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Infantile cavernous hemangioma is a benign vascular tumor in childhood. Cavernous hemangiomas have a tendency of slow enlargement with subsequent complications such as ulceration and bleeding. We report a case of a large cavernous hemangioma affecting the median upper lip in an 11-year-old girl. The lesion was growing and painful on pressure. The patient felt stigmatized. Treatment was wished for by patient and parents. After careful examination including Doppler ultrasound, we suggested a combined approach. In the first step the lesion was coagulated with an intralesional neodymium-YAG laser using a blunt tip. In the second step, immediately after the action of the laser, the lesion was removed completely by surgery. Defect closure was realized with bilateral tissue-expanding vermillion myocutaneous flaps. The patient was followed-up to 5 months. Disfigurement and functional impairment were eliminated. The result was satisfying for both patient and parents. Vascular lesions of the upper lip-particularly when close to Cupid's bow-can become disfiguring and cause functional impairment. We propose a combined approach with intralesional neodymium-YAG laser photocoagulation to avoid bleeding and to shrink the lesion before complete surgical removal. For defect closure, bilateral tissue-expanding vermillion myocutaneous flaps (Goldstein technique modified by Sawada) were used.

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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.

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Background: Hemangiosarcoma is a malignant vascular tumor commonly described in veterinary ophthalmology as emerging at the margin of the third eyelid and bulbar conjunctiva. The primary corneal attachment of the tumor is considered rare, as the cornea is an avascular structure, but there are sparse reports of primary occurrences in the cornea of dogs, cats, and horses. No reports were found in wild animals. The Tapirus terrestris, commonly known as Tapir, is a perissodactyl mammal that inhabits South America regions, being considered the largest terrestrial mammal of Brazil and one of the most popular animals in zoos in various countries. This paper aims to report the first case of primary corneal hemangiosarcoma in a 25-year-old female Tapir (Tapirus terrestris) held in captivity. Case: A 25-year-old Tapirus terrestris female presented an irregular mass in the right cornea, mucopurulent secretion, and inconclusive previous cytological evaluation. It also had a history of a milky cornea for many years, and in the last 12 months, a tissue growth was observed, as well as the occurrence of mucopurulent secretion. The menace response in the right eye was negative. In the ophthalmologic examination, a red-colored, lobed, hemorrhagic, and ulcerated mass occupying approximately 90% of the cornea was observed, and in the peripheral cornea, there was an intense discoloration. Due to the extension of the mass and visual loss, the transpalpebral enucleation was performed under general inhalation anesthesia. The histopathological analysis evidenced a vascular tumor restricted to the central peripheral cornea, composed of blood vessels of small and large caliber, showing polygonal endothelial cells with moderate atypia, anisocytosis, and anisocariasis, in addition to dense stroma and inflammatory cells. The mass occupied the anterior corneal stroma, and there was no involvement of bulbar conjunctiva, sclera, or intraocular structures. The immunohistochemical study revealed the positivity of neoplastic cells for CD31, and the proliferative index of the lesion was evaluated by the Ki-67 as inferior to 10%. Discussion: The histopathologic and immunohistochemical examinations evidenced a hypercellular lesion, and the diagnosis of primary corneal hemangiosarcoma (HSA) was possible due to the significant cellular atypia identified in the lesion. To the authors' knowledge, this is the first case of corneal hemangiosarcoma described in Tapirus terrestris. The HAS is a malignant neoplasm originating in the vascular endothelial cells. There is a significant correlation between the HSA and prolonged exposure to solar radiation. The tapir of this report lived in a high-altitude region with exposure to ultraviolet rays; therefore, it might be an important predisposing factor for the HSA emergence. The chosen treatment for vascular tumors according to the visual prognostic is the lamellar keratectomy; however, the transpalpebral enucleation was the elected treatment since the eye was not visual, the neoplasm had a great extension, the animal was in advanced age and lived in captivity, and it was difficult to handle the animal without sedation to perform adjuvant therapies. Since the mass corresponded to 90% of the cornea, total removal with free edges would not be possible as described in the literature, and, therefore, there was an increased risk of relapse. In the follow-up after 3 years of surgical excision, the animal remained free of metastasis, reinforcing the diagnosis of primary corneal hemangiosarcoma.

