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We report two cases with localized vascular malformations clinically resembling the "dominant lesion" seen in capillary malformation‐arteriovenous malformation (CM‐AVM) syndrome, however, lacking germline RASA1 variants but presenting double somatic RASA1 variants in affected tissue. Both patients presented with localized and superficial high‐flow vascular malformations were treated with surgery and laser therapy and showed partial resolution. The study underscores the rarity of somatic RASA1 variants, contributes to understanding the "second‐hit" pathophysiology in vascular lesions, and emphasizes the significance of clinical distinctions and genotyping for accurate diagnoses, offering implications for diagnosis, prognosis, and genetic counseling. [ABSTRACT FROM AUTHOR]

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

Pulmonary artery intimal sarcoma (PAIS) is a rare and aggressive malignancy originating from the intimal layer of the pulmonary artery with poor prognosis due to its aggressive nature. The management of PAIS poses both diagnostic and therapeutic challenges. It presents with nonspecific symptoms and is often misdiagnosed as pulmonary embolism. While surgical resection is the primary treatment modality, the role of adjuvant chemotherapy and radiotherapy remains uncertain. However, given the high recurrence rate, adjuvant chemotherapy and/or radiotherapy have been utilized in a limited number of case reports. We present the case of a 46-year-old woman who was diagnosed with PAIS and underwent surgical resection followed by adjuvant chemotherapy (ChT) and radiotherapy (RT), demonstrating good tolerance to this multimodal treatment approach. [ABSTRACT FROM AUTHOR]

Infantile hemangioma (IH) is a common condition in children. It may lead to cosmetic disfigurement, functional impairment, and various complications. There are various treatment options available for this condition. This article aims to discuss the various therapeutic options available for the treatment of IH. We searched PubMed, Google Scholar, and Embase (from December 2013 to December 2023) for human studies/case reports/case series assessing the management of IH. We reviewed 198 papers including different therapeutic regimens. β-blockers other than propranolol, such as nadolol, timolol, atenolol, acebutolol, and captopril, have shown comparable results to propranolol. Pulsed dye laser (PDL) and neodymium-doped yttrium aluminum garnet laser have shown promising results. Other therapies observed were sclerotherapy, cryotherapy, and surgical resection. A conventional combination of β-blockers with a laser might be the first-line treatment to arrest the growth of IH. PDL has shown promising results.

Intravascular papillary endothelial hyperplasia (IPEH), also called masson tumor which is a benign vascular tumor named after Pierre Masson, the French pathologist who originally described it in 1923, terming it “hémangioendothéliome végétant intravasculaire.” It is characterized by a reactive proliferation of endothelial cells associated with thrombosis. Ultrasound and MRI are the main imaging examinations, but the diagnosis of IPEH was confirmed by biopsy pathology and immunohistochemistry. It is generally accepted that surgical excision is the first choice of treatment. In this report, we report a case of Masson tumor arising in the right wrist.

Upper arm glomus tumors are uncommon, benign vascular growths originating from skin glomus bodies. They typically cause severe, paroxysmal pain triggered by cold, pressure, or touch. Diagnosis can be complex due to mimicry of other conditions, but histopathological examination confirms the presence of glomus cells. In a case study, a 46-year-old woman with an Upper arm glomus tumor experienced pain and a lump in her left arm. Ultrasound identified a well-circumscribed, heterogeneous, hypoechoic, pedunculated mass with internal vascularity and MRI showed a lobulated soft-tissue mass with skin involvement and polypoid projection, alongside axillary lymphadenopathy and skin thickening. Following an open biopsy that confirmed the diagnosis of a glomus tumor, the patient experienced complete resolution of symptoms after surgical excision, with no recurrence during the 24-month follow-up period.

Infantile hemangioma is a benign vascular tumor, the most common in childhood, whose natural evolution is the disappearance of the lesion in the pediatric age and which has effective and safe treatments that limit its growth and favor its disappearance at younger ages. Infantile hemangioma continues to be a reason for attention to complications, due to erroneous diagnoses, lack of knowledge of the condition, late referral or fear of the effects of the medications used for its treatment. Furthermore, its presence is normalized without taking into account that it can cause uncertainty, anxiety, feelings of guilt and, as a consequence, a significant impact on the quality of life, mainly in the parents or caregivers of the child. The need for a clinical practice guideline in our country arises from the high presentation of late-remitted complications in infantile hemangioma even with the availability of adequate treatments, the continuous evolution of medicine and the appearance of new evidence. Throughout the guide you will find recommendations regarding the diagnosis, treatment and follow-up of patients with infantile hemangioma, taking into account the paraclinical tests that can be performed, topical or systemic management options, as well as adjuvant therapies. For the first time, objective tools for patient follow-up are included in a guide for the management of infantile hemangioma, as well as to help the first contact doctor in timely referral. [ABSTRACT FROM AUTHOR]

