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We present a case of a 62-year-old female who was incidentally found to have a venous malformation. Venous malformations are part of a larger category of slow flow vascular malformations and are associated with various familial syndromes and localized intravascular coagulation. Venous malformations were often misdiagnosed as hemangiomas; however, the treatment modalities of vascular malformations and hemangiomas vary significantly. Here we elucidate the imaging findings of venous malformations from various vascular tumors and other malformations.

Abstract Background Retroperitoneal infantile hemangioma (RIH), a type of primary retroperitoneal tumors, are exceptionally rare in clinical practice. Infantile hemangiomas typically manifest on the skin’s surface. RIHs are exceptionally rare and typically small. In adults, these tumors often manifest without specific clinical symptoms or detectable signs for a definitive diagnosis. This case report details a patient diagnosed with RIH. We recommend complete excision of the tumor after a comprehensive evaluation, followed by postoperative pathology, to achieve a conclusive diagnosis. We believe that managing critical retroperitoneal structures and vessels intraoperatively presents a significant challenge for all procedures involving primary retroperitoneal tumors. Case summary A 47-year-old male was diagnosed with gallstones and underwent surgery 3 months ago at other institution for unexplained nausea and vomiting. Follow-up imaging 2 months after surgery revealed a retroperitoneal mass below the left renal pole. Upon presentation to our hospital, the patient continued to experience intermittent nausea and vomiting, with no other significant symptoms or signs. Considering the patient’s 8-year history of hypertension, a paraganglioma was initially suspected. We performed the laparoscopic mass resection after a detailed assessment. However, postoperative pathology revealed it a capillary hemangioma (old term)/infantile hemangioma. Conclusion RIHs are exceedingly rare benign tumor. The possibility of malignancy should be ruled out, and surgical resection is recommended following a thorough evaluation, with the diagnosis confirmed through pathological examination.

Background: Retroperitoneal infantile hemangioma (RIH), a type of primary retroperitoneal tumors, are exceptionally rare in clinical practice. Infantile hemangiomas typically manifest on the skin's surface. RIHs are exceptionally rare and typically small. In adults, these tumors often manifest without specific clinical symptoms or detectable signs for a definitive diagnosis. This case report details a patient diagnosed with RIH. We recommend complete excision of the tumor after a comprehensive evaluation, followed by postoperative pathology, to achieve a conclusive diagnosis. We believe that managing critical retroperitoneal structures and vessels intraoperatively presents a significant challenge for all procedures involving primary retroperitoneal tumors. Case summary: A 47-year-old male was diagnosed with gallstones and underwent surgery 3 months ago at other institution for unexplained nausea and vomiting. Follow-up imaging 2 months after surgery revealed a retroperitoneal mass below the left renal pole. Upon presentation to our hospital, the patient continued to experience intermittent nausea and vomiting, with no other significant symptoms or signs. Considering the patient's 8-year history of hypertension, a paraganglioma was initially suspected. We performed the laparoscopic mass resection after a detailed assessment. However, postoperative pathology revealed it a capillary hemangioma (old term)/infantile hemangioma. Conclusion: RIHs are exceedingly rare benign tumor. The possibility of malignancy should be ruled out, and surgical resection is recommended following a thorough evaluation, with the diagnosis confirmed through pathological examination. [ABSTRACT FROM AUTHOR]

Pediatric hemangiomas are benign vascular malformations or angiodysplasias characterized by the proliferation of endothelial cells in blood vessels. Their incidence is 4%–10% in children under 1 year of age, and they most frequently occur on the head and neck [ 1 , 2 ]. In addition to causing facial deformities, facial hemangiomas can lead to both functional and aesthetic issues, often resulting in significant complications. These complications negatively impact the quality of life for patients and can also affect the psychological well-being and self-esteem of their families [ 3 ]. With the expected global smartphone penetration reaching 70% [ 4 ], this case report explores the use of smartphones integrated with thermographic cameras to monitor the involution of hemangiomas during sclerosing treatment. This approach represents a promising advancement in managing and assessing hemangioma treatment through accessible, real-time monitoring technology. [ABSTRACT FROM AUTHOR]

