Effectiveness of Pulsed Dye Laser Treatment in Tufted Angioma: A Promising Intervention.

Introduction: 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.

Cerebral Tufted Angioma with Gradually Developing Peritumoral Edema: A Case Report.

Tufted angioma is a benign vascular tumor in which immature endothelial and pericyte cells and lymphatic vascular endothelium grow. It manifests as a flat, painful erythema that gradually expands mainly on the trunk and extremities. Although tufted angiomas can also occur in other areas of the body and may be more locally invasive, they rarely occur intracranially. A 63-year-old man underwent magnetic resonance imaging (MRI) for a brain check-up 8 years before his visit to our institute, which detected a mass lesion with surrounding cerebral edema in the left frontal lobe. The patient was followed up with annual MRI analysis, which indicated slow tumor growth and gradual development of peritumoral edema. The tumor was treated by gross-total resection. Histological analysis showed a slightly dilated microvascular core surrounded by many capillary aggregates in the brain parenchyma. Immunohistochemical findings indicated that the vascular endothelial cells were positive for CD34 and Brahma-related gene-1 and were surrounded by smooth muscle actin-positive pericytes. These findings were consistent with tufted angioma. Intracranial tufted angioma is uncommon, but it should be considered in the differential diagnosis for intracranial tumorous lesions. Long-term follow-up is necessary to unravel the natural history of the disease.

Comprehensive Therapy for Infant Vascular Tumor Associated With Kasabach-Merritt Phenomenon-Single-Center Primary Experience.

Objective: To introduce our single-center experience of infant vascular tumor associated with Kasabach-Merritt phenomenon (KMP) which received combined medicine treatment with intralesional laser photocoagulation (ILP) and sclerotherapy. Methods: A retrospective study was conducted using medical records of all children with a diagnosis of kaposiform hemangioendothelioma (KHE) or tufted angioma (TA) associated with KMP treated with medicine, intralesional laser photocoagulation (ILP), and sclerotherapy between February 2017 and November 2020. Clinical features, response to comprehensive therapy, and outcomes were recorded. Results: A total of 23 patients including nine females (39%) and 14 males (61%) were identified. The mean age was 6.9 months (age range, 11 days-2 years) at the time of treatment. Nine children (39%) demonstrated sensitivity to single corticosteroid therapy; 14 children (61%) received combined therapy with intravenous Vincristine (VCR) and corticosteroid therapy. All children had at least two ILP and sclerotherapy performed, with a mean of 3.5 procedures (range: 2-6). Of these 14 children, only one experienced a relapse of thrombocytopenia and the remaining 13 children had no clinical symptoms recurred. Conclusion: The combined therapy modalities could induce a more rapid tumor response and resolution of KMP and decrease the rebound rates. This research presents a novel and safe multi-modality treatment for infant vascular tumors associated with KMP.

Vascular neoplasia masquerading as cellulitis and persistent hemorrhagic pericardial effusion.

Tufted angioma and kaposiform hemangioendothelioma are considered to represent two ends of the spectrum of benign vascular neoplasms that predominantly present during infancy or early childhood. We report a rare case of a 5-month-old infant with complicated vascular neoplasm involving the pericardial cavity and skin over cervical region, masquerading as infective pericarditis with cellulitis. The patient responded dramatically to therapy with oral prednisolone and sirolimus, with a significant reduction of size of skin lesions and complete resolution of pericardial effusion over 8 weeks. The report also highlights the importance of a multidisciplinary team in managing such complicated cases.

Imiquimod 5% Cream for an Annular Plaque with Progressive Pain on the Right Distal Arm in the Cubital Fossa: Acquired Tufted Angioma.

Tufted angioma, also known as angioblastoma, is a rare benign vascular neoplasm of the skin and subcutaneous tissue. The natural history of tufted angioma is slow, progressive growth for several years, and tends to remain stable thereafter, resulting in disfiguring appearance. The main choice of treatment for tufted angioma is complete surgical excision, but local recurrence is frequent. Here, we report a 33-year-old woman with a four-year history of a steadily increasing pink to violaceous plaque on her right arm accompanied by spontaneous, progressive pain over the last seven months. Histology revealed multiple separated lobules of spindle cells throughout the dermis and subcutaneous tissue to the deep margin, with numerous mitoses but no cellular atypia, the small capillary-sized vessels scattered within the lobules, which were consistent with tufted angioma. To our satisfaction, her pain disappeared and the whole plaque was significantly improved after a four-month course of external application with imiquimod 5% cream.

Vascular Anomalies of the Head and Neck: A Pediatric Overview.

