grade IIb, III, and grade IV lesions as operable because of the disadvantages of percutaneous vertebroplasty. We suggest that our novel classification system may be useful for the determination of diagnostic and therapeutic procedures in the management of VH. Further multicentric trials on larger series are warranted to validate this system and popularize its utility in larger populations. [ABSTRACT FROM AUTHOR]

Capillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with intramedullary extension rare. We present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of the literature. Medical records and imaging data were retrospectively reviewed using the health record software EPIC (Verona, Wisconsin, USA) and the radiology management software system RIS/PACS (Radiology Information System/Picture Archiving and Communication System; QREADS). The report was written in accordance with the CARE (case reports) guidelines. We also performed a systematic review of the literature on all cases of intramedullary spinal capillary hemangiomas in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. We report a case of a 54-year-old man who presented with progressive paraplegia and sensory deficits in the lower extremities. Spinal magnetic resonance imaging showed an intramedullary enhancing lesion centered at T11 with associated spinal cord compression. He underwent thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological examination. Histological examination showed findings consistent with a capillary hemangioma. The literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangioma. Purely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature. These should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients. [ABSTRACT FROM AUTHOR]

Percutaneous vertebroplasty (PVP) is a commonly used technique for the treatment of spinal diseases, but it is rarely employed for cervical lesions. This study presents a case series and a literature review to evaluate the efficacy of cervical PVP. From August 2013 to January 2023, 14 patients underwent cervical PVP in the author's institution. The mean postoperative follow-up time was 20.3 ± 12.1 months (ranging from 5 to 41 months). The pain status and quality of life were assessed preoperatively, postoperatively, and during follow-up using the Visual Analog Scale and Neck Disability Index. Additionally, complications that occurred during the study period were documented. The series of cases included 9 cases of hemangiomas and 5 cases of spinal metastases. The common symptom was axial pain in the neck. All patients were successfully treated with PVP. Visual analog scale scores decreased from 6.6 ± 0.8 preoperatively to 1.9 ± 0.8 at 24 hours postoperatively and to 2.4 ± 1.2 at the last follow-up (P < 0.01). Neck Disability Index decreased from 22.3% ± 8.9% preoperatively to 7.6% ± 8.1% at 24 hours postoperatively and to 6.0% ± 7.2% at 12-month follow-up (P < 0.01). After the operation, a case of dysphagia occurred, but no major complications were observed during the follow-up period. Cervical PVP via the anterolateral approach is a safe option for the treatment of cervical symptomatic hemangiomas and spinal metastases with limited invasiveness. It is effective in relieving pain and improving quality of life. [ABSTRACT FROM AUTHOR]

LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of infantile hemangioma and time to surgery. [ABSTRACT FROM AUTHOR]

Vertebral hemangiomas (VHs), formed from a vascular proliferation in bone marrow spaces limited by bone trabeculae, are the most common benign tumors of the spine. While most VHs remain clinically quiescent and often only require surveillance, rarely they may cause symptoms. They may exhibit active behaviors, including rapid proliferation, extending beyond the vertebral body, and invading the paravertebral and/or epidural space with possible compression of the spinal cord and/or nerve roots ("aggressive" VHs). An extensive list of treatment modalities is currently available, but the role of techniques such as embolization, radiotherapy, and vertebroplasty as adjuvants to surgery has not yet been elucidated. There exists a need to succinctly summarize the treatments and associated outcomes to guide VH treatment plans. In this review article, a single institution's experience in the management of symptomatic VHs is summarized along with a review of the available literature on their clinical presentation and management options, followed by a proposal of a management algorithm. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas (IHs) can be part of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies) syndrome when they are segmental, extensive, and located on the face or neck. The initial assessment is codified and well known, but there are no recommendations for the follow-up of these patients. The aim of this study was to assess the long-term prevalence of different associated abnormalities. Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated. After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities. Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up. [ABSTRACT FROM AUTHOR]

Infantile hepatic hemangioma (IHH) is the most common benign liver tumor in children, and multifocal and diffuse tumors often become life-threatening, necessitating therapy. Propranolol is now considered the first choice of therapy with ample data in Caucasian children. We present a series of nine Indian children with multifocal (n = 5) and diffuse (n = 4) IHH treated with propranolol monotherapy. This was a retrospective clinical data-based single-center study. Propranolol was used at a median dose of 3.2 mg/kg/day (range 3–3.3 mg/kg/day) for a median duration of 12 months (range 6–32 months). The presentations of IHH (either in isolation or combination) were hypothyroidism in six patients (diagnosed by elevated serum TSH levels), heart failure in three (diagnosed based on clinical and echocardiographic features), and imaging evidence of macrovascular shunting in two patients. A good response to propranolol monotherapy (with a median dose of 3.2 mg/kg/day for a median duration of 12 months) was observed in eight patients, with a poor response in one. One patient experienced recurrence but responded adequately to propranolol retreatment. Our data reiterate the excellent response (88.9% responded) and safety profile with propranolol monotherapy in complicated IHH and strengthen the data in Asian (Indian) children. It includes the maximum proportion of complicated IHH treated with propranolol in East and South Asia, and the largest series from India. [Display omitted] [ABSTRACT FROM AUTHOR]

