An anastomosing hemangioma mimicking a renal cell carcinoma in a kidney transplant recipient: a case report.

BACKGROUND: Although anastomosing hemangiomas are very rare and benign vascular neoplasms, these tumors are more common among patients with end-stage kidney disease. Incidental finding of these tumors in the kidney or adrenal gland has been reported. Herein, we describe a case in which an anastomosing hemangioma was misdiagnosed as a renal cell carcinoma before kidney transplant. CASE PRESENTATION: A 35-year-old woman with lupus nephritis was admitted to our emergency department for suspected uremic symptoms of nausea and general weakness. She had received hemodialysis due to end-stage kidney disease, and a living-donor kidney transplantation from her father was planned. On pre-operative contrast-enhanced computed tomography and magnetic resonance imaging, a 1.7 cm renal cell carcinoma was observed in the right kidney. On staining after radical nephrectomy, irregularly shaped vascular spaces of various sizes were observed, with these spaces having an anastomosing pattern. As the findings of the anastomosing hemangioma are similar to those of a renal cell carcinoma on imaging, histology examination was necessary to confirm the diagnosis of anastomosing hemangioma and to prevent delay in listing for kidney transplantation. Good kidney function was achieved after transplantation, with no tumor recurrence. CONCLUSION: Our case underlines the importance for prompt surgical resection of an enhancing renal mass to confirm diagnosis in patients scheduled for kidney transplantation to avoid any delay.

Proanthocyanidins as a Potential Novel Way for the Treatment of Hemangioma.

Hemangioma, the most common benign vascular tumor, not only affects the appearance and psychology but also has a life-threatening potential. It is considered that clonal vascular endothelial cell proliferation and excessive angiogenesis are responsible for hemangioma pathogenesis, in which abnormal cytokines/pathways are closely implicated, primarily including high expression of hypoxia-inducible factor-1α (HIF-1α) and vascular endothelial growth factor (VEGF) as well as their downstream pathways, especially phosphatidylinositol 3-kinase (PI3K)/protein kinase B (Akt). These further stimulate the migration and proliferation of vascular endothelial cells and promote the formation of new vessels, ultimately leading to the occurrence and development of hemangioma. Proanthocyanidins are naturally active substance from plants and fruits. They possess multiple functions like antiproliferation, antiangiogenesis, and antitumor. It has been demonstrated that proanthocyanidins effectively work in various diseases via inhibiting the expression of various factors, e.g., HIF-1α, VEGF, PI3K, and Akt. Considering the pathogenesis of hemangioma and the effect of proanthocyanidins, we hold a hypothesis that proanthocyanidins would be applied in hemangioma via downregulating cytokine/pathway expression, suppressing vascular cell proliferation and arrest abnormal angiogenesis. Taken together, proanthocyanidins may be a potential novel way for the treatment of hemangioma.

Management of benign liver tumors.

Benign liver tumors in children are far less frequent than their malignant counterparts. Recently, there have been advances of diagnostic procedures and novel treatments with improved classification as a result. While malignant pediatric liver tumors have been comprehensively addressed by multicenter international tumor trials, benign tumors have more usually relied upon individualised workup and treatment. Due to the rarity and heterogeneity of these different entities, large studies are lacking. In this article the authors highlight the spectrum of benign liver tumors with special focus on specific clinical features, pathology, and treatment.

The expression and role of glycolysis-associated molecules in infantile hemangioma.