Hemangiomas account for only 0.4% to 0.6% of all parotid tumors, making them extremely rare in adults. Unlike pediatric parotid hemangiomas, those in adults typically present as asymptomatic swellings of the parotid, have no skin discoloration, and usually do not regress spontaneously. Therefore, an accurate diagnosis of parotid hemangiomas in adults before surgical excision is generally challenging. Herein, we present 3 cases of adult parotid hemangiomas. The patients all received parotidectomies with tumor resection. Histopathological analysis of the resected specimens revealed numerous dilated, thin- or thick-walled (small, large, or variably sized) vessels lined with flattened endothelial cells. A diagnosis of cavernous hemangioma of the parotid gland was established only after the histopathological analysis. Parotid cavernous hemangiomas in adults are rare and often misdiagnosed before surgical resection. Clinical presentation and imaging features on ultrasonography, computed tomography, magnetic resonance imaging, and angiography may support an accurate preoperative diagnosis. Surgical resection proved a suitable treatment approach for our 3 cases. [ABSTRACT FROM AUTHOR]

This article discusses the dermoscopic findings of pseudomyogenic hemangioendothelioma (PHE), a rarely metastasizing vascular tumor. The case report describes a 45-year-old man with PHE on his back, presenting as two painful lesions with unclear borders and ulcerated surfaces. Dermoscopic features included a red background, bright yellow structureless areas, scattered scales, white radial streaks, brown streaks in a "polygonal" shape, thick arborizing vessels, and clusters of small serpentine vessels. The article also provides information on the clinical features, histopathological characteristics, and treatment options for PHE. This study is the first to report on the dermoscopic features of PHE. [Extracted from the article]

Vascular tumours (VT) with Kasabach-Merritt phenomenon (KMP) are rare and aggressive tumors. In absence of evidence based treatment guidelines, we studied varied presentation and response to therapy with vincristine and steroids in VT with KMP at our center. In this retrospective observational study, infants with a symptomatic/disfiguring rapidly growing VT with features of KMP were included. Demographic, treatment and outcome data was retrieved from patient file. Complete response (CR) was defined as complete clinical regression of VT with normalization of coagulopathy and thrombocytopenia. Partial response (PR) was defined as decrease in size of VT by more than 80%, absence of clinical bleed with normalization of coagulopathy and platelet count > 50,000/cumm. Five infants (2-male, 3-female) with age range (0–7 month) treated with daily prednisolone and weekly vincristine were included. The location of VT was: face (2), hemi-thorax (2) and urinary bladder (1). Four of five infants showed PR within two months; while two of these attained CR to treatment. There were no significant adverse effects over 9–32 (range) month follow-up. Two children (one in PR, one immediately after presentation) succumbed to intra-cranial hemorrhage. Combination therapy of steroids with vincristine is effective and safe in management of VT with KMP. [ABSTRACT FROM AUTHOR]

Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor with a high risk of mortality. Few studies with large samples of KHE have been reported. KHE may develop into the Kasabach-Merritt phenomenon (KMP), which is characterized by thrombocytopenia and consumptive coagulopathy. The features of severe symptomatic anemia and life-threatening low platelets make the management of KHE associated with KMP challenging. Objective: The aim of this study was to examine the clinical characteristics of patients with KHE and discuss the treatment experience for different risk groups of KHE. Methods: Through a retrospective review of 70 patients diagnosed with KHE between 2017 and 2022 in our center, we classify lesions into three clinicopathological stages based on the tumor involving depth, and divided the severity of KHE into three levels by estimating clinicopathological stages and severity of thrombocytopenia. Treatments of different severity groups were estimated with sufficient data. Results: In our cohort, 27% were neonates, and KHE lesion occurred at birth in 84% of patients. There was a slight male predominance (32 girls and 38 boys). Common clinical characteristics included associated coagulation disorder (100%), locally aggressive cutaneous blue-purple mass (89%), thrombocytopenia (78%), and local pain or joint dysfunction (20%). The lower extremities were the dominant location (35%), followed by the trunk (29%), the maxillofacial region and neck (24%), and the upper extremities (10%). Of the total cohort, 78% developed KMP; the median age at which thrombocytopenia occurred was 27.8 days. The median platelet count of patients who were associated with KMP was 24,000/µL in our cohort. Ninety-two percent of patients were given surgery treatment and 89% of these patients were given high-dose methylprednisolone (5-6 mg/kg daily) before surgery. In 55 patients with KMP, 36% were sensitive to high-dose corticosteroid therapy. Patients from the low-risk group (eight cases) underwent operation, all of whom recovered without recurrence after a maximum follow-up of 5 years. Out of 26 patients from the highrisk group, 25 underwent surgery treatment, with 1 case undergoing secondary surgery after recurrence and 1 case taking sirolimus. Out of 36 cases from the extremely high-risk group, 32 underwent surgery (including 2 cases who underwent external carotid artery ligation and catheterization), 3 of whom underwent secondary operation after recurrence, and the remaining 4 cases took medicine. The mean length of having sirolimus was 21 months; two cases stopped taking sirolimus due to severe pneumonia. Two cases died at 1 and 3 months after discharge. Conclusions: Our study describes the largest assessment of high-risk patients with KHE who have undergone an operation to date, with 5 years of follow-up to track recovery, which provides invaluable knowledge for the future treatment of patients with KHE and KMP from different risk groups: Early surgical intervention may be the most definitive treatment option for most patients with KHE; multimodality treatment is the best choice for the extremely high-risk group. [ABSTRACT FROM AUTHOR]