Vascular anomalies, further classified into vascular tumors and malformations, often involve the head and neck region of children. These entities may raise diagnostic dilemmas, as they often demonstrate heterogenous and overlapping histologic features. The aim of this paper is to provide an overview of the common vascular anomalies in the head and neck region of children. Specific entities discussed include infantile hemangioma, congenital hemangioma, tufted angioma, kaposiform hemangioendothelioma, and various vascular malformations. Clinicopathologic features and associated molecular associations are reviewed.

Comparison of efficacy and safety of corticosteroid and vincristine in treating kaposiform hemangioendothelioma and tufted angioma: A multicenter prospective randomized controlled clinical trial.

Kaposiform haemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors that can cause life-threatening Kasabach-Merritt phenomenon. No evidence-based treatment strategies have yet been established, and its management is still a challenge. The purpose of this multicenter prospective randomized controlled study was to evaluate and compare the efficacy of corticosteroid and vincristine (VCR) in the treatment of KHE and TA. All patients with KHE/TA who met the diagnostic criteria were consecutively recruited. The patients were randomized into a methylprednisolone (MP) group and a VCR group. The primary outcome was the single main parameter effective rate and overall effective rate of corticosteroid and VCR over 1 month after treatment. The single main parameters included platelets, fibrinogen, tumor size, texture, and appearance. From May 2016 to April 2018, a total of 59 patients completed the clinical trial, including 29 in the MP group and 30 in the VCR group. The results showed that VCR was superior to corticosteroid in the improvement of platelet (80.0% vs 44.0%, P = 0.019) and tumor texture (68.9% vs 30.8%, P = 0.007). Although the efficacy of VCR on fibrinogen (23.3% vs 20.7%, P = 1.000), tumor size (23.3% vs 13.8%, P = 0.273), and appearance (65.5% vs 46.2%, P = 0.120) was higher than that of corticosteroid, there was no significant difference (P > 0.05). Meanwhile, the overall effective rate of VCR was higher than that of corticosteroid (56.7% vs 31.0%), but the difference was also not statistically significant (P = 0.067). In conclusion, the therapeutic effect of VCR was significantly better than that of corticosteroid with regard to treating thrombocytopenia and tumor texture. We recommend that VCR could be an option for first-line treatment in KHE/TA patients.

A retrospective study: Clinicopathological and immunohistochemical analysis of 54 cases of tufted angioma.

BACKGROUND: Tufted angioma is a rare benign lesion with vascular proliferation. AIM: To retrospectively analyze the clinicopathological manifestations and immunohistochemical features of tufted angioma. METHODS: Clinical and histopathological features of tufted angioma (n = 54) were evaluated and analyzed retrospectively in the Department of Dermatology, Xijing Hospital from 2003 to 2014. RESULTS: Clinically, tufted angioma usually presented as erythematous plaques and papules on the head and neck (n = 11), trunk (n = 21) and extremities (n = 22), mainly in children (n = 48), without gender difference (24 males and 30 females). A total of 45 cases showed solitary lesions and nine cases showed multiple lesions. Common symptoms included pain (n = 11), tenderness (n = 7), itching (n = 1), hypertrichosis (n = 7), hyperhidrosis (n = 6) and Kasabach-Merritt phenomenon (n = 1). Histopathologically, typical tufted angioma (n = 37) showed proliferation of endothelial cells in a so-called cannonball pattern, while in the early (n = 4) and regressed (n = 13) stages the tufted appearance was not prominent. The proliferated endothelial cells were diffusely positive for CD31 and Wilms tumor 1, focally positive for D2-40 and Prox1, and negative for Glut-1. LIMITATIONS: Our research was confined to patients of Chinese origin and our sample size was limited. CONCLUSIONS: Tufted angioma is a rare vascular neoplasm with diverse clinical manifestations and unique pathological features. It should be recognized as a vascular tumor with lymphatic differentiation. We emphasize the importance of considering tufted angioma in the differential diagnoses of any congenital or acquired vascular tumor.

Propranolol Followed by a Foam Sclerotherapy for Treatment of Tufted Angioma over Neck: A Novel Therapeutic Approach.

Tufted angioma is a rare vascular tumor with cutaneous angiomatous proliferation, commonly localized in the skin and subcutaneous tissues. Most cases are usually acquired in childhood, having protracted course with minimal tendency for spontaneous regression. Various treatment modalities have been described in the literature in the management of tufted angioma with variable response. Thus, there is an urge for simple yet effective and less invasive procedure for tufted angioma, particularly involving aesthetically important areas such as face, head, and neck. We report a case of a large tufted angioma over the neck of a young girl, successfully treated with a combined approach of oral propranolol and foam sclerotherapy.