AIMS: Infantile hemangioma (IH) is one of the most common tumors in infancy, which etiology and pathogenesis has not been fully elucidated, hypoxia and abnormal glucose metabolism is regarded as critical pathogenic factors. This study investigated the expression and function of glycolysis-associated molecules (GLUT1, HK2, PFKFB3, PKM2, and LDHA) under normoxic and hypoxic conditions to further understand the pathogenesis of IH. MAIN METHODS: Hemangioma-derived endothelial cells (HemECs) were isolated from proliferating phase infantile hemangiomas and identified by immunofluorescence. HemECs and human umbilical vein endothelial cells (HUVECs) were cultured under normoxic and hypoxic conditions. RNA and protein expression of glycolysis-associated molecules were analyzed by quantitative real-time RT-PCR, western blotting, and immunohistochemistry. Glucose consumption, ATP production and lactate production were measured. Glycolysis-associated molecules were inhibited by WZB117, 3BP, 3PO, SKN, and GSK 2837808A and the resulting effects on HemECs proliferation, migration, and tube formation were quantified. KEY FINDINGS: Glycolysis-associated molecules were highly expressed at both mRNA and protein levels in HemECs compared with HUVECs (P < 0.05). Glucose consumption and ATP production were higher in HemECs than in HUVECs, while lactate production in HemECs was lower than in HUVECs (P < 0.05). Inhibition of some glycolysis-associated molecules reduced the proliferation, migration, and tube formation capacity of HemECs (P < 0.05). SIGNIFICANCE: Our study revealed that glycolysis-associated molecules were highly expressed in IH. Glucose metabolismin HemECs differed from normal endothelial cells. Altering the expression of glycolysis-associated molecules may influence the phenotype of HemECs and provide new therapeutic approaches to the successful treatment of IH.

Failed angioembolization of a ruptured liver hemangioma complicated by iatrogenic injury of subclavian vein during catheter insertion.

In this study, we report a case of failed angioembolization of a ruptured liver hemangioma complicated by iatrogenic injury of the subclavian vein during catheter insertion. A 30-year-old woman experienced blunt trauma upon falling from her bed. Laceration of a seemingly preexisting hepatic hemangioma was diagnosed. No other injury was detected during a preoperative diagnostic workup. Subclavian vein catheterization was performed, followed by angioembolization to control bleeding due to the ruptured hemangioma. After angioembolization, the patient's systolic blood pressure and hemoglobin levels were 70 mmHg and 5.3 g/dL, respectively. She underwent emergency laparotomy. During the surgery, a large volume of blood in the abdominal cavity due to profuse bleeding from the ruptured hemangioma was observed. Because of a hemothorax found on chest radiography, we performed thoracoscopy, which revealed a large volume of blood in the right thoracic cavity and perforation of the subclavian vein by the catheter. After the damage-control surgery, the patient recovered safely. In this case, ruptured liver hemangioma complicated by subclavian vein catheter-related injury was treated safely using damage-control surgery. The catheter-related injury could be identified and treated using thoracoscopy.

Vertebral hemangiomas: Diagnosis and management. A single center experience.

OBJECTIVE: Vertebral hemangiomas (VH) are common benign lesions involving the spine. Owing to the multiplicity of treatments, the management of VH has not always been consistent. In this retrospective review of a single center experience, indications and options available for the treatment of VH are outlined. PATIENTS AND METHODS: This is a retrospective review of 71 cases of VH managed at our institution between 2005 and 2019. Sixty of these cases were managed non-operatively, with 11 cases undergoing operative intervention. Of the 11 cases that underwent surgery, there were 2 cervical cases and 9 in the thoracic spine. Ten cases were symptomatic, and 1 incidental. Three patients presented with localized pain, and the remaining 7 had neurological deficit. Decompression with maximal resection of the hemangioma was undertaken in 10 cases, and vertebroplasty in 1. RESULTS: Of the 60 patients who were managed non-operatively, 13 patients had presented with back/neck pain, with the remaining 47 patients being asymptomatic and diagnosed incidentally. Among the 13 symptomatic patients, all were offered surgical intervention for pain management, but given lack of severity of symptoms, all had opted for conservative approaches of pain control. In the 11 patients who underwent surgery, the preoperative diagnosis of VH was accurate in all but 1 case. There were 2 cervical cases treated with corpectomy. One patient was treated with vertebroplasty, and the remaining 8 with decompression. Radiation was used in 2 cases. Of the 10 patients undergoing decompression, 7 patients had improvement of the neurologic deficit, with resolution of pain in the remaining 3. None of our cases demonstrated deterioration. CONCLUSION: VH are often discovered incidentally during evaluation of spinal pain. Except in rare cases, the diagnosis of VH is made correctly from the radiographic and MRI studies. Observation for the asymptomatic lesion is appropriate. For VH presenting with deficit or intractable pain, decompressive surgery is recommended. Radiation is appropriate in cases of recurrent VH.

