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Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma. In this study, we further our understanding and morphologic spectrum of NFATC -fusion positive vascular neoplasms by describing 9 new cases, including soft tissue locations and novel fusion partners. Combining with the initial cohort of 5 cases, a total of 14 patients were analyzed, showing slight female predilection and an age range of 10 to 66 (mean 42 y). Twelve patients had solitary lesions, while 2 had multifocal polyostotic (pelvic bones) disease. Overall, 12 lesions were intra-osseous and 2 in soft tissue. By targeted RNA Fusion panels or FISH, there were 6 cases of EWSR1::NFATC1 , 4 EWSR1::NFATC2 , 2 FUS::NFATC2 , 1 EWSR1 rearrangement, and 1 with a novel FABP4::NFATC2 fusion. Follow-up was available in 4 patients. One patient experienced 2 local recurrences, 11 and 15 years postdiagnosis, and one patient experienced progressive disease despite multimodality treatment (curettings, embolization, radiation) over 3 years. In summary, our extended investigation confirms that NFATC -related fusions define a distinct group of vascular neoplasms with variable architecture, epithelioid phenotype, and cytologic atypia, commonly located in the bone, occasionally multifocal and with potential for local recurrence and aggressive behavior but no metastatic potential. Molecular analysis is recommended in diagnostically challenging cases with atypical histology to exclude malignancy., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [ Pediatr Ann . 2024;53(4):e129-e137.] .

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are nonproliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement. These simple malformations include capillary, lymphatic, venous, and arteriovenous malformations. The pediatric hematologists and oncologists are becoming increasingly involved in the diagnosis and management of these disorders. This review presents four cases as a means to discuss the diagnosis, clinical and imaging features, and management strategies of simple, localized vascular malformations., (© 2021 Wiley Periodicals LLC.)

Vascular tumors are a rare subset of vascular anomalies. These are classified based on their malignant potential or local destruction potential. Classification has been historically difficult and treatment recommendations are based on case series. The purpose of this chapter is to review the presentation, pathologic and imaging characteristics. Treatment recommendations are summarized based on the current literature. Congenital and infantile hemangiomas are covered separately in a separate chapter in this issue., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose, (Copyright © 2020. Published by Elsevier Inc.)

Background/objectives: Congenital hemangiomas (CH) are a group of benign vascular tumors that are present at birth and exhibit variable involution during infancy. Congenital hemangiomas that do not involute are typically solitary patch or plaque-type tumors that grow proportionally with somatic growth. We report a case series of 9 patients with persistent CH, which exhibited uncommon features including segmental involvement, recurrent or severe pain, or growth via volumetric increase in size or apparent increased extent of anatomic involvement over time., Methods: Via retrospective chart review, we included patients with persistent CH and atypical presentations. Available data regarding clinical characteristics, natural history, histopathology, imaging, and genetic tests were collected., Results: Data on 9 patients were collected, including 7 noninvoluting CH and 2 partially involuting CH. Three of the 9 cases had segmental distribution, 6 had apparent growth or clinical evolution, and 4 were symptomatic with pain. One also had marked localized intravascular coagulopathy., Conclusions: Ongoing or recurrent pain and large extent of anatomic involvement can be features of CH, albeit uncommon ones, and can pose both diagnostic and management challenges. Tissue genomic studies can offer a novel tool for CH diagnosis., (© 2019 Wiley Periodicals, Inc.)

Competing Interests: Competing interests: None declared.