This narrative review aims to summarise the classification of vascular anomalies, their clinical presentation, and their radiological features to propose a diagnostic algorithm to approach patients with suspected soft tissue vascular anomalies of the extremities. The management of vascular anomalies necessitates a multidisciplinary approach. Clinical presentation and physical examination are sufficient in most cases to achieve a correct diagnosis. This is especially true for small congenital lesions of the skin and subcutaneous tissue. Imaging is used for accurate characterization of these lesions, especially in cases of atypical or vague clinical presentation, and to assess extension in cases of lesions that are larger and localized in deeper tissues. [ABSTRACT FROM AUTHOR]

A basic understanding of vascular anomalies will aid physicians-in-training as they seek to properly diagnose and determine interventions for these patient presentations. The aim of this review is to create a resource for physicians in training that encompasses the most important clinical aspects of vascular anomalies. Vascular anomalies of the head and neck are divided into two categories: vascular tumors and vascular malformations. This review will first describe vascular tumors followed by vascular malformations. This review discusses major pathology found in both categories of vascular anomaly. Vascular anomalies are frequently found in pediatric populations and can persist into adulthood, making it important for trainees to identify them on physical exam. This developing field seeks to improve form, function, and quality of life for patients with vascular anomalies and often requires a multidisciplinary approach (i.e., otolaryngology, dermatology, genetics, plastic surgery, interventional radiology). Various medical and surgical treatment options are available. A basic knowledge of these anomalies will allow for accurate, early diagnosis and appropriate intervention which can ultimately improve patient outcomes. Search Strategy and Selection Criteria: The MEDLINE/PubMed database was searched for primary research and reviews discussing various vascular anomalies which include infantile hemangioma, congenital hemangioma, pyogenic granuloma, tufted angioma, kaposiform hemangioendothelioma, capillary malformations, lymphatic malformations, venous malformations, and arteriovenous malformations. This search was conducted from 7/5/2023 to 3/21/2024.

The burden of treatment (BOT) related to propranolol treatment for infantile hemangiomas (IH) has never previously been explored. A modified validated questionnaire, the Treatment Burden Questionnaire, and one‐on‐one semi‐structured interviews were used to assess the BOT for propranolol for IH. Out of 80 caregivers, the overall burden score was very low at 1.2 out of 10; thematic analysis of interviews grouped themes into administration, monitoring, financial, and associated anomalies. The BOT of propranolol for IH is very low but could be reduced further by offering age‐based risk stratification related to feeding frequency and risk of hypoglycemia, pragmatic advice around timing of doses before sleep, and reducing frequency of vital sign monitoring. [ABSTRACT FROM AUTHOR]

Abstract Nanotechnology has demonstrated immense potential in various fields, especially in biomedical field. Among these domains, the development of nanotechnology for diagnosing and treating vascular anomalies has garnered significant attention. Vascular anomalies refer to structural and functional anomalies within the vascular system, which can result in conditions such as vascular malformations and tumors. These anomalies can significantly impact the quality of life of patients and pose significant health concerns. Nanoscale contrast agents have been developed for targeted imaging of blood vessels, enabling more precise identification and characterization of vascular anomalies. These contrast agents can be designed to bind specifically to abnormal blood vessels, providing healthcare professionals with a clearer view of the affected areas. More importantly, nanotechnology also offers promising solutions for targeted therapeutic interventions. Nanoparticles can be engineered to deliver drugs directly to the site of vascular anomalies, maximizing therapeutic effects while minimizing side effects on healthy tissues. Meanwhile, by incorporating functional components into nanoparticles, such as photosensitizers, nanotechnology enables innovative treatment modalities such as photothermal therapy and photodynamic therapy. This review focuses on the applications and potential of nanotechnology in the imaging and therapy of vascular anomalies, as well as discusses the present challenges and future directions. Graphical Abstract