Infantile hepatic hemangiomas associated with high-output cardiac failure and pulmonary hypertension.

BACKGROUND: Infantile hepatic hemangioma (IHH) is a rare endothelial cell neoplasm, which may be concurrent with severe complications and result in poor outcomes. Moreover, the coexistence of IHH and congenial heart disease is even rarer. CASE PRESENTATION: We present a 10-day-old male born with IHH associated with patent ductus arteriosus (PDA), atrial septal defect (ASD) and pulmonary hypertension. Moreover, we reviewed a series of studies of IHH-associated high-output cardiac failure between 1974 and 2018, and summarized the treatment outcomes. CONCLUSIONS: Infantile hepatic hemangioma (IHH) has been known to induce high-output heart failure. There is no literature to summarize the severity of its impact on heart, which can lead to a high mortality rate. When IHH is detected by ultrasound, the heart should be evaluated to facilitate treatment. The outcomes of IHH associated with heart failure are good.

An extremely rare and atypical paediatric presentation of a maxillary sinus haemangioma in the UK.

We present a rare and unusual case of a 16-year-old girl, with no significant medical history, presenting with right nasal obstruction and suspected sinusitis with occasional epistaxis and haemoptysis. On examination, she had a mass lesion in the right nasal cavity, with no evidence of other pathology on assessment of the ears, nose, throat or head and neck. A CT scan revealed an opacified right maxillary sinus with polypoidal mucosa, extending and passing through the accessory ostium into the right nasal cavity. Examination under anaesthesia with functional endoscopic sinus surgery and excision of the lesion was subsequently undertaken. Histological analysis confirmed the mass lesion as a haemangioma. This case report is the first to present a maxillary haemangioma presenting as nasal obstruction with intermittent sinusitis symptoms in a child. The authors discuss the incidence, presentation and management of maxillary haemangiomas in the paediatric population.

The Effects of Ocular Perfusion Pressure Variations on a Case of Circumscribed Choroidal Hemangioma Affected by Primary Open-Angle Glaucoma.

The authors describe the clinical and anatomical correlation between mean ocular perfusion pressure (MOPP) changes and macular exudation in a 62-year-old man affected by circumscribed choroidal hemangioma (CCH) and primary open-angle glaucoma. During the 5-year follow-up, we noted an increase of the subretinal fluid (SRF) and cystoid macular edema (CME) when the intraocular pressure (IOP) was in the low teens. Optical coherence tomography scans performed during an isometric exercise revealed a transient increase of the retinal (choroidal) thickness. In the presented case, MOPP appears to play a role in SRF and CME associated with CCH. Management of the macular exudation could improve if IOP and blood pressure are considered and treated accordingly. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e163-e165.].

Combined Parafoveal Telangiectasia, Multifocal Central Serous Chorioretinopathy, and Choroidal Hemangioma.

The exact pathogenesis of parafoveal telangiectasia (PFT) is unknown, but various associations have been reported in literature such as diabetes and hypertension. Coexisting pathologies like diabetic retinopathy with PFT are not uncommon. We encountered a unique combination of PFT with multifocal central serous chorioretinopathy and choroidal hemangioma in a patient with diabetes mellitus. Multimodal imaging confirmed the findings and focal treatment to central serous chorioretinopathy leaks led to resolution of the subretinal fluid. This unique combination has been rarely reported so far. The long-term follow-up of such cases can help us to understand if there exists common pathogenesis of these varied diseases.

Synovial hemangioma of the knee joint in pediatrics: our case series and review of literature.