Objective: Vascular anomalies in the neonatal period are a diagnostic challenge for the lack of evident signs, symptoms and follow-up, and the convenience of restricting aggressive diagnostic tests. The aim of this work is to review the characteristics of neonatal cases presented to our Vascular Anomalies Unit in the last 5 years., Materials and Methods: All cases of suspected vascular anomaly presented to our unit before 1 month of age between 2010 and 2015 were reviewed, diagnostic tests and treatments carried out with chronology were analyzed. Presumptive diagnosis and final diagnosis (when available) were compared., Results: Fifteen vascular tumors were found, 2 with visceral involvement: 6 infantile hemangiomas (IH), 3 NICH, 4 RICH, 1 tufted hemangioma, 1 unspecified liver vascular tumor, 3 venous malformations (2 equivocal MRI and a hyperkeratotic venous malformation), 4 lymphatic malformations, 3 of them macrocystic, and 2 vascular lesions that were diagnosed of fibrosarcoma and sclerema neonatorum and they were not vascular anomalies. Only 3 patients with macrocystic lymphatic malformations had prenatal diagnosis., Conclusion: Accurate diagnosis of vascular anomalies during the first month of life is difficult, even with MRI. Only in a few cases early treatment is needed, so it is worth taking time to follow-up. Different types of treatment (observation, propranolol, biopsy, laser, embolization, and resection) will depend on the condition to be treated. A continuous observation can avoid unnecessary procedures and risks.

Superficial vascular anomalies are a heterogeneous group of diseases, developed from various types of vessels and differentiated by their clinical presentation, progression, and prognosis. Their classification was first published by Mulliken and Glowacki in 1982, then endorsed by the International society of the study of vascular anomalies (ISSVA) in 1996 and updated in 2014. It separates vascular anomalies into two categories: vascular tumors characterized by cellular proliferation and vascular anomalies that are structural anomalies of blood vessels. Infantile hemangiomas (IHs) are the most frequent childhood vascular tumor. In most cases, IHs are small, involute progressively with or without a few sequelae, and do not require any treatment. However, in particular localizations or in complicated hemangiomas, treatment is essential to limit tumor growth and accelerate their involution. Propranolol is now the first-line treatment. Its efficacy and safety have been clearly demonstrated., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

We present a case of non-involuting congenital haemangioma (NICH) of the right eyelid which was present at birth as a purpuric macule but increased in size to cause significant obstruction of vision. At four years of age the lesion was treated with fluroscopic ultrasound-guided sclerotherapy using 0.5% sodium tetradecyl suphate foam and surgically debulked 16 days later. Histopathology was negative for glucose transporter-1 stain confirming the diagnosis. The residual segments were subsequently treated in three further sessions of sclerotherapy in the ensuing three years. This treatment approach resulted in a good cosmetic and functional outcome with no associated complications. To our knowledge, this is the first published case of a histologically confirmed NICH treated primarily with sclerotherapy.

Vertebral hemangiomas (VHs) are common lesions in the adult population. They are usually asymptomatic and found incidentally on radiological imaging. New-onset back pain followed by subacute progression of thoracal myelopathy is the most common presentation in patients with neurological deficit. Differential diagnoses would include metastasis, multiple myeloma, lymphoma, Paget disease, osseous tumors such as Ewing sarcoma or hemangioblastoma and blood dyscrasia. We present a 41 year-old-male patient with thoracal VH causing myelopathy that completely improved after rehabilitation program with embolization and vertebroplasty procedures.

Infantile hemangiomas (IHs) are the most common vascular tumors of childhood, affecting ~5% of all infants. Although most lesions proliferate and then involute with minimal consequence, a significant minority can be disfiguring, functionally significant, or, rarely, life-threatening. Recent discoveries concerning hemangioma pathogenesis provide both an improved understanding and more optimal approach to workup and management. Important detrimental associations can be seen with IH, such as significant structural anomalies associated with segmental IH. Standards of care have dramatically changed evaluation and management of hemangiomas. The goal of timely recognition and therapy is to minimize or eliminate long-term sequelae. New modalities, such as oral propranolol, provide the caregiver with better therapeutic options, which can prevent or minimize medical risk or scarring, but the side effect profile and risk-benefit ratio of such interventions must always be evaluated before instituting therapy.