Congenital cervicofacial vascular anomalies are extremely rare and present many difficulties in diagnosis and treatment requiring a multidisciplinary approach. Firstly, there is little consensus on this subject among head and neck specialists. There are two main types of vascular anomalies: vascular tumors and vascular malformations. Vascular malformations are also divided into malformations with slow blood flow (veins, lymphatics, capillaries or combined) and malformations with a fast blood flow (arteriovenous malformations and fistula). Vascular tumors like hemangiomas are known for their spontaneous involution with aging, while vascular malformations grow in dimensions with age. It is very important to choose the correct differential diagnosis between cervicofacial hemangiomas and vascular malformations for proper therapy management. Anamnesis and clinical exams help in raising suspicions about the real nature of a cervico-vascular anomaly. Furthermore, imaging brings in-depth details of the anomaly, ranging from ultrasound and contrast CT to MRI scanning and minimally invasive angiography. Angiography with selective embolization is rarely a curative procedure for arteriovenous malformations, being more suitable as a preliminary step before attempted surgical removal. Surgery is clearly necessary when there are aesthetic and functional deficits. Slow-flow vascular malformations present a reduced morbidity, and in cases without involution, the surgical ablation is reserved for the cases with aesthetic dysfunctions or psychological trauma. Lymphatic malformations must undergo surgical ablation when they are associated with mass effects and compression of great vessels or aerial viscera. The prognosis after surgical removal is good, with a low rate of recurrence or morbidity. Fast-flow vascular malformations require a combined approach, with embolization and excision in the next 48 h for safety reasons. Removal may be followed by reconstructive surgery depending on the location and dimensions of the malformation, with a possible secondary recovery of the normal microscopic vessels. Some of the masses may hinder the normal airflow and swallowing. Pathology is the gold standard for confirming the clinical and imaging diagnosis. [ABSTRACT FROM AUTHOR]

Soft tissue vascular anomalies may be composed of arterial, venous, and/or lymphatic elements, and diagnosed prenatally or later in childhood or adulthood. They are divided into categories of vascular malformations and vascular tumors. Vascular malformations are further divided into low-flow and fast-flow lesions. A low-flow lesion is most common, with a prevalence of 70%. Vascular tumors may behave in a benign, locally aggressive, borderline, or malignant manner. Infantile hemangioma is a vascular tumor that presents in the neonatal period and then regresses. The presence or multiple skin lesions in an infant can signal underlying visceral vascular anomalies, and complex anomalies may be associated with overgrowth syndromes. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation. [ABSTRACT FROM AUTHOR]

Background: Masson's tumor, commonly referred to as intravascular papillary endothelial hyperplasia (IPEH), is an uncommon growth of endothelial cells within a vessel wall that is frequently assumed to indicate an abnormal resolution of thrombosis. IPEH is most typically found in the extremities however it is rare for IPEH to appear as a neck tumor. The issue with IPEH is that it could clinically, radiologically, and pathologically imitate some malignant neoplasms such as angiosarcomas creating a diagnostic challenge. Case Report: We describe a 21-year-old male patient who presented with right anterolateral neck swelling for 12 months. Ultrasound revealed a 9.0 × 8.0 cm well-defined echogenic hyper-vascular lesion. The contrast computed tomography (CT) scan of the neck revealed an oval, well-defined subcutaneous mass, measuring 9 × 4.5 cm, situated over and separable from the right sternocleidomastoid muscle with no significant enhancement in the post-contract study. T1-weighted and T2-weighted MRI revealed a 10 × 9 × 7 cm well-defined subcutaneous lobulated lesion superficial to the sternocleidomastoid expanding upward to the Rt. side of the cheek and below to the suprasternal region, eliciting an intermediate signal in T1 and a heterogenous bright signal (mostly fluid) in T2 with low signal foci within the mass. The decision had been reached to entirely excise the lesion surgically with safety margins for histological evaluation. Histological examination indicated thrombosed variable-sized ectatic vascular spaces with papillary formations related to the thrombus, covered with a single layer of flat endothelium, and no features suggestive of malignancy. There was no recurrence at 18 months follow-up post-surgery. Conclusion: Masson's tumor is a benign intravascular disease with an unclear origin and no confirmed inheritance pattern. Presentation of Masson's tumor as a neck mass is incredibly uncommon. Masson's tumor lacks a distinct or distinguishing clinical and radiological appearance. Histopathologic examination is the sole definitive way for diagnosing the disease and the only tool for distinguishing it from angiosarcoma. Surgical excision is the best treatment for IPEH. Recurrence is extremely rare. [ABSTRACT FROM AUTHOR]