Propranolol is the first-line treatment for infantile hemangiomas (IH). Cases of propranolol-resistant infantile hemangiomas are rarely reported. The purpose of our study was to investigate the predictive factors for poor response to propranolol. A prospective analytical study was conducted between January 2014 and January 2022 including all patients with IH who received oral propranolol therapy at a dose of 2–3 mg/kg/day maintained for at least 6 months. A total of 135 patients with IH were treated with oral propranolol. Poor response was reported in 18 (13.4%) of the patients: 72% were girls and 28% were boys. Overall, 84% of the IH were mixed, and hemangiomas were multiple in three cases (16%), nasal tip hemangiomas accounted for four cases (22%), and 15 patients (83%) had segmental hemangiomas. There was no significant association between the age or sex of the children and type of response to treatment (p > 0.05). No significant association was found between the type of hemangioma and the therapeutic outcome as well as the recurrence after treatment discontinuation (p > 0.05). Multivariate logistic regression analysis revealed that nasal tip hemangiomas, multiple hemangiomas, and segmental hemangiomas were at greater risk of poor response to beta-blockers (p < 0.05). Poor response to propranolol therapy has rarely been reported in the literature. In our series, it was approximately 13.4%. To our knowledge, no previous publications have focused on the predictive factors of poor response to beta-blockers. However, the reported risk factors for recurrence are discontinuation of treatment before 12 months of age, mixed or deep type IH, and female gender. In our study, the predictive factors for poor response were multiple type IH, segmental type IH, and location on the nasal tip. [ABSTRACT FROM AUTHOR]

Hereditary cerebral cavernous malformations (CCMs) are characterized by clustered dilated capillary-like vessels in the brain. Autosomal dominant polycystic kidney disease (PKD) is characterized by renal cysts and extra-renal abnormalities. We report a Taiwanese family in which the index case exhibited coexisting phenotypes of both CCMs and PKD. The index case was a 55-year-old woman with known PKD who developed an intracerebral hemorrhage (ICH) in the right medulla. Neuroimaging revealed numerous microbleeds in the bilateral cerebrum and cerebellum. Radiological CCMs were suspected given the absence of other imaging markers of small vessel disease. A comprehensive panel of 183 cerebral vascular malformation genes were investigated through genome sequencing. A novel CCM2 frameshift variant (c.607_608delCT, p.Leu203Valfs∗53) causing a pathogenic premature stop codon, and a known PKD2 nonsense variant (c.2407C > T, p.Arg803∗), were found. Segregation analysis revealed that four siblings were affected by either isolated aforementioned PKD2 or CCM2 variant. Notably, radiological CCMs were exclusively found in siblings who had this CCM2 variant, and bilateral internal carotid artery aneurysms were restricted to one sibling who had the PKD2 variant but not the CCM2 variant. Our study expands the genetic spectrum of CCM2 and demonstrates unambiguous cosegregation of CCM2 and PKD2 variants with their respective phenotypes. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas are vascular anomalies. However, they rarely cause genital bleeding. Here, we present the case of a young female with an endocavitary hemangioma who presented with abnormal uterine bleeding (AUB). The patient was an 8-year-old female with genital bleeding. Transabdominal pelvic ultrasound showed a 20-mm highly vascularized focal intrauterine endocavitary lesion. Vascular computerized tomography excluded vascular anomalies. Magnetic resonance imaging suggested a hemangioma. Minimally invasive open surgery was performed to remove the lesion. Subsequent pathology analyses confirmed an infantile/capillary hemangioma. Infantile hemangiomas are vascular anomalies that should be considered potential causes of AUB in early puberty. The study of these cases should include pelvic ultrasound and vascular magnetic resonance imaging. Experienced surgeons can successfully accomplish fertility-sparing surgical procedures. We describe an unusual case of peripubertal AUB caused by an endocavitary capillary hemangioma. Management included fertility-sparing surgery and the complete resolution of symptoms. [ABSTRACT FROM AUTHOR]

Spinal vertebral hemangiomas (SVHs) are the most common benign tumors of the spine. We performed a systematic review and meta-analysis of radiosurgery (RS) for SVHs. We reviewed articles published between January 1990 and December 2020 on PubMed. Tumor control, pain relief, and damage to surrounding tissues were evaluated with separate meta-analyses. This study was performed in accordance with the published Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A total of 23 patients with 24 SVHs were reported in 3 studies. Follow-up time was 7.3–84 months. The vast majority of lesions were located at dorsal level (n = 18; 75%). In 20 (83.3%) patients, pain was the initial clinical presentation. Complete, partial, and stable responses after radiation were reported in 45.7% (P < 0.001), 23.6% (P = 0.02), and 37.2% (P = 0.7) of cases. Overall response was reported in 94.1% (P = 0.7). No progressive disease was reported. Pain relief was achieved in 87.5% of patients (P = 0.2). Damage to surrounding tissue caused by irradiation was reported in 22.3% (P = 0.02) of cases in 1 study, in which higher doses of radiation were delivered. Radiosurgery is safe and effective for SVHs. Pain relief after RS in symptomatic patients was extremely high, while no progressive disease was reported. Damage to surrounding tissues was reported in only 1 series and included osteitis, osteonecrosis, or soft tissue injury after higher radiation doses. [ABSTRACT FROM AUTHOR]

Anastomosing hemangiomas represent a rare variant of benign vascular hemangioma which rarely arise from the liver parenchyma. The peculiarity of these tumors is that they can mimic malignant tumors, such as hepatocellular carcinoma, hepatoblastoma, and angiosarcoma. Notably, all documented cases where this type of liver tumor was described in the world's literature exclude cases involving infant patients. Our clinical case demonstrates the presence of an anastomosing hemangioma in the right lobe of the liver in a 5-month-old child, necessitating radical surgical intervention. According to ultrasound and CT scan findings, a tumor measuring 7.5 by 6 by 4.5 cm was detected in the segments V, VII and VIII of the liver. A differential diagnosis between hepatoblastoma and angiosarcoma was conducted. Blood tests, including alpha-fetoprotein levels, were within normal limits. Percutaneous biopsy revealed no cellular atypia, while immunohistochemical examination identified anastomosing hemangioma positive for CD-31 and CD-34 staining. Given the size of the tumor, an anatomical right hepatectomy was performed. The patient was discharged on the 7th postoperative day without surgical complications. After 6 months, no recurrence of the tumor was detected. This particular case highlights the importance of differential diagnosis of this type of tumor in children since anastomosing hemangiomas can mimic malignant liver tumors. [ABSTRACT FROM AUTHOR]