We report a case of esophageal cancer in a patient with a racemose hemangioma of the bronchial arteries. A 76-year-old man was admitted to the hospital with unexplained weight loss. Endoscopic examination revealed enhancing vessels in the upper to middle esophageal wall and a type 3 tumor in the lower esophagus. He underwent neoadjuvant chemotherapy with cisplatin and 5-fluorouracil and underwent a lower esophageal resection through a left thoracoabdominal incision. He was free from hemoptysis and had no recurrence of esophageal cancer for 1 year following the procedure. This case is not only rare, it supports the decision to perform an operative procedure.

A hallmark of infantile hemangioma, the most common tumor of infancy, is its dramatic growth after birth, by diffuse proliferation of immature endothelial cells, followed by spontaneous regression. The growth and involution of infantile hemangioma is quite different from other vascular anomalies, which do not regress and can occur at any time during life. Some hemangioma lesions can be extremely disfiguring and destructive to normal tissue and may even be life-threatening. Unfortunately, existing therapeutic approaches have limited success and significant adverse effects of some treatment modalities limit their use. Better understanding of the pathogenesis of hemangioma will enable the development of better therapeutic strategies. Herein, we review recent studies and new hypotheses on the pathogenesis of the tumor. Detailed mechanisms of activated vascular endothelial growth factor (VEGF) signaling in tumor cells, identification of their origin and characterization of multipotent stem cells that can give rise to infantile hemangioma are shedding new light on this intriguing vascular tumor., (© 2010 Japanese Dermatological Association.)

Vascular tumours (haemangiomas and malformations) are common tumours of infancy and childhood. They represent a group of mostly benign conditions, which present early, can grow rapidly and be symptomatic or disfiguring. There are various management options, with different cosmetic and functional outcomes. Haemangiomas and vascular malformations have different clinical courses which dictate respective management; differentiating them is key. Haemangiomas are generally self-limiting after initial proliferation; generally management is conservative. Symptoms can call for treatment. Options include laser, steroids, chemotherapy and surgery. Propanolol, the newest modality of treatment, may have a dramatic effect in problematic lesions. Vascular malformations are a less common group of heterogenous lesions, with some overlap between entities. They do not involute. Treatment options include laser therapy, sclerotherapy, embolisation, and surgery. Emerging therapies include photodynamic therapy and angiogenesis inhibitors. This review will outline the evidence for the various modalities in managing these conditions., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

A 55-year-old man was admitted to our hospital with chronic cough. Although his chest X-ray was normal, chest computed tomography revealed a nodular lesion that was causing beaded stenosis of the lumen of the upper lobe bronchus. Nodules approximately 10 mm in size were seen in the mediastinum. We could not deny the possibility of vascular disease, and enhanced chest computed tomography was performed, which showed these abnormalities to be caused by a dilated and convoluted bronchial artery. Because no pulmonary diseases that would lead to secondary vascular dilatation were evident, a diagnosis of primary racemose hemangioma of the bronchial artery was made. We performed bronchial artery embolization to prevent potential fatal bleeding from vascular rupture. The patient has been followed regularly as an outpatient, and recanalization has not been detected. Primary racemose hemangioma of the bronchial artery is a rare entity; it is also rare for this entity to be detected by findings of a nodular protrusion in the bronchial wall or in a patient presenting with a cough. Here, we report this case and review previous reports.

A 54-year-old male presented with haemoptysis. Bronchial arteriography revealed a bent, meandering and dilated bronchial artery with vascular hyperplasia in multiple locations. The patient was diagnosed as having primary racemose haemangioma of the bronchial artery. Using a microcatheter, TorconNB (5 Fr) and Progreat (2.7 Fr), selective gelfoam embolization of the descending branch of the right bronchial artery was performed using the double catheter method. This approach would allow effective treatment of the haemorrhage and avoid spinal cord injury.