Haemangiopericytoma, an uncommon vascular tumor originating from perivascular cells, encompasses a sinonasal variant known as glomangiopericytoma (GPC). A 75-year-old female presented with complaints of recurrent episodes of bleeding from the left nasal cavity for 8 months, along with a history of recurrent episodes of headaches and left nasal obstruction for 6 months. DNE was done and showed a polypoidal vascular mass filling the left nasal cavity. CECT PNS was done, showing signs of sinonasal malignancy. Biopsy showed features of low-grade spindle cell carcinoma. Debulking of the tumour followed by FESS with medial maxillectomy was done successfully, with currently no signs of recurrence.

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

Introduction: Infantile hemangioma (IH) is a benign tumor of the vascular endothelium, characterized by early appearance, rapid growth, and slow involution. It is the most common tumor in the pediatric age group, and several risk factors are associated with its development. Case Report: Monozygotic twins, female, 29 weeks premature and with low birth weight, presenting reddish spots that after 15 days evolved into erythematous tumors, with a diagnosis of IH. Conclusion: This case report exemplifies simultaneously the main risk factors associated with the evelopment of IH. Recognition of these factors by pediatricians is crucial for early diagnosis, appropriate follow-up, and management.

Background: Vascular anomalies are a group of disorders that are divided into two categories: tumors and malformations. Given the vast diversity of clinical presentations with overlapping signs and symptoms, diagnosis can be difficult. Objective: We conducted a cross-sectional study to evaluate the common cutaneous vascular anomalies encountered in our outpatient department. Materials and Methods: A total of 28 patients suspected to be having cutaneous vascular anomalies were screened and subjected to biopsy. The diagnosis was based on clinicopathological correlation. Results: In our study, pyogenic granuloma was the most common vascular anomaly seen in 25% of patients; majority of these patients gave a history of trauma and spontaneous bleeding. It was followed by angiokeratoma (17.8%), infantile hemangioma, port-wine stain, and verrucous hemangioma which was seen in 10.7% of patients. Other common vascular anomalies included are glomus tumor and tufted angioma. Conclusion: Vascular anomalies comprise a spectrum of diseases, diagnosis of which is often challenging. Accurate diagnosis is critical to generate a management plan for the patient. Our study highlights the varied clinical spectrum of vascular tumors that we come across in clinical practice and it will increase familiarity of the various clinical presentations, thereby enabling clinicians to better identify and manage these challenging entities.

Ameloblastoma, a benign epithelial odontogenic tumor, has an intriguing feature by virtue of its diverse histopathologic variants. One such variant that is not yet acclimatized in the literature due to the paucity of cases is “hemangiomatous ameloblastoma” (HA). HA exhibits the histology of conventional ameloblastoma with marked vascularity, which resembles the enamel organ and dental sac. Herein, we present the case of a 33-year-old male patient with swelling of the lower left back region of the jaw for 6 months, which on imaging presented multilocular radiolucency. Incisional biopsy was suggestive of ameloblastoma, and an excisional procedure showed odontogenic epithelial islands with marked vascular proliferation, based on which the diagnosis of HA was rendered. The rarity of this histologic variant and lack of long-term follow-up records in the literature challenges the clinician to predict its biological behavior. Hence, further research is required to understand the same.

Objective: Kaposi’s sarcoma (KS) is a vascular proliferation associated with Human Herpes Virus 8. Typical histopathological findings of KS are characterized by vascular proliferation, inflammatory cell infiltration, extravasated erythrocytes and spindle cell proliferation, although it varies according to the stage. In this study, the clinical-histopathological features of patients with KS were examined. The value of histopathological parameters in the diagnosis was investigated. Methods: Patients with KS diagnosed in University of Health Sciences Turkey, Samsun Training and Research Hospital Medical Pathology Department between 2016-2020 were retrospectively scanned. Clinical and tumor features and histopathological changes in the surrounding tissue were evaluated. Results: The most common histopathological features belonging to tumor were extravasated erythrocytes, spindle cell changes, fascicle formation, slit-like space; the most common epidermal features were hyperkeratosis and acanthosis; the most common peritumoral features were the presence of large vessels and ectatic vessels in the periphery. There was a significant relationship between the promontory sign and the lymphangioma-like area and ulcer. Also there was a significant relationship between nuclear atypia and lymphangioma-like area. Conclusion: While most of the histopathological features are characteristic for KS, none of them alone is specific. They should be evaluated together with all structural, tumoral and peritumoral features during the diagnostic approach.