We present the case of an infant with rapidly progressing orbital tumor that had initial radiological and clinical features of both rhabdomyosarcoma and capillary hemangioma. The patient was eventually diagnosed with malignant rhabdoid tumor of the orbit. We discuss the salient histological and radiological features of our case and review the literature on orbital malignant rhabdoid tumors. [ABSTRACT FROM AUTHOR]

• Definitive radiotherapy could effectively treat choroidal hemangioma. • A low dose of 21.6 Gy was recommended in IMRT/VMAT. • Only 1.9 % of patients present disease progression after radiotherapy. To determine the efficacy of low-dose intensity-modulated radiation therapy (IMRT)/volumetric intensity-modulated arc therapy (VMAT) in the treatment of symptomatic choroidal hemangioma (CH). Fifty-three consecutive patients with CH were retrospectively reviewed. All the patients underwent IMRT/VMAT as a unique treatment. Resolution of subretinal fluid (SRF), improvement of best-corrected visual acuity (BCVA), and reduction in tumor thickness were compared before and after radiotherapy. After definitive radiotherapy, 100 % of SRF and 76.7 % of exudative retinal detachment were resolved. 56.6 % of BCVA improvement in more than two lines was observed. The mean best-corrected visual acuity was 20/280 (range, 20/1200–20/40) at diagnosis and 20/100 (range, 20/1200–20/20) after treatment. The mean tumor thickness decreased significantly from 3.8 mm initially to 1.2 mm after treatment (p < 0.01). 66.0 % of patients were delivered with 21.6 Gy (range, 21.6–42 Gy), 84.9 % of fractional dose was 1.8 Gy (range, 1.8–2 Gy). No radiation-induced keratitis, retinopathy, or optic neuropathy were observed. Initial vision (p = 0.042), duration time of vision (p = 0.004), and tumor thickness (p = 0.049) were prognostic factors for vision recovery. Low-dose IMRT/VMAT could effectively induce involution of the CH, with reduction of subretinal fluid and relief of damage to the neurosensory retina, which is an effective treatment mode for CH. [ABSTRACT FROM AUTHOR]

Adrenal hemangiomas are exceedingly rare benign tumors. Hemangiomas are commonly found in the skin and liver. When they arise in the adrenal gland, they are typically found incidentally on imaging, large size at presentation and are usually surgically resected in view of their large size and heterogeneous imaging appearance. We present a 66-year-old female who presented with right abdominal pain and weight loss and was found to have a large adrenal hematoma arising from a hemangioma. • Adrenal hemangiomas are very rare, non-functioning, benign tumors that are usually incidentally detected and large in size at diagnosis. • They have varied imaging appearances however imaging can help in the diagnosis and surgical planning especially in large tumors that infiltrate into adjacent structures particularly vascular structures. Pre-operative planning with imaging can reduce the risk of operative complications. • Surgical resection is recommended in large tumors more than 4 cm in size in view of the risk of bleeding and malignancy mimicry. • Patient age, hormonal symptoms, history of non-adrenal malignancy, and biochemical test results should be considered in imaging diagnosis. [ABSTRACT FROM AUTHOR]

Giant chorioangiomas are a potentially life-threatening condition that may require intrauterine therapy. We describe a case of a large chorioangioma (>4cm) diagnosed at 30 weeks of gestation causing severe fetal anemia and hydrops. An intrauterine blood transfusion was performed at 31 weeks with reversal of the anemia and hydrops. The neonate was born at 37 weeks showing respiratory distress syndrome that required neonatal intensive care unit admission but was discharged at 30 days of life. Further evaluation at two months of age showed no signs of abnormal neurodevelopment. When timely indicated, intrauterine transfusion of a hydropic fetus with anemia due to a giant chorioangioma is a potentially life-saving therapy that shows good neurodevelopment of the surviving fetus. [ABSTRACT FROM AUTHOR]

Vertebral hemangiomas are benign but highly vascular lesions and are one of the most common lesions of the vertebral column. Anterior soft-tissue compression of spinal cord due to vertebral body hemangioma is challenging to manage. Our objective was to assess long-term clinical and radiologic effects of direct transpedicular absolute alcohol embolization, laminectomy, and short-segment instrumented fusion on resolution of extraosseous epidural soft tissue and improvement in myelopathy in cases of vertebral hemangioma causing anterior soft-tissue compression. This was a retrospective analysis that included patients with single-level vertebral hemangioma with anterior intraspinal soft-tissue growth causing spinal cord compression and clinical features of myelopathy between June 2007 and June 2019 at authors' institute. Transpedicular vertebral body injection of absolute alcohol, laminectomy, and pedicle screw rod instrumentation was performed in all patients. Clinicoradiologic outcomes of surgery were noted. We proposed a grading system for the extent of anterior extraosseous epidural soft-tissue compression. In total, 14 patients were included in study. Their mean age was 28.4 ± 14.4 years. Mean follow-up duration was 67.5 ± 36.2 months. All patients had preoperative radiologic grade 4 compression. Postoperative radiologic grading improved to grade 1 in 4 (28.6%), grade 2 in 8 (57.1%), and grade 3 in 2 (14.3%). All the patients improved neurologically after surgery. Eleven patients improved to American Spinal Injury Association (ASIA) grade E, 2 improved to ASIA D, and 1 improved to ASIA C at the last follow-up visit. Vertebral hemangiomas with anterior extraosseous epidural extension causing spinal canal compromise and myelopathy can be managed with direct transpedicular ethanol embolization, laminectomy, and short-segment instrumented fusion with resolution of the extraosseous soft tissue and improvement in myelopathy. The procedure is relatively simple, cost effective, and has a good outcome. [ABSTRACT FROM AUTHOR]