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Intramuscular hemangioma (IMH) represents less than 1% of all hemangiomas. In the head and neck region, it occurs mostly in the masseter, temporalis and sternocleidomastoid muscles. Despite its infiltrative growth pattern and several worrisome histological features, such as increased mitotic activity, plumpness of the nuclei, intraluminal papillary projections or perineural infiltration, the lesion is benign, and complete surgical excision is the preferred treatment for such oral lesions. Herein, we report three rare cases of IMH in the tongue and lip, discuss the clinical and histological aspects, and review the literature regarding this lesion.

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Las anomalías vasculares han sido clasificadas por primera vez en 1982, por Mulliken y Glowacki y recientemente, la Sociedad Internacional para el Estudio de las Anomalías Vasculares (ISSVA) modificó dicha clasificación, por la que hoy es aceptada mundialmente, que divide a las lesiones vasculares en tumorales y malformaciones. Dentro de la misma, encontramos una variedad de tumores, desde los más frecuentes como los hemangiomas infantiles, hasta los de menor frecuencia, integrando los síndromes clínicos y genéticos. El hemangioma hemosiderótico targetoide (HHT), también conocido como hemangioma hobnail, se encuentra dentro de dicha clasificación en el grupo de "otros" tumores vasculares, por su baja frecuencia. Fue descrito por primera vez por Santa Cruz y Aronberg en 1988, como una proliferación vascular benigna localizada con más frecuencia, en el tronco y las extremidades de los adultos jóvenes o de mediana edad. El objetivo es la presentación de un caso de hemangioma hobnail, patología vascular que se encuentra en constante revisión en la actualidad, profundizar en el conocimiento de los tumores vasculares, su clasificación y marcadores inmunohistoquímicos, revisar la bibliografía nacional e internacional y determinar bajo qué clasificación incluiríamos a esta entidad. La finalidad también es realizar un exhaustivo análisis de los diagnósticos diferenciales de esta patología, teniendo en cuenta la práctica clínica y demostrar como las nuevas tecnologías de diagnóstico, nos ayudan a aproximarnos al origen de la misma.

Vascular anomalies have been classified by Mulliken and Glowacki in 1982 and recently modified this classification by the International Society for the Study of Vascular Anomalies (ISSVA) today accepted worldwide, dividing vascular tumors and malformations. Within the same we find a variety of tumors from the more frequent as infantile hemangiomas, to the others with lower frequency, integrating clinical and genetic syndromes. Targetoid haemosiderotic haemangioma (THH), also known as hobnail haemangioma, is within that classification in the group of "other" vascular tumors due to their low frequency. It was described by Santa Cruz and Aronberg in 1988. It`s a benign vascular proliferation that arises most commonly on the trunk and limbs of young or middle-aged adults. The aim is to present a case of hobnail haemangioma, which is constantly being revised now a days, deepen of the knowledge of vascular tumors, their classification and immunohistochemical markers, review national and international literature and determine under what classification we would include this entity. The purpose is also to conduct a thorough analysis of the differential diagnosis of this disease, taking into account the clinical practice and demonstrate how new diagnostic technologies help us to approach it´s origin.

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Los corioangiomas placentarios son tumores vasculares benignos y los tumores placentarios más comunes, con una prevalencia de 1%. Raras veces sobrepasan los 4 a 5 centímetros y, cuando esto ocurre, es descrito como corioangioma gigante. Los embarazos con corioangiomas gigantes están asociados con complicaciones maternas y fetales, tales como anemia hemolítica microangiopática severa, parto pretérmino, polihidramnios, restricción del crecimiento intrauterino del feto, trombocitopenia e hidropesía. Se presenta un caso de un corioangioma gigante diagnosticado a las 22 semanas. A pesar del seguimiento estricto, el parto se produjo a las 30 semanas debido a polihidramnios y rotura prematura de membranas. Tanto la madre como el recién nacido se recuperaron sin complicaciones.