Abstract Background Epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin which can be primarily be seen in various tissues. EHE originating from the pleura is an even more uncommon subtype which may mimic mesothelioma and pleural carcinomatosis. The prognosis of pleural EHE is poor and there is no consensus on the optimal therapeutic approach. Case presentation A 39-year-old middle-eastern female presented with progressive dyspnea and left shoulder discomfort. Chest computed tomography scan revealed a left side pleural effusion and pleural thickening. Pleuroscopy was done and biopsies were taken which were positive for CD31, CD34, CK, factor 8-R-antigen, and vimentin. Patient was diagnosed with pleural epithelioid hemangioendothelioma (PEHE) and chemotherapy was started and underwent extrapleural pneumonectomy 7 months later. Unfortunately, the patient passed away 10 months after diagnosis due to disease complications. Conclusions Once PEHE is suspected in histology it can be confirmed with immunohistochemistry. Chemotherapy, surgery or a combination of both is currently used as the treatment but the standard treatment remains a question.

Intramuscular hemangiomas, although considered rare, represent a unique subset of vascular tumors that arise within the skeletal muscle tissue. These benign neoplasms are characterized by the proliferation of blood vessels within the muscle fibers, and their clinical presentation can vary widely, often posing diagnostic challenges for clinicians. The management of intramuscular hemangiomas is often dictated by the lesion’s size, location, and symptomatic impact on the patient. The present case report aims to contribute to the evolving understanding of intramuscular hemangiomas by presenting a comprehensive analysis of a recent clinical case, discussing diagnostic modalities, and highlighting management strategies that can aid health-care professionals in their approach to this rare vascular entity. [ABSTRACT FROM AUTHOR]

Nanotechnology has demonstrated immense potential in various fields, especially in biomedical field. Among these domains, the development of nanotechnology for diagnosing and treating vascular anomalies has garnered significant attention. Vascular anomalies refer to structural and functional anomalies within the vascular system, which can result in conditions such as vascular malformations and tumors. These anomalies can significantly impact the quality of life of patients and pose significant health concerns. Nanoscale contrast agents have been developed for targeted imaging of blood vessels, enabling more precise identification and characterization of vascular anomalies. These contrast agents can be designed to bind specifically to abnormal blood vessels, providing healthcare professionals with a clearer view of the affected areas. More importantly, nanotechnology also offers promising solutions for targeted therapeutic interventions. Nanoparticles can be engineered to deliver drugs directly to the site of vascular anomalies, maximizing therapeutic effects while minimizing side effects on healthy tissues. Meanwhile, by incorporating functional components into nanoparticles, such as photosensitizers, nanotechnology enables innovative treatment modalities such as photothermal therapy and photodynamic therapy. This review focuses on the applications and potential of nanotechnology in the imaging and therapy of vascular anomalies, as well as discusses the present challenges and future directions. [ABSTRACT FROM AUTHOR]

Background: Epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin which can be primarily be seen in various tissues. EHE originating from the pleura is an even more uncommon subtype which may mimic mesothelioma and pleural carcinomatosis. The prognosis of pleural EHE is poor and there is no consensus on the optimal therapeutic approach. Case presentation: A 39-year-old middle-eastern female presented with progressive dyspnea and left shoulder discomfort. Chest computed tomography scan revealed a left side pleural effusion and pleural thickening. Pleuroscopy was done and biopsies were taken which were positive for CD31, CD34, CK, factor 8-R-antigen, and vimentin. Patient was diagnosed with pleural epithelioid hemangioendothelioma (PEHE) and chemotherapy was started and underwent extrapleural pneumonectomy 7 months later. Unfortunately, the patient passed away 10 months after diagnosis due to disease complications. Conclusions: Once PEHE is suspected in histology it can be confirmed with immunohistochemistry. Chemotherapy, surgery or a combination of both is currently used as the treatment but the standard treatment remains a question. [ABSTRACT FROM AUTHOR]

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal neoplasm of intermediate malignant potential showing endothelial differentiation. Around 20 cases of primary osseous PHE have been reported to date. A 16-year-old boy presented with complaints of pain in his right leg. Imaging revealed multifocal intramedullary and cortical-based lytic lesions involving long and small bones. Microscopic examination revealed plump, spindled cells arranged in fascicles and admixed “epithelioid” and “rhabdoid” cells sans vasoformative areas. By immunohistochemistry, the lesional cells were reactive for AE1/AE3, CD31, Erg, Fli1, and SMA, while immunonegative for CD34, myogenin, and S100. Nuclear expression of the INI1/SMARCB1 protein was retained. PHE is a rare entity, more so as a primary osseous lesion; therefore, awareness of the presence of this entity in the bone is the key to making a diagnosis. We discuss its clinicopathological features, differential diagnosis, and an attempt a short review of the literature.