Objective: Kaposi’s sarcoma (KS) is a vascular proliferation associated with Human Herpes Virus 8. Typical histopathological findings of KS are characterized by vascular proliferation, inflammatory cell infiltration, extravasated erythrocytes and spindle cell proliferation, although it varies according to the stage. In this study, the clinical-histopathological features of patients with KS were examined. The value of histopathological parameters in the diagnosis was investigated. Methods: Patients with KS diagnosed in University of Health Sciences Turkey, Samsun Training and Research Hospital Medical Pathology Department between 2016-2020 were retrospectively scanned. Clinical and tumor features and histopathological changes in the surrounding tissue were evaluated. Results: The most common histopathological features belonging to tumor were extravasated erythrocytes, spindle cell changes, fascicle formation, slit-like space; the most common epidermal features were hyperkeratosis and acanthosis; the most common peritumoral features were the presence of large vessels and ectatic vessels in the periphery. There was a significant relationship between the promontory sign and the lymphangioma-like area and ulcer. Also there was a significant relationship between nuclear atypia and lymphangioma-like area. Conclusion: While most of the histopathological features are characteristic for KS, none of them alone is specific. They should be evaluated together with all structural, tumoral and peritumoral features during the diagnostic approach. [ABSTRACT FROM AUTHOR]

Ameloblastoma, a benign epithelial odontogenic tumor, has an intriguing feature by virtue of its diverse histopathologic variants. One such variant that is not yet acclimatized in the literature due to the paucity of cases is "hemangiomatous ameloblastoma" (HA). HA exhibits the histology of conventional ameloblastoma with marked vascularity, which resembles the enamel organ and dental sac. Herein, we present the case of a 33-year-old male patient with swelling of the lower left back region of the jaw for 6 months, which on imaging presented multilocular radiolucency. Incisional biopsy was suggestive of ameloblastoma, and an excisional procedure showed odontogenic epithelial islands with marked vascular proliferation, based on which the diagnosis of HA was rendered. The rarity of this histologic variant and lack of long-term follow-up records in the literature challenges the clinician to predict its biological behavior. Hence, further research is required to understand the same. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin. It is frequently seen in the liver, lungs and bones. Cases with mastoid bone and intracranial extension are rarer. A 40 year old male, presented with a history of progressive headaches and a mass in the postauricular region for a duration of three months. Computer tomography of the temporal bone showed an approximately 40 × 30 mm soft tissue with osteolytic bony changes located in the mastoid bone. Epithelioid hemangioendothelioma is a rare sarcoma of vascular origin. Surgery is the first line of therapy. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. [ABSTRACT FROM AUTHOR]

Reiva Farah Dwiyana,1 Elisabet Risubekti Lestari,1 Raden Mohamad Rendy Ariezal Effendi,1 Srie Prihianti Gondokaryono,1 Inne Arline Diana,1 Hermin Aminah Usman,2 Kamelia Utami Suhada,1 Rafithia Anandita1 1Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital Bandung, West Java, Indonesia; 2Department of Anatomical Pathology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital Bandung, West Java, IndonesiaCorrespondence: Reiva Farah Dwiyana, Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Jl. Pasteur 38, Bandung, West Java, 40161, Indonesia, Tel +62222032426 ext. 3449, Fax +62222032426, Email reiva@unpad.ac.idIntroduction: Tufted angioma (TA) is a rare benign vascular tumor usually occurring in the first year of life. It may present as reddish papules or purplish red to black plaques. Tender lesions commonly affect the neck, upper trunk, and shoulders. Histologic tufts of capillaries infiltrating the dermis in a “cannon ball” distribution pattern confirm the diagnosis. However, effective treatments for TA are scarce. Pulsed dye laser (PDL) is an alternative therapy for TA, particularly concerning cosmetics and pain relief. This case report demonstrates the effectiveness of PDL as a treatment for TA.Case: We report a 15-year-old girl with five-year history of painful purplish red plaques on her left cheek, neck, chest, left shoulder, and back. Histopathological examination from skin biopsy showed discrete “cannon ball” pattern in the dermis. Laboratory examination revealed normal platelet count and fibrinogen level. Based on these presentations, the diagnosis of TA without complications was made. We treated the lesion using 595-nm PDL with 6.5− 10 J/cm2 fluence of, 1.5 ms pulsed duration, and 5− 7 mm spot size every three weeks. Reductions in redness and pain were seen after four sessions of treatment.Discussion: PDL promotes selective vascular damage with minimal injury to the surrounding skin. Capillary tufts in TA could therefore serve as a target for laser treatment, which may result in fading redness and pain reduction.Conclusion: PDL is effective in reducing redness and pain in TA.Keywords: pulse dye laser, tufted angioma, vascular tumor