BACKGROUND: Synovial hemangioma is a benign intra-articular tumor. This condition is rare and unfamiliar soft tissue tumor to most orthopedic surgeons. Delayed diagnosis causes osteoarthritic damage and the destruction of joint structures due to infiltrating tumor growth. We discuss the patterns of tumor location and the appropriate surgical procedure for this condition. METHODS: Ten patients were treated surgically in our department. These comprised five males and five females ranging in age from 0 to 17 years (average age 12.4 years). Preoperative diagnosis was made using clinical findings, plain radiographs and magnetic resonance imaging. The follow-up time after surgery was at least 3 years. RESULTS: The main symptom was pain. Three cases revealed hemarthrosis. The range of motion of the affected knee joint was limited in five cases. The average time between onset of pain and diagnosis was 3 years. Tumor location was classified into three patterns: (1) anterior patellofemoral joint type in five, (2) posterior popliteal type in two and (3) diffuse proliferation type in two. Open arthrotomy with synovectomy was performed in all cases. No tumor recurrences were experienced after a minimum follow-up of 3 years. CONCLUSION: Clinical symptom and magnetic resonance imaging are helpful to obtain the diagnosis and determine the extent of the lesion. Depending on the tumor location, synovial hemangioma in the knee joint can be classified into patellofemoral, popliteal and diffuse types. Open arthrotomy with sufficient tumor and synovectomy is important to prevent tumor recurrence.

Non-involuting congenital hemangiomas (NICH) with postnatal atypical growth: A case series.

BACKGROUND: Non-involuting congenital hemangiomas (NICH) are fully formed vascular tumors at birth, with a distinctive clinical, radiologic, and histopathological profile, and classically lack expansion or involution over time. We describe a series of NICH cases with atypical postnatal growth. METHODS: The authors retrospectively analyzed all NICH cases diagnosed from 2007 to 2017. We reviewed charts and photographic databases from our Vascular Anomalies Clinic. We included in the study all NICH with an atypical postnatal growth. Clinical data, imaging, and histopathology were analyzed. RESULTS: Eighty cases of NICH were identified. Nine presented with atypical postnatal growth after a stable period, at ages from 2 to 10 years (mean: 5.3 years). Two patients had associated pain; 5 patients showed new red papules on the surface of the lesion; 2 reported bleeding from the papules; and 1 developed a pyogenic granuloma. All patients had Doppler ultrasound and/or MRI compatible with NICH, and a confirmatory biopsy was performed in 4 cases. In treatment, 2 patients received endovascular embolization, and one required further surgery. CONCLUSIONS: Non-involuting congenital hemangiomas (NICH) may develop significant postnatal growth over time (10% in our series), requiring closer follow-up for longer periods. The development of red papules, pyogenic granulomas, and superficial bleeding may be observed. Since this is a small series, we were not able to establish risk factors for NICH with postnatal growth.

TREATMENT OF EXUDATIVE CIRCUMSCRIBED CHOROIDAL HEMANGIOMA: Efficacy of Fractionated Proton Therapy (20 Gray Relative Biological Effectiveness in 8 Fractions).

PURPOSE: To evaluate treatment of circumscribed choroidal hemangioma by hyperfractionated proton beam therapy protocol (20 gray relative biological effectiveness in 8 fractions) on tumor control, attachment of retina and visual function. METHODS: Retrospective review of patients treated between January 2010 and April 2015 with at least 6 months of follow-up. RESULTS: Forty-three patients with exudative and symptomatic circumscribed choroidal hemangioma were included. Before treatment, 41 (95%) presented an exudative retinal detachment, median visual acuity was 20/63 and median tumor thickness was 3.3 mm. Mean follow-up was 26 months (7-62). At last follow-up, all patients presented regression of ultrasound tumor thickness and 23/43 (53.5%) a totally flat scar. The mean time to achieve a flat scar was 20 months. Retina was reattached in all patients except one with 9 months of follow-up. Visual acuity was improved or stabilized in 37 patients (86%) and final median visual acuity was 20/25. No patient presented radiation maculopathy or papillopathy. CONCLUSION: Proton beam therapy with a dose of 20 gray relative biological effectiveness delivered in 8 fractions provides excellent anatomical and functional results and are comparable with those obtained with the same dose delivered in 4 fractions. Longer follow-up is required to determine the long-term radiation sequelae.

Intralesional Bevacizumab for the Treatment of Recurrent Sinonasal Hemangioma.