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

Conjunctival capillary hemangioma is very rare, with few cases being reported in the literature. We present a case of a 60-year-old male with conjunctival capillary hemangioma, a unique case adding to the literature of uncommon vascular tumor. The patient had a mass over the left eye for 20 years, without any associated systemic diseases or cutaneous manifestations. The lesion was imaged with anterior-segment optical coherence tomography and the findings have been presented and the diagnosis was confirmed by histopathology.

Epithelioid angiomatous nodule (EAN) is a rare benign vascular lesion that was first reported in 2004. This article presents the case of a 29-year-old male with multiple focal EANs on his penis, a highly uncommon location for this condition. The patient had been experiencing painful nodules for 6 months, initially misdiagnosed as hemangioma and later as epithelioid hemangioendothelioma. Physical examination revealed bluish, well-circumscribed lesions on the corona and glans penis. Microscopic evaluation of an incisional biopsy confirmed the diagnosis of a penile EAN. EAN falls within the spectrum of epithelioid vascular tumors, but it is considered a newly described acquired vascular proliferation. It typically presents as small, fast-growing nodules on the extremities and trunk, though it can also occur on the face and mucosal surfaces. While most cases are solitary, conglomerated multiple nodules may also be seen. The condition predominantly affects adults between 15 and 45 years old. Differential diagnoses for EAN include epithelioid hemangioma, epithelioid angiosarcoma, and bacillary angiomatosis. EAN is clinically benign but can significantly impact the patient's quality of life, especially when located on sensitive areas like the penis. Simple local excision is a curative method, though reconstructive procedures may be necessary in some cases. Due to the limited number of cases reported in the literature, the pathogenesis and possible causes of EAN remain unclear. Further research and increased study of this condition are needed to gain a better understanding of its nature and potential etiology. Accurate diagnosis is crucial to distinguish EAN from other malignant vascular pathologies and prevent unnecessary interventions.

Background: Anastomotic hemangioma is a rare subtype of capillary hemangioma primarily found in the genitourinary tract. We present a case of a patient with an anastomotic hemangioma located in the retroperitoneal space; then, we explore and summarize the imaging features from previously reported cases for accurate diagnosis. Case presentation: A 57-year-old woman complained of left lower back pain. Contrast-enhanced ultrasound revealed a hypoechoic mass with “slow-in and slow-out” enhancement. Abdominal CT scan displayed a well-defined, round soft tissue mass in the right retroperitoneal region with obvious enhancement. MRI indicated low signal on T1-weighted imaging, high signal on T2-weighted imaging and diffusion-weighted imaging, and progressive enhancement after enhancement. Surgical removal of the tumor was performed. Histopathological examination exhibited a distinct tumor border with interconnected blood vessels and a cavity lined by a single layer of cubic endothelial cells. Immunohistochemistry confirmed the presence of CD31[+] and CD34[+]. The final pathological diagnosis was anastomotic hemangioma. No recurrence was observed during a 40-month follow-up. Conclusion: Retroperitoneal anastomotic hemangioma is a rare and benign neoplasm that may be misdiagnosed as ectopic pheochromocytoma or angiosarcoma. This case report presents and analyzes the imaging characteristics of a series of retroperitoneal anastomotic hemangiomas, which can be valuable for future diagnoses and help prevent unnecessary surgeries. [ABSTRACT FROM AUTHOR]