ABSTRACT Infantile hemangiomas are the most common benign vascular tumors in children. They present a characteristic natural history of spontaneous involution after a phase of initial proliferation. A small but significant minority demonstrates incomplete regression or complications and requires prompt intervention. Prediction of the evolution of infantile hemangiomas is challenging because of their morphological and behavioral heterogeneity. The decision between referral for treatment and observation is sometimes difficult, especially among non‐expert physicians, with the risk of missing the period for optimizing outcomes in case of delayed intervention. The aim of this review is to update our knowledge, especially of the primary care providers, regarding the ongoing difficulties of the early clinical evaluation of infantile hemangiomas, and to outline the importance of current practical scoring tools for the identification of the lesions which require expert consultation and referral.

Giant carotid body tumors, defined as those >8 cm in size, are extremely rare. Definitive surgical management is a complex undertaking because these large tumors tend to have grown to envelop cranial nerves and the carotid artery, and few data exist regarding the long-term outcomes for these patients. We present the case of a patient with bilateral giant carotid body tumors who underwent staged embolization and excision of a >10-cm carotid body tumor. After 5 years of follow-up, we demonstrated that elective open repair can provide long-term symptomatic relief. We describe and illustrate the crucial steps and considerations regarding the excision of complex Shamblin type III carotid body tumors.

BackgroundManaging complex vascular anomalies in pediatric care requires comprehensive approaches. Sirolimus, an mTOR inhibitor with immunosuppressive and anti-angiogenic properties, offers promise. We evaluated sirolimus's effectiveness and safety in pediatric patients with complex vascular anomalies at a tertiary children's hospital.MethodsOur study included 20 patients, aged 1 month to 19 years, with diverse vascular anomalies resistant to conventional therapies or located in high-risk areas precluding surgery. The evaluation of response encompassed measuring the reduction in the size of the targeted vascular or lymphatic lesions as observed on radiologic imaging, along with considering improvements reported by the patients.ResultsPatients used sirolimus for a median of 2.1 years, ranging from 0.6–4.3 years. Results indicated that 60% of patients achieved complete or partial response (CR/PR), whereas 40% had stable disease (SD). Notably, no disease progression occurred. Lesion size assessment was complex, yet patients' self-reported improvements were considered. Three patients reinitiated sirolimus after discontinuation due to worsening lesions. Sirolimus treatment demonstrated good tolerability, with minor complications except for one case of Pneumocystis jiroveci pneumonia. Group comparisons based on response highlighted better outcomes in patients with vascular tumors (CR/PR group 58.0% vs. SD group 0.0%, P = 0.015) or localized measurable lesions (83.3% vs. 12.5%, P = 0.005).ConclusionOur study underscores sirolimus's potential for treating complex vascular anomalies in pediatric patients. Challenges associated with optimal treatment duration and concurrent interventions necessitate a comprehensive approach and genetic testing to optimize outcomes.

Objective: Rare coexistence of disease or pathology Background: Hemangiomas are defined as benign soft tissue vascular tumors that are histologically classified as capillary, cavernous, or mixed types. Hemangiomas can also be described based on clinical appearance as superficial, mixed, or deep lesions. Following a thorough search, only 3 case reports of superficial protruding lip mass were found in the literature. Other cases of tongue hemangioma have been reported in infants or young toddlers, and only rarely in adults. Case Reports: The first case was a 43-year-old pregnant woman, with an unremarkable medical and surgical history, in the second trimester who presented to the Otolaryngology Clinic with a chief concern of a progressively growing lesion, measuring 0.7×0.5 cm, over the lateral right side of the tongue for the last 2 weeks after accidentally biting her tongue during dinner. The second case was a 26-year-old woman with unremarkable medical and surgical history who presented to our Otolaryngology Clinic with a chief concern of a non-painful soft fungating pink-red lip lesion 1.5×1 cm across the right lower lip growing for the last 4 months. This lesion appeared during the third trimester of pregnancy following a lip injury that was described as minor trauma. Conclusions: Although hemangiomas can occur anywhere on the body, they are most commonly found in the head and neck. These lesions are usually recognized quickly by patients and treating physicians and are thus clinically diagnosed. Most vascular benign lesions regress on their own, but if detected early, they are surgically excised for cosmetic and functional reasons. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas are the most common benign vascular tumors in children. They present a characteristic natural history of spontaneous involution after a phase of initial proliferation. A small but significant minority demonstrates incomplete regression or complications and requires prompt intervention. Prediction of the evolution of infantile hemangiomas is challenging because of their morphological and behavioral heterogeneity. The decision between referral for treatment and observation is sometimes difficult, especially among non‐expert physicians, with the risk of missing the period for optimizing outcomes in case of delayed intervention. The aim of this review is to update our knowledge, especially of the primary care providers, regarding the ongoing difficulties of the early clinical evaluation of infantile hemangiomas, and to outline the importance of current practical scoring tools for the identification of the lesions which require expert consultation and referral. [ABSTRACT FROM AUTHOR]