Two fresh Co(II)-based coordination polymers (CPs) have been synthesized and hydrogels loaded with Co(II) metal–organic frameworks of bleomycin were produced on the basis of the drug's sluggish release. Their application values were assessed on osteosarcoma cells, and the associated mechanism was investigated. [Display omitted] • Two fresh Co(II)-based coordination polymers (CPs) have been synthesized. • HA/CMCS hydrogel was prepared by chemical synthesis. • We designed and synthesized two metallogel particle frameworks encapsulating bleomycin drugs. • We compared the effects of the two metallogel particle frameworks for the treatment of hemangiomas. In the current paper, through utilizing two symmetrical pentacarboxylate ligands, namely, 3,5-di(2′,5′-dicarboxylphenyl)benzoic (H 5 L 1) and 3,5-di(2′,4′ dicarboxylphenyl)benzoic acid (H 5 L 2), two fresh Co(II)-basd coordination polymers (CPs), [Co 5 (μ 3 -H 2 O) 2 (L1) 2 ]·3DMA·4H 2 O (1 , DMA is N, N -dimethylacetamide) along with [Co 3 (μ 3 -OH)L2(H 2 O) 3 ]·H 2 O (2) have been synthesized under the conditions of solvothermal. With hyaluronic acid (HA) and carboxymethyl chitosan (CMCS) as raw materials, HA/CMCS hydrogel was prepared by chemical synthesis and its internal microstructure was observed by scanning electron microscope. Using bleomycin as a drug model, we designed and synthesized two metallogel particle frameworks encapsulating bleomycin drugs and compared the effects of the two metallogel particle frameworks as drug carriers for delivering bleomycin drugs for the treatment of hemangiomas. [ABSTRACT FROM AUTHOR]

Cavernous hemangiomas are among the most common liver lesions encountered in abdominal imaging. While classical imaging characteristics usually aid the radiologist in confidently arriving at its diagnosis, atypical hemangiomas can prove to be difficult to distinguish from other more worrisome hepatic lesions such as metastases and hepatocellular carcinoma. Furthermore, some malignant lesions can display features that simulate hemangiomas. The radiologist must be aware of these pitfalls to make an accurate diagnosis, when possible. • A diagnosis of hemangioma is established by classical imaging characteristics. • Hemangiomas with atypical imaging features may present a diagnostic dilemma. • Malignant liver lesions may display imaging features that overlap with hemangiomas. [ABSTRACT FROM AUTHOR]

Infantile hemangioma (IH) is a common vascular tumor in children. It is reported that IHs are associated with immunochemical markers such as vascular endothelial growth factor (VEGF)-A, glucose transporter isoform 1 (GLUT1), and insulin-like growth factor-2 (IGF-2). This cross-sectional study focused on pediatric patients with IH. A total of 46 patients (mean age 14.2 ± 21.9 months) with IH and 45 healthy controls (mean age 21.8 ± 15.08 months) were enrolled. Demographic data, clinical findings, and laboratory parameters were recorded. Blood samples were collected. Serum GLUT1, IGF-2, VEGF-A, fibroblast growth factor 1 (FGF1), and angiopoietin 2 levels were assessed by enzyme-linked immunosorbent assay. Serum GLUT1, IGF-2, and VEGF-A levels were significantly higher in patients with IH than in healthy controls (8.80 ± 4.07 pg/mL vs. 5.66 ± 4.34 pg/mL, 281.10 ± 84.12 pg/mL vs. 234.19 ± 75.38 pg/mL, 1196.99 ± 389.34 pg/mL vs. 996.99 ± 349.16 pg/mL, respectively, p = 0.026, p = 0.030, and p = 0.036). Serum GLUT1, IGF-2, and VEGF-A levels in patients with complicated hemangioma were significantly higher than in healthy controls (9.69 ± 3.94 pg/mL vs. 5.66 ± 4.34 pg/mL, 289.94 ± 83.18 pg/mL vs. 234.19 ± 75.38 pg/mL, 1276.22 ± 388.24 pg/mL vs. 996.99 ± 349.16 pg/mL, respectively, p = 0.017, p = 0.022, and p = 0.011). Serum GLUT1, IGF-2, and VEGF-A levels in patients with hemangioma receiving propranolol treatment were significantly higher than in healthy controls. Serum FGF1 levels were higher in patients with IH, complicated hemangioma, and hemangioma receiving propranolol treatment than in healthy controls but the difference was not statistically significantly. Serum GLUT1, IGF-2, and VEGF-A levels were positively correlated with disease severity in patients with hemangioma, for example, in complicated hemangioma and hemangioma requiring propranolol treatment. However, further research on larger and different age subgroups is warranted to assess these markers. [ABSTRACT FROM AUTHOR]

Resection is rarely indicated in giant hepatic hemangiomas (HHs) that are symptomatic. Enucleation (EN), compared with anatomical resection (AR), is considered the better technique to resect them as EN has been reported to have lower morbidity while conserving the normal liver tissue. But no study has yet clearly established the superiority of EN over AR. In addition, the independent predictors of postoperative morbidity have not been established. All consecutive patients operated for HH at two specialized hepatobiliary centers were reviewed. Patient demographics, operative variables, and postoperative outcomes were analyzed and compared between two techniques. Postoperative complications were graded as per Clavien-Dindo classification of surgical complications. The aims of this study were to compare two techniques of HH resection with respect to postoperative outcomes and to identify the risk factors for 90-day major postoperative morbidity and mortality. A total of 64 patients, including 41 who underwent AR, 22 who underwent EN, and 1 who underwent liver transplantation, were operated for hemangiomas during the study period. Ten patients (9 who were operated for hemangiomas of size ≤4 cm and 1 who underwent transplantation) were excluded. Fifty-four patients, the majority being women (85%), with a median age of 48 years, were operated for giant HH. These patients were classified into two groups based on the technique of resection, namely, EN (22 patients) and AR (32 patients). Both groups were comparable in all aspects except that the number of liver segments resected was significantly more with AR. Postoperative outcomes were similar in both groups. Independent predictors of 90-day major complications including mortality were the use of total vascular exclusion (relative risk [RR]: 2.3, p = 0.028) and duration of surgery >4.5 h (RR: 2.3, p = 0.025). Both techniques yield similar results with respect to 90-day postoperative morbidity and mortality. The choice of technique should be based on the location of tumor and simplicity of liver resection. [ABSTRACT FROM AUTHOR]