The International Society for the Study of Vascular Anomalies (ISSVA) has devised a classification system. The most recent version of this classification system was published in 2018. This classification appears widely used and splits vascular malformations into high- and low-flow lesions. The high-flow lesions are further subdivided into arteriovenous malformations (AVM) and arteriovenous fistulas. Low-flow lesions are subdivided into capillary malformations (CM), venous malformations (VM) and lymphatic malformations (LM). The ISSVA classification then describes mixed lesions; for example, lymphovenous malformations. It also has a category for vascular tumors. There are benign vascular tumors, also known as hemangiomas, local aggressive, or malignant tumors. [ABSTRACT FROM AUTHOR]

Kaposiform haemangioendothelioma (KHE) is a rare vascular tumor, occurring predominantly in infancy, with an incidence of 0.091 per 100,000 children. Typically presenting as a solitary large cutaneous lesion with no distant metastases. Children with KHE are at high risk of developing the Kasabach-Merritt phenomenon (KMP), a consumptive coagulopathy. KMP is defined as profound thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. KMP has a mortality of 10 - 30%. Due to its rarity, there is little robust evidence for KHE management. Rapamycin (mTOR) inhibitors are a safe and effective treatment. This report is of a newborn full-term infant with no antenatal complications, presenting with abdominal cutaneous vascular malformation and, subsequently, coagulopathy. [ABSTRACT FROM AUTHOR]

Background: Knowledge on the management of kaposiform hemanigioendothelioma (KHE) with kasabach-merritt phenomenon (KMP) rely on single case reports, case series and a few retrospective studies. Case report: A 3-month old girl was admitted for a violaceous, palpable mass in the right posterior neck. Laboratory studies showed thrombocytopenia and consumptive coagulopathy with hypofibrinogenemia and raised Dimer-D values. A diagnosis of KHE with KMP was confirmed histopathologically. Treatment regimen for KMP was commenced with oral prednisone and salicylic acid, followed by oral sirolimus at 1 mg/m2/day. It led to an involution of the vascular lesion at 12-month follow-up. Conclusion: The case contributes with another example of KHE with KMP that was effectively treated with the novel mTOR inhibitor. Prospective studies for sirolimus are urged and clinical guidelines are yet to be defined.

Composite hemangioendothelioma (CHE) is a rare locally aggressive vascular neoplasm of intermediate malignancy. We describe a 2‐year‐old patient diagnosed with this tumor. Careful documentation of patients with CHE is crucial to better define the prognosis and treatment of this entity. [ABSTRACT FROM AUTHOR]

This narrative review aims to summarise the classification of vascular anomalies, their clinical presentation, and their radiological features to propose a diagnostic algorithm to approach patients with suspected soft tissue vascular anomalies of the extremities. The management of vascular anomalies necessitates a multidisciplinary approach. Clinical presentation and physical examination are sufficient in most cases to achieve a correct diagnosis. This is especially true for small congenital lesions of the skin and subcutaneous tissue. Imaging is used for accurate characterization of these lesions, especially in cases of atypical or vague clinical presentation, and to assess extension in cases of lesions that are larger and localized in deeper tissues.

Epithelioid angiomatous nodule (EAN) is a rare benign vascular lesion that was first reported in 2004. This article presents the case of a 29‑year‑old male with multiple focal EANs on his penis, a highly uncommon location for this condition. The patient had been experiencing painful nodules for 6 months, initially misdiagnosed as hemangioma and later as epithelioid hemangioendothelioma. Physical examination revealed bluish, well‑circumscribed lesions on the corona and glans penis. Microscopic evaluation of an incisional biopsy confirmed the diagnosis of a penile EAN. EAN falls within the spectrum of epithelioid vascular tumors, but it is considered a newly described acquired vascular proliferation. It typically presents as small, fast‑growing nodules on the extremities and trunk, though it can also occur on the face and mucosal surfaces. While most cases are solitary, conglomerated multiple nodules may also be seen. The condition predominantly affects adults between 15 and 45 years old. Differential diagnoses for EAN include epithelioid hemangioma, epithelioid angiosarcoma, and bacillary angiomatosis. EAN is clinically benign but can significantly impact the patient’s quality of life, especially when located on sensitive areas like the penis. Simple local excision is a curative method, though reconstructive procedures may be necessary in some cases. Due to the limited number of cases reported in the literature, the pathogenesis and possible causes of EAN remain unclear. Further research and increased study of this condition are needed to gain a better understanding of its nature and potential etiology. Accurate diagnosis is crucial to distinguish EAN from other malignant vascular pathologies and prevent unnecessary interventions. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic has caused significant shifts in patient care including a steep decline in ambulatory visits and a marked increase in the use of telemedicine. Infantile hemangiomas (IH) can require urgent evaluation and risk stratification to determine which infants need treatment and which can be managed with continued observation. For those requiring treatment, prompt initiation decreases morbidity and improves long-term outcomes. The Hemangioma Investigator Group has created consensus recommendations for management of IH via telemedicine. FDA/EMA-approved monitoring guidelines, clinical practice guidelines, and relevant, up-to-date publications regarding initiation and monitoring of beta-blocker therapy were used to inform the recommendations. Clinical decision-making guidelines about when telehealth is an appropriate alternative to in-office visits, including medication initiation, dosage changes, and ongoing evaluation, are included. The importance of communication with caregivers in the context of telemedicine is discussed, and online resources for both hemangioma education and propranolol therapy are provided.