Abstract Background Non-involuting congenital hemangiomas (NICHs) are fully formed vascular tumors at birth with distinctive clinical, radiologic, and histopathological profiles. In the literature, there is no effective therapy strategy for patients with NICH except surgery. Currently, no cell line or animal model exists for studying the mechanism of NICH and drug validation. We plan to construct a new strategy by constructing NICH organoids for further study. Result Here, we report a novel NICH organoid system construction and optimization process. Both HE and immunohistological staining exactly matched NICH tissue. We further performed transcriptome analysis to elucidate the characteristics of NICH organoids. Both NICH tissue and NICH organoids manifested similar trends in download sites. NICH organoids display novel features to new cells derived from organoids and show spectacular multiplication capacity. In the preliminary verification, we found that cells splitting from NICH organoids were human endothelial cells. Drug validation demonstrated that trametinib, sirolimus, and propranolol showed no inhibitory effects on NICH organoids. Conclusion Our data show that this new NICH-derived organoid faithfully captured the features of this rare vascular tumor. Our study will boost further research on the mechanism of NICH and drug filtering in the future.

A 30-year-old nulligravid woman with a history of polycystic ovarian syndrome presented for evaluation of left adnexal mass. The patient was referred to the gynecologic oncology clinic after endorsing signs of abdominal pain for a month and the pelvis ultrasound demonstrated hypoechoic solid mass in the left ovary. Magnetic resonance imaging with T1- and T2-weighted images demonstrated progressive centripetal “filling-in” of the mass suggesting a unique variation of malignant ovarian mass, similar to what is seen in hepatic hemangioma. Upon resection of the ovarian mass, pathology reported that the mass was filled with numerous small blood vessels with single later of endothelial cells confirming the diagnosis of ovarian hemangioma, capillary-type—a rare finding.

Background Hemangiomas are vascular lesions frequently seen in the head & neck region. Lip is an important site for such lesions. Most of the hemangiomas are seen at birth or the neonatal age group. It is uncommon to see such a lesion in adults. They mostly present as soft, smooth lobulated masses. Patients with hemangioma of lip seek treatment for aesthetic alteration and functional limitations caused by the lesion. The various treatment options include surgical excision of the tumor, embolization, electrosurgery, cryosurgery, laser excision, steroid injection, or injecting sclerosing materials. Here we report two cases of lip hemangiomas in adults. Methods In the first case, a 34 years old man presented to ENT OPD with a painless progressively enlarging nodular growth in the vermilion border of lower lip appreciated for 2 months which was surgically excised using electrocautery. In the second case, a 58 years old man presented to ENT OPD with a painless progressively enlarging lobulated growth in the lower lip appreciated for 4 months which was surgically excised using electrocautery. Results Histopathological examination of resected specimen in both the cases confirmed the diagnosis as capillary hemangioma. Conclusion Hemangiomas should be kept as differential diagnosis of benign lesions of lip.

BACKGROUND: Acral arteriovenous hemangioma is an acquired vascular tumor, asymptomatic in most cases and benign. It has characteristics of both hemangioma and vascular malformation. It is distinguished by thick-walled blood vessels intermingled with thin-walled and dilated vessels. In the past it was known as cirsoid aneurism. Superficial acral arteriovenous hemangioma predominate in women in the fourth or fifth decades of life. In the oral cavity it is most frequently seen on the labial mucosa. CLINICAL CASE: A 51-year-old female patient presented with a 14-month history of a 3 mm circular, crusted and well circumscribed lower vermilion border ulcer with incoercible spontaneous bleeding that healed, but recurred frequently. Histological study of the ulcer showed numerous blood vessels with different wall thicknesses and sizes with an ascending spiral arrangement. The patient reported chronic alcohol intake with a non-specified hepatic disease.CONCLUSIONS: A typical case of acral arteriovenous hemangioma is described by both topography and epidemiology. Association with hepatic diseases is reviewed. Surgical excision is recommended for these lesions and clinical suspicion is important if a recurrent or asymptomatic bleeding ulcer of long evolution is observed. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumors are rare vascular tumours, earlier referred to as hemangiopericytoma. Their occurrence in parapharyngeal space is very rare. Hence, they should also be considered in the differential diagnosis of parapharyngeal soft tissue tumours. This case is being reported to bring out an extremely rare vascular tumour at rare site and represents a surgical challenge because of difficult access in parapharyngeal space with difficult planes between tumour and rest of parapharyngeal space, approach to it is also difficult. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that causes progressive angiogenesis and lymphangiogenesis, which often occurs in the skin or soft tissue, with an acute onset and rapid progression. A 4-year-old girl was admitted to our hospital with a 2-year history of thrombocytopenia, combined with right hepatic atrophy and pancreatic lesion for 3 months. At the age of two, she developed purpura and thrombocytopenia was detected, after treatment with gamma globulin and corticosteroids, the platelet count normalized, but it dropped immediately at lower doses. One year after the cessation of corticosteroids therapy, the patient presented with abdominal pain and abnormal liver function and the magnetic resonance imaging (MRI) revealed right hepatic atrophy and pancreatic occupancy, but the first liver biopsy did not reveal any positive pathological results. By analyzing the clinical manifestations in conjunction with MRI and abnormal coagulation, we considered that the patient might be diagnosed as KHE with Kasabach-Merritt phenomenon, however, sirolimus treatment was ineffective and pancreatic biopsy only showed a tendency for tumors of vascular origin. Finally, we performed a Whipple operation after the right hepatic artery embolization, histological and immunohistochemical examination suggested KHE. Three months postoperatively, the patient’s liver function, pancreatic enzymes and blood clotting function gradually returned to normal. KHEs may result in significant blood loss with worsening of the coagulopathy and functional impairment, timely surgical intervention for KHE is necessary when non-invasive or minimally invasive treatment is ineffective, or the symptoms of tumor compression are obvious. [ABSTRACT FROM AUTHOR]