Spinal hemangiomas are common primary tumors of the vertebrae. Although these tumors are most frequently benign and asymptomatic, they can rarely exhibit aggressive growth and invasion into neighboring structures. Treatment for these aggressive variants is controversial, often involving surgery, chemotherapy, and/or radiotherapy. This study sought to investigate current trends affecting overall survival (OS) using the National Cancer Database (NCDB) and to formulate treatment recommendations. The National Cancer Database was queried for spinal hemangiomas between 2004 and 2016. A Cox proportional hazards model was used to perform multivariate regression analysis of survival. Survival curves for comparative visualization of demographic and treatment factors were generated using a semiparametric Cox approach. A cohort of 102 patients with histologically confirmed spinal hemangiomas was identified in the database. Mean OS was 1.94 years. Administered treatments included partial surgical resection (n = 17), radical resection (n = 14), chemotherapy (n = 34), and radiotherapy (n = 56). Multivariate analysis revealed associations between decreased OS and advanced age (>65 years) and presence of metastasis. Cox survival analysis further revealed improved OS in patients who received surgical treatment and higher radiation dose. This retrospective analysis finding that treatment with surgical resection and/or radiotherapy is associated with increased OS constitutes the largest cohort of patients with aggressive vertebral hemangiomas to date. Given that the mean OS of the study cohort was 1.94 years, our findings suggest that the optimal treatment regimen to maximize survival should consist of early surgical resection with adjuvant high-dose radiotherapy. [ABSTRACT FROM AUTHOR]

Background: Individuals with Sturge-Weber syndrome (SWS) often expereince intractable epilepsy and cognitive decline. We hypothesized that the extent of the leptomeningeal capillary malformation (LCM) may correlate with the severity of neurological impairment due to SWS. We tested the hypothesis in a cross-sectional study of seizure severity and electroencephalographic (EEG) findings and a retrospective cohort study for surgical indications related to the extent of the LCM.Methods: We enrolled 112 patients and classified them according to LCM distribution: (1) bilateral, (2) hemispheric, (3) multilobar, and (4) single lobe. Age at seizure onset, seizure semiology and frequency, and EEG findings were compared. Surgical indications were evaluated for each group by Fisher exact test, and predictors for surgery were evaluated by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-Neurological Score (SWS-NS).Results: The bilateral and hemispheric groups had early seizure onset (4.0 months old and 3.0 months old), frequent seizures (88.9% and 80.6% had more than one per month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). The groups' EEG findings did not differ substantially. Surgical indications were present in 77.8% of the bilateral, 88.1% of the hemispheric, and 46.8% of the multilobar groups. Seizure more than once per month was a predictor of surgical treatment. Seizure subscore improved postoperatively in the hemispheric and multilobar groups. Even after surgical treatment, the bilateral and hemispheric groups exhibited higher SWS-NSs than members of the other groups.Conclusion: Our study demonstrated a strong association between extensive LCM and epilepsy severity. Surgical intervention improved seizure outcome in patients with SWS with large LCMs. [ABSTRACT FROM AUTHOR]

The aim of the study described here was to clarify the diagnostic value of the fluttering sign, a new sign that characterizes hepatic hemangiomas in gray-scale ultrasonography (US). It refers to a phenomenon in which the speckled echogenicity inside the hemangioma changes continuously and seems to be moving. A total of 172 hemangiomas diagnosed with contrast-enhanced US were evaluated. The fluttering sign was found in 123 of 172 hemangiomas (71.5%). Its prevalence was significantly higher than that of the marginal strong echo (89/172, 51.7%, p < 0.001), posterior acoustic enhancement (103/172, 59.9%, p = 0.031) and chameleon sign (100/172, 58.1%, p = 0.013). In addition, the fluttering sign was observed significantly more frequently in mixed or hypo-echoic tumors than in hyper-echoic tumors (p < 0.001), relatively large tumors (p < 0.001) and tumors that were less than 5 cm from the body surface (p = 0.015). The fluttering sign in gray-scale US has great potential to be a new complementary sign for the diagnosis of hemangioma. [ABSTRACT FROM AUTHOR]

Background: Infantile hepatic hemangioma (IHH) is a rare but life-threatening disorder that must be considered in the newborn presenting with high-output heart failure (HF). IHH is a tumor comprised of large vascular beds, which require a significant increase in blood flow as the lesion grows. This, in turn, creates an undue burden on the cardiovascular system, leading to high-output HF and potentially, respiratory distress. Recent changes have been made in the classification of certain hepatic hemangiomas and their treatments.Case Report: A 10-day-old girl presented to the Emergency Department with increased respiratory effort and an episode of apnea and cyanosis. A chest x-ray study was obtained and showed cardiomegaly and pulmonary edema concerning for HF. The patient was promptly admitted to the pediatric intensive care unit, where advanced imaging was obtained revealing findings consistent with IHH. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: HF in an infant is a critical condition often requiring prompt intervention and rapid diagnosis of the correct etiology to save a life. IHH is an example of an extrathoracic etiology of pediatric HF that has undergone recent changes in terminology and diagnosis. Increased awareness among emergency physicians of this disease process and its treatments can lead to expeditious diagnosis and treatment of this potentially life-threatening illness. [ABSTRACT FROM AUTHOR]