INTRODUCTION:: Sinonasal hemangioma is a rare benign tumor of vascular endothelial cells. The pathogenesis is closely linked to abnormalities in the vascular endothelial growth factor signaling pathway. Multiple treatment modalities are available, though wide local excision remains the preferred treatment. Bevacizumab, an anti-vascular endothelial growth factor monoclonal antibody, has known efficacy against hemangiomas, though there are no cases of sinonasal hemangiomas managed with bevacizumab. METHODS:: Case report. RESULTS:: The authors review the case of a 67-year-old man with a left-sided nasal hemangioma originating from the nasal septum. He presented with progressive left nasal obstruction and recurrent epistaxis. At the time of his presentation, the lesion had recurred after 1 excision/polypectomy at an outside institution. He then underwent revision surgery via wide local excision and septoplasty. After tumor recurrence following the revision surgery, the hemangioma was noted to recur on 1 year postoperative surveillance nasal endoscopy. In-office intralesional injection of 50 mg bevacizumab was then performed under endoscopic visualization. No improvement in the tumor size was noted at 2 months after injection, with the tumor measuring 1.5 cm. At the 10-month surveillance clinical visit following injection, the tumor had dramatically involuted to 3 mm in greatest dimension. The patient reported complete resolution of his primary symptoms of epistaxis and nasal obstruction. CONCLUSIONS:: This report demonstrates the first reported successful treatment of a sinonasal hemangioma with intralesional bevacizumab. Intralesional bevacizumab confers an additional option for adjuvant treatment of sinonasal hemangiomas. Further evaluation of intralesional bevacizumab in the treatment of these tumors is warranted.

Expression profile of circular RNAs in infantile hemangioma detected by RNA-Seq.

BACKGROUND: Circular RNAs (circRNAs) have emerged as a novel class of widespread non-coding RNAs, and they play crucial roles in various biological processes. However, the characterization and function of circRNAs in infantile hemangioma (IH) remain elusive. METHODS: In this study, we used RNA-Seq and circRNA prediction to study and characterize the circRNAs in IH tissue and a matched normal skin control. Specific circRNAs were verified using real-time polymerase chain reaction. RESULTS AND CONCLUSION: We found that of the 9811 identified circRNAs, 249 candidates were differentially expressed, including 124 upregulated and 125 downregulated circRNAs in the IH group compared with the matched normal skin control group. A set of differentially expressed circRNAs (in particular, hsa_circRNA001885 and hsa_circRNA006612 expression) were confirmed using qRT-PCR. Gene ontology and pathway analysis revealed that compared to matched normal skin tissues, many processes that were over-represented in IH group were related to the binding, protein binding, gap junction, and focal adhesion. Specific circRNAs were associated with several micro-RNAs (miRNAs) predicted using miRanda. Altogether, our findings highlight the potential importance of circRNAs in the biology of IH and its response to treatment.

Ultrasonographic and Imaging Appearance of Peripheral Intraneural Vascular Anomalies: Report of Two Cases and Review of the Literature.

The purpose of the paper is to present the ultrasonographic and imaging appearance of two cases of peripheral nerve intraneural vascular anomalies and provide a comprehensive review of the publications on this subject.The clinical presentation, ultrasonographic appearance, corresponding imaging and outcome of a case of ulnar nerve venous malformation and a case of median nerve arteriovenous malformation are presented.Literature search revealed 35 papers presenting 52 cases of vascular anomalies involving the peripheral nerves. The ultrasonographic appearance was described only in ten cases. Our review suggests that peripheral intraneural vascular anomalies are twice more frequent in women. About three quarters of them are located in the upper limb, with the median nerve involved in one third and the ulnar nerved involved in a quarter of all cases. Most of the cases are hemangiomas. Peripheral neural compartment syndromes in patients with coexisting vascular anomalies may prompt for vascular neural involvement, requiring diagnostic imaging studies. Gray-scale and Doppler ultrasound are the methods of first choice, as they provide not only direct visual proof of neural involvement but also contribute to the differential diagnosis between hemangiomas and vascular malformations.