Kaposiform hemangioendothelioma and tufted angioma - (epi)genetic analysis including genome-wide methylation profiling.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular condition of childhood and is clinicopathologically related to tufted angioma (TA), a benign skin lesion. Due to their rarity molecular data are scarce. We investigated 7 KHE and 3 TA by comprehensive mutational analysis and genome-wide methylation profiling and compared the clustering, also with vascular malformations. Lesions were from 7 females and 3 males. The age range was 2 months to 9 years with a median of 10 months. KHEs arose in the soft tissue of the thigh (n = 2), retroperitoneum (n = 1), thoracal/abdominal (n = 1), supraclavicular (n = 1) and neck (n = 1). One patient presented with multiple lesions without further information. Two patients developed a Kasabach-Merritt phenomenon. TAs originated in the skin of the shoulder (n = 2) and nose/forehead (n = 1). Of the 5 KHEs and 2 TAs investigated by DNA sequencing, one TA showed a hot spot mutation in NRAS, and one KHE a mutation in RAD50. Unsupervised hierarchical clustering analysis indicated a common methylation pattern of KHEs and TAs, which separated from the homogeneous methylation pattern of vascular malformations. In conclusion, methylation profiling provides further evidence for KHEs and TAs potentially forming a spectrum of one entity. Using next generation sequencing, heterogeneous mutations were found in a subset of cases (2/7) without the presence of GNA14 mutations, previously reported in KHE and TA.

Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma.

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) primarily occurring in infants are difficult to distinguish. This study evaluated ultrasonography (US) and magnetic resonance imaging (MRI) features of KHE and TA. Pathologically proven TA (n = 21) and KHE (n = 40 [11 KHE + Kasabach-Merritt phenomenon [KMP]]) occurring between January 2015 and December 2017 were reviewed. US (n = 61) and MRI (n = 50) findings were retrospectively evaluated. On US, KHE and TA lesions were subcutaneous, while 40% of KHE exhibited an infiltrative pattern extending into adjacent muscles. Of TA lesions, 42.9% were hyperechoic and 96.15% of KHE lesions exhibited mixed echogenicity. Of TA lesions, 76.2% exhibited well-defined margins and all KHE lesions exhibited ill-defined margins. The depth and vascular density of KHE and KHE + KMP were significantly increased compared with TA. The arterial peak systolic blood flow velocity of KHE + KMP was significantly higher than that in TA and KHE. KHE and KHE + KMP were significantly harder than TA on elastography. 3-D color Doppler revealed branch-shape blood flow for KHE and KHE + KMP lesions; for TA, it revealed a dot-like and striped pattern. Considering MRI findings, KHE and KHE + KMP were more likely to exhibit diffuse heterogeneous enhancement after contrast than TA. KHE was infiltrative and more likely to be thick, hypoechoic, ill-defined, richly vascular and hard than TA on US. KHE lesions were subcutaneous and reticular, with heterogeneous enhancement on MRI. Awareness of these features should prompt radiologists in the differential diagnosis of pediatric masses.

Tacrolimus ointment for the treatment of superficial kaposiform hemangioendothelioma and tufted angioma.

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare infiltrative vascular tumors. Currently, no standard treatment regimens exist for KHE/TA. The purpose of our study was to evaluate the efficacy and safety of topical application of tacrolimus for superficial KHE/TA. We examined six patients with superficial KHE/TA. All patients were treated with tacrolimus 0.1% ointment twice daily for at least 12 months. The response rate was 100%, including three nearly complete remissions. Only one patient experienced local pruritus during treatment. The data constituted an intriguing rationale for clinical trials of topical tacrolimus in the treatment of superficial KHE/TA.

Indurated Dusky Red Swelling on the Forearm of an Infant: Tufted Angioma.

Tufted angioma (TA) is a rare benign vascular tumor that may be congenital or acquired and generally presents as an asymptomatic or painful solitary erythematous to violaceous poorly defined plaque. Hyperhidrosis and hypertrichosis may be associated. The lesions have tendency to resolve spontaneously, in majority, within 2 years of disease onset. However, occurrence of Kassbach-Merritt phenomenon should be looked for. We present a case of 8-months-old infant with TA over the right forearm with classical clinical, histoptahological and immunohistochemistry features.

Surgical excision of a tufted angioma of the hand in an adult-a rare case report with a review of literature.