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

Background: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors. Methods: We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment. Results: We included 327 patients with a mean age at diagnosis of 9.1 months (SD ± 9). Patients were treated with an mTOR inhibitor for a mean of 15.2 months (SD ± 4.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach–Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1). Conclusion: This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first-line therapy. [ABSTRACT FROM AUTHOR]

Abstract Background Lymphangiomas are benign vascular tumors arising from the lymphatic system. They commonly affect the head and neck regions. Pancreatic involvement is extremely rare. Even though they are rare, it should be considered as a differential diagnosis for patients who present with pancreatic mass. Case presentation We report the case of a 6-year-old African male patient who presented with abdominal mass and dull aching pain of 6 months duration. He was examined and underwent excision, with biopsy showing pancreatic lymphangioma. Conclusion These tumors are benign and slow growing and have potential to regress spontaneously. Thus, most literature agrees that surgical interventions should be reserved for symptomatic cases.

Infantile hemangiomas (IHs) are the most common tumors of infancy and, in rare instances, can present in the setting of congenital structural anomalies or as part of syndromic disorders. In this study, we present three cases of children with segmental IHs born with concurrent pulmonary anomalies: congenital pulmonary airway malformations and bronchopulmonary sequestration. To date, no known association between these entities and hemangiomas has been described. [ABSTRACT FROM AUTHOR]

Capillary hemangioma is a benign, vascular tumor characterized by proliferation of blood vessels that are primarily reported to be a developmental hamartomatous lesion of infancy and childhood. Till date, many cases of capillary hemangiomas have been reported in the literature, but juvenile hemangiomas with other systemic manifestations such as hepatitis B have not been studied well so far. The present paper reports a rare case of capillary hemangioma in a ten-year-old female with hepatitis B, which was treated under general anesthesia using electrocautery and followed up for one year and no recurrence was found.

Aim: Hemangioendotheliomas (HEs) are malignant vascular tumors with sparse descriptions in literature owing to their rarity. Study design: Ours is a retrospective study among patients of advanced HEs registered between September 2015 and April 2021. Results: There were 13 patients with median age 34.6 (range: 4–69 years), male preponderance (69%) and predominant subtype of epithelioid HE (76.9%). Common primary sites were viscera (46.2%) and bone (30.8%). Tyrosine kinase inhibitors (TKIs) yielded objective responses in 30% patients whereas chemotherapy only produced disease stabilization in 7.7%. Conclusion: We recognize an aggressive subset of HEs with manifestations such as acute liver failure and splenic rupture. Currently no biomarkers predict the efficacy of TKIs over chemotherapy; however, TKIs showed promising outcomes in this series.

Abstract A 55 year‐old woman presented with an invasive hemangioma of the hard palate. One year after partial surgical excision there was no sign of recurrency or oroantral fistulation. No malignant cells could be identified.