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Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign vascular tumor. Although its etiology is not clearly known, infection, hormonal factors, and trauma are the suspected reasons. There are few cases considered to be related to pregnancy described in the literature. Granulomas are rarely seen in the histopathology and only four patients with granulomas have been described. Herein, we will present a 28-year-old woman who has been pregnant three times; in every pregnancy, she developed pink nodular lesions on the back of her ear. In the first two pregnancies, the lesions had appeared during the pregnancy and regressed completely in the postpartum period. In her third pregnancy, the condition emerged again and lasted 1.5 years after birth of her child. Histopathology exhibited ALHE with granulomas. Although the relationship between ALHE and pregnancy is already known, there are few cutaneous ALHE cases associated with pregnancy reported in the literature. This association is further supported by this case having recurrent ALHE during each pregnancy period. Also, the presence of naked granuloma in histopathology, unlike most of the cases, contributes to the current histopathological data.

Abstract Positron emission tomography (PET) combined with a magnetic resonance (MR) scanner (PET/MR) with 18F-fluorodeoxyglucose (FDG) tracer is being used in quite a few nuclear medicine centers. The aim of this study is to illustrate two uncommon cases of primary inferior vena cava leiomyosarcoma which were formerly evaluated with anatomical images such as computed tomography and ultrasound. These techniques were inferior in the definition of the tumor and its characteristics. F-18 FDG PET/MR was essential and provided all the necessary information: its origin, local extension, anatomo-metabolic behavior, form of presentation, and distant metastasis in one single diagnostic technique. PET/MR accurately contributed to the diagnosis in a shortened period of time and, therefore, in the prognosis of this disease with greater benefits.

Hemangioma of the facial sinuses is a rare pathology, and given the lack of clinical specificity, the differential diagnosis with a malignant lesion often arises. We report the case of a 32-year-old patient who consulted for recurrent epistaxis of moderate severity. The preoperative diagnosis of a hemangioma of the left maxillary sinus was based on computed tomography and magnetic resonance imaging data, confirmed by the anatomopathological study of the surgical specimen, preceded by an embolization that facilitated the endoscopic surgical excision.

Background: Non-involuting congenital hemangiomas (NICHs) are fully formed vascular tumors at birth with distinctive clinical, radiologic, and histopathological profiles. In the literature, there is no effective therapy strategy for patients with NICH except surgery. Currently, no cell line or animal model exists for studying the mechanism of NICH and drug validation. We plan to construct a new strategy by constructing NICH organoids for further study. Result: Here, we report a novel NICH organoid system construction and optimization process. Both HE and immunohistological staining exactly matched NICH tissue. We further performed transcriptome analysis to elucidate the characteristics of NICH organoids. Both NICH tissue and NICH organoids manifested similar trends in download sites. NICH organoids display novel features to new cells derived from organoids and show spectacular multiplication capacity. In the preliminary verification, we found that cells splitting from NICH organoids were human endothelial cells. Drug validation demonstrated that trametinib, sirolimus, and propranolol showed no inhibitory effects on NICH organoids. Conclusion: Our data show that this new NICH-derived organoid faithfully captured the features of this rare vascular tumor. Our study will boost further research on the mechanism of NICH and drug filtering in the future. [ABSTRACT FROM AUTHOR]

Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case–control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data. Conclusions: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality. What is Known: • Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening. What is New: • Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients. [ABSTRACT FROM AUTHOR]