Tufted Angiomas, also known as angioblastomas/Angioblastoma of Nagakawa, are rare vascular neoplasms of both sexes localised to the skin and subcutaneous tissues with the upper trunk and neck being the most common sites. They are more common in children but a few cases in juveniles and adults have been reported. Typically, Tufted Angioma remains stable or can show rarely a spontaneous regression. This article wants to evaluate the efficacy of current treatment modalities for tufted angioma (TA). A review was performed using PubMed database (Medline) for clinical studies. We report our case, a 29-year-old female who presented with a second finger of the left hand, painful, slowly progressive, firm swelling diagnosed as Tufted Angioma on histopathology and immunohistochemistry after complete surgical excision.

Tufted-angioma-like lesion associated with vascular endothelial growth factor and interleukin-6 in TAFRO syndrome: Is it a common histological feature of multicentric Castleman disease/POEMS syndrome?

The histology of skin lesions of TAFRO (thrombocytopenia, anasarca, reticulin fibrosis/renal failure, and organomegaly) syndrome has rarely been reported. We report herein two cases of TAFRO syndrome with characteristic vascular skin lesions. The lesions resembled a tufted angioma, although those of case 1 partially resembled a glomeruloid hemangioma, which was known as a specific lesion in POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes), a variant of multicentric Castleman disease (MCD). The high titer of serum vascular endothelial growth factor and interleukin-6 could explain common characteristic vascular lesions in both TAFRO syndrome and POEMS syndrome/MCD.

Congenital tufted angioma: A multicenter retrospective study of 30 cases.

BACKGROUND: Recent reports indicate that tufted angioma is a rare vascular neoplasm that manifests more frequently at birth than previously thought. Few studies specifically address congenital presentation. OBJECTIVES: We analyzed the clinicopathological characteristics, clinical course, and treatment of congenital tufted angioma (cTA) and evaluated variables that were indicative of problematic lesions. METHODS: We performed an observational retrospective study of 30 patients with cTA in 9 Spanish hospitals over a 14-year period. Histopathology and immunohistochemistry studies were performed. RESULTS: Congenital tufted angioma mainly affected the limbs (56.67%), followed by the face and/or neck (23.33%). Almost three-quarters of facial cTA were located over the mandibular area. Immunohistochemically, proliferating cells expressed markers of endothelial cells, with some clusters of cells, especially at the periphery of the aggregates, showing positivity for podoplanin. As no associated complications were observed in 66.67% of cases, no treatment was started. LIMITATIONS: Data were collected retrospectively. CONCLUSIONS: Our findings emphasize the clinical features and course of cTA. The possibility of cTA should be considered when a poorly defined congenital infiltrative vascular tumor with(out) overlying hirsutism appears over the mandibular area. Location on the face and/or neck requires a more comprehensive workup, since potentially severe complications often appear early.

Topical rapamycin (sirolimus) for the treatment of uncomplicated tufted angiomas in two children and review of the literature.

The mTOR inhibitor rapamycin is used systemically for the treatment of vascular lesions. We report the first use of topical rapamycin for the successful treatment of two cases of tufted angioma. The evidence for the use of topical rapamycin in other dermatologic conditions is summarized to aid in clinical decision making on preparations and anticipated side effects.

Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma.

BACKGROUND: Sirolimus has been used to manage various complex vascular anomalies. Kaposiform hemangioendothelioma and tufted angioma may develop Kasabach-Merritt phenomenon in infancy. METHODS: We retrospectively reviewed the clinical and laboratory data of eight patients with kaposiform hemangioendothelioma and tufted angioma who were initially treated using oral sirolimus in our center, including six with Kasabach-Merritt phenomenon. RESULTS: Five girls and three boys seen between September 2012 and March 2015 were included. Age at initiation of sirolimus ranged from 30 days to 14 weeks (mean±SD 8.6 ± 3.5 weeks). Six of these eight patients had kaposiform hemangioendothelioma, and two had a tufted angioma. Platelet count before start of oral sirolimus ranged from 5 × 109 /L to 189 × 109 /L ((78.8 ± 65.2)×109 /L) and fibrinogen level from 68 to 215 mg/dL (123.1 ± 50.5 mg/dL). All patients received standard doses of sirolimus (0.05 mg/kg orally, twice daily) as initial therapy. All patients with thrombocytopenia or hypofibrinogenemia reached a normal platelet count and a normal fibrinogen level within 3 to 4 weeks after sirolimus treatment. Length of treatment ranged from 12 to 79 weeks (39.9 ± 15.3 weeks). Two patients developed grade 2 oral mucositis during treatment. CONCLUSION: Sirolimus as first-line therapy shows great promise in the treatment of kaposiform hemangioendothelioma and tufted angioma.