Placental chorioangiomas are benign vascular tumors and are the most common placental tumors, with a prevalence of 1%. It rarely surpasses 4 - 5 centimeters in length and, when it happens, is referred to as giant chorioangioma. Pregnancies with giant chorioangiomas are associated with maternal and fetal complications, such as severe microangiopathic haemolytic anemia, preterm labor, polyhidramnios, intrauterine growth restriction, thrombocytopenia and hydrops. A case of giant chorioangioma diagnosed at 22 weeks is presented. Despite close follow-up, delivery occurred at 30 weeks of pregnancy due to polyhidramnios and premature rupture of membranes. Both mother and newborn recovered without complications.

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El angioma en penacho es un raro tumor vascular benigno de comportamiento localmente agresivo que aparece en la infancia temprana; puede complicarse con el fenómeno de Kasabach-Merritt. Presentamos el caso de un paciente masculino de 2 años de edad con la lesión clínica característica.

Tufted angioma is a rare benign vascular tumor with a locally aggressive behavior that appears in early childhood; it may be complicated by the Kasabach - Merritt phenomenon. We report a 2 year old boy with a characteristic clinical lesion.

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El Angioma en Penacho es un raro tumor vascular de la infancia, que comparte características con el hemangioendotelioma kaposiforme, teniendo ambos posibilidad de desarrollar Síndrome de Kasabach-Merrit. Presentamos una paciente portadora de un angioma en penacho desde el nacimiento, que hemos seguido por 13 años: clínicamente, con estudios de imágenes y biopsias. El caso presenta similitudes con la literatura, con algunas interesantes peculiaridades en su evolución. Consideramos que por el comportamiento clínico e histológico, el Angioma en Penacho es parte de la misma enfermedad que el Hemangioendotelioma Kaposiforme.

Tufted angioma is a rare vascular tumor of childhood that shares features with the Kaposiform hemangioendothelioma, having both tumors the possibility of developing Kasabach-Merrit phenomenon. We report the case of a patient with a tufted angioma since birth, which we have followed for 13 years: clinically, with imaging studies and biopsies. The case presents similarities with literature, with some interesting peculiarities in its evolution. We consider that because of the clinical and histological behavior, Tufted angioma is a part of the same disease that Kaposiform hemangioendothelioma.

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El Granuloma piógeno (GP), es una lesión vascular benigna común en piel y mucosas, que se incluye en la clasificación de anomalías vasculares de la “International Society for the study of vascular anomalies (ISSVA)”, dentro de “otros” tumores vasculares, a pesar de su controversial denominación y su patogenia “reaccional”. Presentamos un caso de granuloma piógeno en una paciente de 18 años de edad, en el segundo trimestre de embarazo con una presentación atípica por su gran tamaño y localización acral, con excelente respuesta al tratamiento quirúrgico.

Pyogenic Granuloma (GP) is a common vascular lesion of the skin and mucous membranes, usually presented as a solitary neoplasm with a bright red, smooth surface; usually sessile or pedunculated that grows rapidly to reach 1 to 2 inches in a few weeks and then remains stationary. It is included in the classification of vascular anomalies of the International Society for the study of vascular anomalies (ISSVA) to under "other" vascular tumors despite their controversial name and their "reactional" pathogenesis. Our goal is to present a case of pyogenic granuloma in an 18-year-old rural worker in the 2nd trimester with an atypical presentation for its size (4 x 4 cm) and acral location with excellent response to surgery and emphasize that such damage can result from repeated trauma, impaired wound healing and neovascularization and overexpression of the growth factor vascular endothelial (VEGF) at this period of life.