Intracranial capillary hemangiomas (ICHs) are rare vascular tumors composed of a bed of many narrow thin-walled vessels. Within the confines of the skull, these tumors can lead to serious neurologic deficits including cranial nerve dysfunction, mood/personality disturbances, and signs of intracranial mass effect. We report the case of a 23-year-old, 5-week postpartum woman with a history of progressive painful ophthalmalgia of the right eye presenting with rapid onset of ptosis, diplopia, and right-sided facial pain and hypesthesia. Imaging demonstrated a small extraaxial mass within the right cavernous sinus. She underwent 2 operations via an endoscopic endonasal approach for biopsy followed by complete resection. Histology showed a highly mitotic capillary hemangioma, which was negative for both estrogen and progesterone receptors. We review cases of ICH reported in the literature and provide an updated summary of the presentation, diagnosis, and treatment of ICH. We then present a brief analysis of the reported cases with respect to age and sex. We conclude that, in experienced hands, the endoscopic endonasal approach can be used to access the cavernous sinus for complete resection of ICHs of the cavernous sinus. We also suggest that further attention be paid to such cases in pregnant and peripartum women as these tumors may progress more quickly in this subpopulation. [ABSTRACT FROM AUTHOR]

Beta-blockers (BB) are an heterogenous set of molecules actively blocking β adrenergic receptors. Their pharmacological properties depend on their various effects on the adrenergic signalling. Although they are no longer a first-choice treatment in hypertensive patients, they remain a cornerstone of pharmacological strategy in several cardiovascular diseases such as stable angina, heart failure, arrythmia and aortic related connective diseases. Beyond their usual non cardiovascular indications such as migraine, hepatic cirrhosis, glaucoma, infantile hemangioma, and hyperthyroidism, new therapeutic fields are under scrutiny. Potential BB therapeutic repurposing is being investigated in COPD and cancer patients. This narrative review first encompasses the basic pharmacological knowledge that may be useful for the clinician. Then it will detail BB main indications before exploring new therapeutic fields. [ABSTRACT FROM AUTHOR]

We describe the clinical outcomes in patients with aggressive vertebral hemangiomas (AVHs) after total tumor excision and discuss the treatment options for AVHs. A retrospective data review of 15 patients (6 men, 9 women) with AVHs who underwent total excision between 1996 and 2018 was performed. In total, 13 thoracic and 2 lumbar lesions were involved with 8 type A−D tumors and 7 type B−D tumors based on the Weinstein-Boriani-Biagini classification. All tumors showed low or low-iso signal intensity by T1-weighted magnetic resonance imaging. All patients received a combination of preoperative transarterial embolization and total tumor excision including the tumor margins. Eleven patients underwent total tumor excision as the initial surgery (total en bloc spondylectomy = 10 patients, piecemeal total tumor excision = 1 patient), and 4 underwent it as either a revision procedure 2 weeks after ineffective laminectomy or in the long-term follow-up (4–14 years) as a piecemeal total tumor excision. Intraoperative blood loss ranged from 150 to 3400 mL (mean, 1314 mL). None of the cases had a recurrence during the mean follow-up period of 128.4 ± 88.6 months. Low signal intensity on T1-weighted magnetic resonance imaging was observed in all the patients with AVHs. The long-term clinical results of the preoperative transarterial embolization and total tumor excision were satisfactory. The effect of decompressive incomplete tumor excision is temporary for AVHs, and repeated tumor excision may be necessary because of tumor recurrence in the long term. Reliable total tumor excision during the initial surgery is desirable. [ABSTRACT FROM AUTHOR]

Extraaxial cavernous hemangiomas (EACHs) are an extremely rare form of cerebral vascular malformations, occurring infrequently in children and rarely exceeding 6.0 cm in diameter (labeled as "giant"). Our case study highlights the radiographic, diagnostic, and interventional uncertainties in the management of giant EACHs in a pediatric population. Our patient, a 15-year-old male with 3-month history of hemiparetic symptoms, was shown to have a 9.0-cm extraaxial parietal-occipital mass upon imaging. Differential diagnosis included cavernoma versus meningioma because of similarities in imaging presentation. Pathologic confirmation of EACH was achieved after resection, and the patient experienced positive outcomes including total recovery from his hemiparesis. EACHs are difficult to characterize radiographically and are often mistaken to be meningiomas before resection and pathologic confirmation. Therefore cavernous hemangiomas should be considered in the differential diagnosis of pediatric extraaxial masses. Additionally, preoperative hemorrhage control efforts should be pursued to reduce adverse outcomes stemming from resection of vascular malformations such as EACHs. [ABSTRACT FROM AUTHOR]