Racemose hemangioma of the bronchial arteries is a rare abnormality and is characterized by enlarged and convoluted bronchial arteries arranged segmentally along the longitudinal axis of bronchus. Primary racemose hemangioma may arise from inborn malformation, and secondary one may develop following primary inflammatory, stenosing or deforming diseases of the bronchus, the peribronchial tissues and the surrounding lung tissues. The symptom at onset is usually hemoptysis. Although the typical treatments have not been established, various treatments, like embolization or ligation of the bronchial arteries and surgical resection of the involved area of lung, were reported. However the term 'Racemose hemangioma' seems to be used only in Japan, so it is favorable to make a consensus of its definition among countries.

Hemangiomas of infancy (HOIs) are among the most common benign tumors of childhood and classically appear as a vascular stain or small vascular papule at birth. They are unique tumors due to their propensity to proliferate, involute, and finally regress. These lesions can be associated with visceral malformations that have been shown to affect mainly the liver and the gastrointestinal tract, but rarely the spinal cord. The authors report a rare case of a spinal HOI in a 2-month-old infant presenting with quadriplegia due to intratumoral hemorrhage. Following resection of the lesion, the child regained function. This first reported case of spinal HOI suggests another location for hemangiomatosis. Spinal HOI should be included in the differential diagnosis of acute intraspinal hemorrhage in infants.

A 22-year-old woman was admitted to our hospital because of hemoptysis and respiratory insufficiency. The chest roentgenogram and the chest computed tomogram showed infiltrative shadows in the bilateral lower lobes and mediastinal emphysema. On the second day of hospitalization, we performed double lumen endotracheal tube intubations for the repeated life-threatening hemoptysis. Bronchoscope examination revealed normal bronchus with fibrin formation. Bronchial autobiography (BAG) showed a convoluted and enlarged right bronchial artery and bronchial-pulmonary artery shunt. We diagnosed primary racemose hemangioma of a bronchial artery and performed bronchial artery embolism (BAE) of the right upper bronchial artery using coil. There was no reccurence of hemoptysis after BAE procedure. BAE with coil seems to be effective for life-threatening hemoptysis due to racemose hemangioma.

Vascular anomalies are a complex pathological group. They are especially difficult to study because of confusion in the terminology used. The classification developed by the ISSVA (International Society for the Study of Vascular Anomalies) now allows using a common scientific language. The classification is based on clinical, radiological, hemodynamic, and histological arguments. There are two groups of lesions: vascular tumors and vascular malformations. Vascular tumors are associated to vascular proliferation. They are called hemangioma and can be infantile or congenital. Vascular malformations are associated to vessels with morphologic anomalies. They are classified according to the distorted vessel type, capillary, venous, lymphatic, and arteriovenous). Such a classification has many implications. It is a guide for the orientation of radiological exams and treatment of vascular anomalies. The management of these anomalies is still difficult and must involve an interdisciplinary approach.

A 69-year-old man had an abnormal shadow on chest X-ray and bronchoscopic examination showed that left B4 was completely occluded by a tumor. A non-pulsatile polypoid nodule was also found in right B'. The tumor in the left B4 was diagnosed as carcinoid, but the nodule in right B' was suspected to be hemangioma and biopsy was not performed. Bronchial arteriography revealed hypervascularization with dilated vessels distributing to the lingular lobe and convoluted and a dilated bronchial artery extending to the right upper lobe. From these findings, we diagnosed racemose hemangioma of the bronchial artery of the right upper lobe. After bronchial artery embolization of the right and left bronchial arteries, he underwent segmentectomy of the lingula and was discharged without complications. Two months after the operation, bronchoscopic examination showed that the racemose hemangioma had shrunk and the swelling in the surrounding mucosa had decreased. If a submucosal small nodule is observed during bronchoscopy, biopsy should be performed after bronchial arteriography, and if the nodule is diagnosed as racemose hemangioma, bronchial artery embolization should be performed.