Copyright of China Journal of Oral & Maxillofacial Surgery is the property of Shanghai Jiao Tong University, College of Stomatology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Even though vascular anomalies of the hand are rare entities (7%), they are relevant regarding soft tissue mass differential diagnosis on the hand. 2 The majority of cases tend to be malformations, once denominated as deep soft tissue hemangiomas. 1,3 Due to evolving knowledge regarding vascular anomalies, the prognosis, guidelines regarding treatment and aftercare following therapy/surgery will be more accurate and detailed if the correct diagnosis is established. Retrospective data collection and analysis between 2008 and 2019, from the Orthopedic Department of Semmelweis University tumor registry. Comparison to the current literature (PubMed, Ovid). Study level of evidence III. Standardly distributed data with confidence level of 95%. N = 16, average age of 34,2. 63,5% of lesions were digital. Hemangiomas accounted for 67%. The accuracy of clinical vs. histological diagnosis was 77%. Recurrences 25%. Variable follow up period. Clinical examination, radiological evaluation, patient history and fine needle biopsy are often accurate guides. Nevertheless in our study, histological analysis of the surgical biopsy was the most effective method in establishing a definite diagnosis. Conservative and watchful waiting approach are the first line, and when complemented with appropriate imaging, should suffice until absolute or relative operative indications are present. [ABSTRACT FROM AUTHOR]

Background: The classification of vascular anomalies includes terms of nomenclature that are not based on histogenesis resulting in confusion among health professionals of different specialties. Ongoing efforts to classify them properly have taken place. This literature review aimed to identify erroneous nomenclature of vascular anomalies and to investigate their continued use over the past four years after the last International Society for the Study of Vascular Anomalies (ISSVA) update. Methods: Literature research was based on pertinent classifications (ISSVA, WHO) and books related to vascular anomalies and soft tissue pathology. After identifying twelve entities with confusing terminology, new research in the Pubmed database was conducted to verify their continued use in the last four years. Results: The literature review highlighted terms referring to vascular malformations as neoplasms. In addition, terms used as equivalents represent entirely different entities. On the other hand, different terms to characterize the same entity were also recorded. Furthermore, regardless of the last ISSVA update in 2018, terms that are only descriptive or do not correspond to vascular anomaly histogenesis are consistently used. Conclusion: Despite intensive efforts in the last decades for correct terminology and classification of vascular anomalies, modifications are still required. A common and broadly accepted scientific terminology should be applied, accurately representing histogenesis or pathogenesis, to obtain a common language among medical specialists, given that a multidisciplinary approach is crucial for managing vascular anomalies. [ABSTRACT FROM AUTHOR]

Capillary hemangioma is a benign, vascular tumor characterized by proliferation of blood vessels that are primarily reported to be a developmental hamartomatous lesion of infancy and childhood. Till date, many cases of capillary hemangiomas have been reported in the literature, but juvenile hemangiomas with other systemic manifestations such as hepatitis B have not been studied well so far. The present paper reports a rare case of capillary hemangioma in a ten-year-old female with hepatitis B, which was treated under general anesthesia using electrocautery and followed up for one year and no recurrence was found. [ABSTRACT FROM AUTHOR]

Introduction: Epithelioid hemangiomas (EHs) are rare vascular lesions which generally affect the skin and subcutaneous tissue but rarely seen in bones. It is a benign entity but intermediate grade, i.e., locally aggressive in nature. It has very confusing clinicoradiological and histopathological features which make diagnosis difficult and help us to avoid inappropriate treatment., Case Report: We present with a 32-year-old male with multifocal EH involving the distal radius, a few carpal bones (trapezium, trapezoid, and capitate), and the base of 2nd and 3rd metacarpals with extensive surrounding soft-tissue involvement. He was managed with intralesional extended curettage + bone grafting and is disease free till follow-up., Conclusion: As EH is a rare entity, this comes as a differential diagnostic in locally aggressive-looking lytic lesions in the bone which helps the treating orthopedic surgeons or orthopedic oncosurgeons to have this differential in their mind for a better diagnosis and further management., Competing Interests: Conflict of Interest: Nil, (Copyright: © Indian Orthopaedic Research Group.)