The uptake of 18F-FDG is higher in most malignancies than in benign tumors. This study aimed to investigate the diagnostic value of 18F-FDG PET/CT in vertebral vascular tumors. We retrospectively collected PET/CT and clinical data of patients with vertebral vascular tumors and analyzed the location, number, and bone destruction and FDG uptake features of the lesion. We measured SUVmax and maximum diameter and analyzed the correlations between SUVmax and the pathological results, size, and CT features. Twenty-one pathology-proven vertebral vascular tumors were included: 2 angiosarcomas (SUVmax, 11.6 and 32.3), 1 epithelioid hemangioendothelioma (SUVmax, 5.7), 1 epithelioid hemangioma (SUVmax, 8.5), and 17 aggressive hemangiomas. Twelve cases of typical hemangiomas were included as controls. The SUVmax and diameter of the aggressive hemangiomas were higher than those of the typical hemangiomas. The mean SUVmax of aggressive hemangiomas with cortical destruction was higher than that of those without cortical destruction (t = −2.566, P = 0.022). Radioactive distribution in aggressive hemangiomas was homogeneous and heterogeneous in nine and eight cases, respectively. In six aggressive hemangiomas, the FDG uptake of residual and marginal sclerosing bone was higher than that of the osteolytic destruction area and/or paravertebral soft tissue. Six aggressive hemangiomas involved the spinal canal, without clear visualization on PET/CT. 18F-FDG uptake of vertebral malignant vascular tumors is higher than that of hemangiomas. The FDG uptake of hemangiomas varies and may be related to concurrent cortical destruction. 18F-FDG PET/CT shows limitations in evaluating the spinal canal involvement of aggressive hemangioma. • This study compared the 18F-FDG PET/CT findings of vertebral vascular tumors with those of typical hemangiomas. • The results showed that 18F-FDG uptake of vertebral malignant vascular tumors is higher than that of hemangiomas. • The FDG uptake of hemangiomas varies and may be related to concurrent cortical destruction. • We demonstrate some features of these tumors seen on 18F-FDG PET/CT scans. [ABSTRACT FROM AUTHOR]

Infantile hemangioma is one of the most common vascular tumors, which might result in morbidity and mortality without timely intervention. Propranolol is currently the first-line therapy for hemangiomas, but its potential side effects and high frequency of administration make it urgent to develop a suitable drug delivery system for propranolol. In the present study, we formulated a propranolol delivery system based on mesoporous silica nanoparticles (PRN@MSN) and investigated the interplay between autophagic activities mediated by nanoparticles and improved therapeutic efficacy of PRN@MSN. The results showed that PRN@MSN nanoparticles exhibited higher cytotoxicity compared with free propranolol in vitro and in vivo, which could induce excessive autophagosome accumulation through increased autophagosome formation and impaired autophagic degradation. Inhibition of autophagy in the early stage could attenuate the cytotoxicity of PRN@MSN. ROS generation was essential for nanoparticle-mediated autophagy and cytotoxicity, and PRN@MSN-induced autophagy dysfunction could enhance endoplasmic reticulum (ER) stress in hemangioma stem cells. Our study revealed a promising PRN delivery system based on a mesoporous silica nanoplatform that could induce autophagy dysfunction with excessive autophagosome accumulation to promote the therapeutic efficacy of PRN therapy. PRN@MSN drug delivery system combined with autophagy modulation may act as a promising treatment pattern in the treatment of hemangiomas. [ABSTRACT FROM AUTHOR]

To assess the practicality of 3-D power Doppler angiography (3-D-CPA) for local drug perfusion dosage guidance of refractory infantile hemangioma (IH) treatment, 47 cases (48 lesions) of refractory IH were selected for local bleomycin infusion (once a month). Ultrasound was performed before treatment and 1 and 2 months after the first treatment. The 3-D volume (V) change of infantile hemangiomas and the ratio of bleomycin injection to 3-D V before treatment were calculated, and statistical analysis was performed. One month after percutaneous local drug perfusion, 37 participants (77.08%) exhibited significant improvement; controlled growth was observed on six lesions (12.5%); and treatment of the remaining 5 lesions (10.42%) failed to suppress growth. The calculated tolerable and effective dose of bleomycin for refractory IH was 0.34 ± 0.03 mL/cm3; the corresponding 3-D V decreased approximately 70.27 ± 6.27%. Three-dimensional CPA can provide abundant information on internal lesions. In particular, 3-D-CPA can quantitatively assess changes in lesion volume and guide the effective and rational use of interventional drugs. [ABSTRACT FROM AUTHOR]

To evaluate the efficiency of a comprehensive treatment strategy for patients with Enneking stage 3 thoracic aggressive vertebral hemangioma (AVH). A retrospective analysis of radiographic and clinical outcomes was performed for 17 patients who received treatment for Enneking stage 3 thoracic AVH accompanied by pain and neurologic deficits between January 2010 and February 2015. A visual analog scale (VAS) was used to clinically evaluate the level of pain. Neurologic examinations were performed to assess the patients' sensory symptoms, motor deficits, and Frankel grade. The average operative time was 181.8 ± 37.1 minutes, and the average intraoperative blood loss was 1226.5 ± 151.2 mL (range, 900–1450 mL). All patients underwent preoperative embolization to minimize intraoperative blood loss. All patients experienced immediate pain relief and resolution of their neurologic symptoms. All 17 patients achieved Frankel grade D at the final follow-up; moreover, they reported that their pain was relieved (mean VAS score, 2.82 ± 0.81; P < 0.05) and their neurologic deficits had resolved. No surgery-related complications were noted. No patients exhibited signs of recurrence. We recommend a comprehensive treatment strategy for patients with Enneking stage 3 thoracic AVH that includes preoperative embolization, vertebroplasty, posterior decompression, and internal fixation. We recommend that absorbable gelatin sponge particles be used for intraoperative embolization prior to the injection of bone cement, which may significantly reduce intraoperative bleeding, operative time, and occurrence of surgery-related complications. [ABSTRACT FROM AUTHOR]

Choroidal hemangioma (CH) is a benign vascular tumor that induces subretinal fluid collection or exudative retinal detachment and consequent visual symptoms. Current standard treatments for CH include cryotherapy, diathermy, photocoagulation, photodynamic therapy, transpupillary thermotherapy, and radiation therapy. Stereotactic radiosurgery has recently been applied to the treatment of CH because of its characteristic stiff dose-fall-off and accuracy. We have adopted gamma knife radiosurgery (GKRS) to treat CH and have retrospectively assessed tumor volume reductions and improvements to visual acuity achieved thereby. Fourteen patients with CHs were treated with GKRS from November 2006 to December 2017. Eight patients had circumscribed CH, and 6 exhibited diffuse CHs and were diagnosed with Sturge-Weber syndrome. The mean age of patients was 27.1 years (range: 8–68 years) and the mean duration of clinical or radiological follow-up was 40.2 months (range: 5–105 months). The mean volume of the tumors at the time of GKRS was 533.5 mm3 (range: 124–1150 mm3), and the mean prescribed marginal dose was 11.6 Gy (range: 10–16 Gy) with 50% isodose lines. The tumor volume decreased by the last follow-up in all patients. The visual acuity improved in 9 patients (64%) and decreased in 1 (7%). Six patients (43%) required trans-pars plana vitrectomy before or after GKRS. There were no symptomatic complications from radiation injury during the follow-up periods. GKRS could be an acceptable alternative treatment for symptomatic CH when standard therapy is not feasible. [ABSTRACT FROM AUTHOR]