We encountered a case of primary racemose hemangioma treatment with successful bronchial artery embolism for massive hemoptysis. A 56-year-old woman with massive hemoptysis was transported to our hospital. The source of the massive hemoptysis was observed to be from around a non-pulsatile polyp covered by normal mucosa occluding the truncus intermedius by fiberoptic bronchoscopy. We stopped the bleeding temporarily using differential lung ventilation, and then bronchial artery angiography was performed. The main right bronchial artery was enlarged, and enlarged and convoluted right peripheral bronchial vessels were also observed. We diagnosed the massive bleeding to be due to racemose hemangioma. A successful bronchial artery embolization (BAE) was performed with gelforms and metallic coils for the treatment of racemose hemangioma. There has been no recurrence of hemoptysis for one year after BAE. There have been many reports on massive hemoptysis as in this patient who were treated by lobectomy, nevertheless we would like to state BAE should be considered as a suitable treatments for primary racemose hemangioma with hemoptysis if there is no recognizable shunt artery.

We report the clinicopathological features of two cases of intravascular large B-cell lymphoma involving cutaneous hemangiomas. The cases were identified from the consultation files of two of the authors. Both patients were women, 64 and 55 years of age, who presented with long-standing cutaneous hemangiomas of the posterior scalp and left shoulder, respectively. The lesions were brought to medical attention by an increase in size and change in color. Biopsies and immunohistochemical evaluation of the hemangiomas revealed extensive involvement by intravascular large B-cell lymphoma. The neoplastic cells were diffusely positive for CD20 in both cases and negative for CD3, pan-cytokeratin (AE1/AE3), epithelial membrane antigen, S-100, Factor VIII-related antigen, CD34 and CD31. Disease was limited to the hemangiomas in both patients. Treatment consisted of chemotherapy (both patients) and adjuvant radiation therapy (one patient). One patient had a recurrence of disease 33 months after initial diagnosis, leading to an autologous stem cell transplant. The other patient is without evidence of disease 27 months after initial diagnosis. Although this is a rare neoplasm, it is important to consider intravascular large B-cell lymphoma in the differential diagnosis of vascular lesions containing intravascular neoplastic cells.

We report two cases of primary racemose hemangioma in patients with hemoptysis. In the first, a 59-year-old man had a pulsatile polypoid lesion in the bronchus of the lingula; and in the second, a 24-year-old woman had a nodule in the bronchus of the right lower lobe. In both patients, arteriography of the bronchial artery revealed enlargement and convolution of its branches. Primary racemose hemangioma was diagnosed in both patients. The arteriography also revealed a shunt between the pulmonary and bronchial arteries in the woman. Bronchial artery embolization (BAE) was an effective treatment for the hemoptysis. To date, eight months after the embolization, hemoptysis has not recurred. Bronchoscopy and arteriography of the bronchial artery are both useful for diagnosing primary racemose hemangioma. BAE seems to be effective in treating primary racemose hemangioma.

An hemangioma is a common benign growth of vascular endothelium that may have multiple clinical manifestations. Either identified at birth or shortly thereafter, hemangiomas have both proliferative and involutional phases. Certain varieties are associated with congenital anomalies. Numerous therapy options are discussed.

The typical vascular anomalies (tumors and vascular malformations) that involve the liver in infants and children are summarized. Many of these lesions are complex and require multiple imaging modalities, often including angiography, for precise diagnosis.

Lymphangioma and venous angioma are a group of pathological entities which have required surgical treatment since years. Extension, localization and poor delimitation of some lesions have resulted in technical difficulties and serious complications. Our group began the sclerosing therapy with an injection of fibrin adhesive and, due to the lack of response in the first patient, a program with Ethibloc as an alternative substance was developed. We present 10 cases, 4 lymphangiomas and 6 venous angiomas. In 50% of our patients one single session of sclerotherapy was made, in 25% two sessions, and in the rest 25% three. From 10 cases, 4 are still on treatment, complete remission in 5 cases, and good evolution in one case (mixed lymphangioma). As a long-term complication, we have noticed fistulization in 3 cases, and expulsion of the sclerosing agent. In our experience, percutaneous sclerosis with Ethibloc should be the first therapeutic alternative.