Hemangiomas, abnormal growths of vascular endothelial cells, are common vascular tumors in children. In contrast, their prevalence is 1–3 % in full-term newborns. Their unique location and associated complications can cause significant distress for patients and families. Prompt treatment is crucial to prevent complications such as fertility issues, hemorrhage, or other related problems. This case study explores the diagnosis and management of scrotal hemangiomas, emphasizing the importance of timely intervention. A 10-year-old boy with a scrotal hemangioma since birth underwent surgical excision due to increased discomfort caused by a new lesion. 2.5 × 2.3 cm hemangioma was successfully removed through En Bloc Excision. The patient experienced a swift recovery, and histopathologic analysis confirmed the diagnosis of cavernous hemangioma. This case underscores the importance of careful monitoring and decisive intervention in managing scrotal hemangiomas. Scrotal hemangiomas often present challenges in diagnosis, mimicking malignant tumors. Clinical examination, ultrasonography, and histopathology are crucial for accurate diagnosis. Various surgical techniques, including En Bloc Excision, have been employed, with postoperative care tailored to individual cases. Precise preoperative diagnosis is essential to distinguish scrotal hemangiomas from other conditions and guide appropriate management strategies. This case study highlights the management of a scrotal hemangioma through En Bloc Excision, emphasizing the importance of considering definitive treatment options. The comprehensive scrotal examination is crucial for accurate diagnosis and effective management of this rare vascular tumor. • Scrotal hemangiomas are rare vascular tumors. Their location can cause significant distress and complications for patients. • Precise diagnosis is essential to distinguish scrotal masses and guide appropriate therapy. • Review of literature shows surgery as definitive treatment for scrotal hemangiomas. • Highlight the rarity of disease, role of precise diagnosis and surgical excision as optimal therapy. [ABSTRACT FROM AUTHOR]

SG is a 50-year-old nonsmoker with a history of cerebral cavernoma referred for optimal management of lipid disorder. To review a clinical scenario involving management of dyslipidemia in a patient with cerebral cavernoma with high risk of hemorrhage. SG was seen in the office at Lipid Clinic at Penn for further evaluation. SG is a 50-year-old nonsmoker male with family history of premature coronary artery disease (CAD)/myocardial infarction in father at age 50 and personal history of mild CAD with trace atherosclerotic calcification in proximal LAD on CT angiogram presents for cardiovascular risk management. Case is complicated by the recent diagnosis of 18 mm left frontal cerebral cavernoma with a very small microhemorrhage in left temporal lobe on MRI performed due to acute confusional migraines. While untreated LDL-c is elevated in the setting of family history of premature CAD and personal history of mild CAD, lipid lowering with first line therapy statins may be challenging with the background of cerebral cavernoma. Hence, non-statin option ezetimibe is used in this case with appropriate ∼20% reduction in LDL cholesterol with a goal below 100 mg/dL for a patient with high risk of hemorrhage. Cerebral cavernomas are rare but highly fatal vascular malformations, affecting about 0.4-0.6% of the population with 2.5% annual risk of hemorrhage and mortality rate up to 20%. While statins have showed to reduce the overall incidence of strokes and cardiovascular events in patients with recent stroke or transient ischemic attack, they also may increase the risk of hemorrhagic stroke in a dose-dependent and type of vascular brain injury-dependent manner. This may be attributed to antithrombotic properties of statins including: anticoagulant effects (via downregulation of tissue factor and upregulation of thrombomodulin); antiplatelet effects (via inhibition of platelet thromboxane A2 and platelet isoprostane formation); and profibrinolytic effects (via inhibition of the expression of plasminogen activator inhibitor-1 and upregulation tissue-type plasminogen activator from vascular smooth muscle and endothelial cells via inhibition of Rho geranylgeranylation). In contrast, non-statin options such as ezetimibe and PCSK9 inhibitors may not increase hemorrhagic stroke risk, in part due to their lack of antithrombotic properties. Particularly in patients with cerebral cavernous malformations, use of statins for cardiovascular risk reduction has been controversial. While observational studies have raised concerns about the safety of statins in patients with cerebral cavernomas via increased permeability within malformations, large randomized controlled trials have yet to confirm. Hence, non-statin lipid-lowering therapies such as ezetimibe and PCSK9 inhibitors may be preferred over statins in patients with elevated risk of hemorrhagic stroke, including patients with cerebral cavernomas. No [ABSTRACT FROM AUTHOR]

Vascular anomalies include both tumors and malformations. Infantile hemangiomas are the most common benign vascular tumor of infancy that proliferate after birth and eventually involute. By contrast, congenital hemangiomas are formed at birth and are categorized into three groups: rapidly involuting, partially-involuting, and non-involuting congenital hemangiomas (NICH). NICH do not regress and grow with age. Pyogenic granulomas, another acquired vascular tumor, develop over vascular lesions and cause bleeding. Primary treatment options for NICH and pyogenic granulomas are surgical resection. Here, we report a case of a NICH with a co-existing pyogenic granuloma involving the penile shaft and scrotum treated surgically. [ABSTRACT FROM AUTHOR]