Guía mexicana para el diagnóstico y el tratamiento del hemangioma infantil.

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.

El hemangioma infantil es un tumor vascular benigno, el más frecuente de la infancia, cuya evolución natural favorece la desaparición de la lesión en la misma edad pediátrica y que cuenta con tratamientos eficaces y seguros que limitan su crecimiento y favorecen su desaparición a edades más tempranas. Continúa siendo motivo de atención de complicaciones, debido a diagnósticos erróneos, desconocimiento del padecimiento, referencia tardía o temor de los efectos de los fármacos utilizados para su tratamiento. Además, se normaliza su presencia sin tomar en cuenta que puede llegar a causar incertidumbre, ansiedad, sentimientos de culpa y, como consecuencia, importante afectación de la calidad de vida, principalmente en los padres o cuidadores del niño. La necesidad de una guía de práctica clínica en nuestro país surge ante la alta presentación de complicaciones del hemangioma infantil referidas de manera tardía aun con la disponibilidad de tratamientos adecuados, la evolución continua de la medicina y la aparición de nueva evidencia. A lo largo de la guía se encontrarán recomendaciones en relación con el diagnóstico, el tratamiento y el seguimiento de los pacientes con hemangioma infantil, tomando en cuenta los paraclínicos que pueden realizarse, las opciones de manejo tópico o sistémico, y las terapias adyuvantes. Por primera vez se incluyen en una guía para el manejo del hemangioma infantil herramientas objetivas para el seguimiento de los pacientes, así como para ayudar al médico de primer contacto en su referencia oportuna.

Hemangiopericitoma en el cuello / Hemangiopericytoma in the Neck

Introducción: El hemangiopericitoma es un raro tumor mesenquimal (vascularizado y potencialmente maligno) derivado de los pericitos, que puede aparecer en cualquier parte del cuerpo; sin embargo, en el cuello se describen casos aislados. La resección quirúrgica completa constituye la piedra angular del tratamiento. Objetivo: Presentar un caso de un hemangiopericitoma en el cuello, como un caso inusual, con potencial maligno desconocido, diagnóstico y tratamiento oportuno. Presentación de caso: Paciente de sexo masculino, de 39 años de edad, sin antecedentes de enfermedad conocidos, con una masa perceptible a nivel V del cuello derecho. Estudios de imagen muestran un tumor vascularizado de aproximadamente 6 x 7 x 6 cm, entre los músculos escalenos, que fue originado en la arteria cervical profunda. Se confirmó mediante biopsia incisional el hemangiopericitoma, el cual fue tratado mediante resección tumoral completa y radioterapia adyuvante. Actualmente el paciente no tiene actividad tumoral después de su tratamiento inicial. Conclusiones: El hemangiopericitoma en el cuello es raro, el diagnóstico constituye un reto clínico e histológico, ya que, al ser poco común, su potencial maligno resulta desconocido. Aquellos tumores que tienen bajo grado de malignidad pueden ser controlados, de acuerdo a su localización y tamaño, mediante resección completa; mientras que los tumores de alto grado pueden recurrir y dar origen a metástasis. Nuestro paciente tuvo características histopatológicas con invasión capsular, lo que trajo como consecuencia un incremento del riesgo de recurrencia local. Por ese motivo, se decidió aplicar tratamiento adyuvante con radioterapia. El paciente se mantiene sin recurrencia tumoral local y a distancia después de 9 años de vigilancia médica(AU)

Introduction: Hemangiopericytoma is a rare mesenchymal tumor (vascularized and potentially malignant) derived from pericytes. It can occur anywhere in the body; however, isolated cases are described in the neck. Complete surgical resection is the cornerstone of treatment. Objective: To present a case of hemangiopericytoma in the neck, as an unusual case, with unknown malignant potential, as well as its timely diagnosis and treatment. Case presentation: A 39-year-old male patient, with no known history of disease, had a noticeable mass at the V level of the right neck. Imaging studies showed a vascularized tumor of approximately 6 x 7 x 6 cm, between the scalene muscles, which originated in the deep cervical artery. Hemangiopericytoma was confirmed by incisional biopsy, as well as treated by complete tumor resection and with adjuvant radiotherapy. Currently, the patient has no tumor activity after his initial treatment. Conclusions: Hemangiopericytoma in the neck is rare. Its diagnosis is a clinical and histologic challenge because, being uncommon, its malignant potential is unknown. Those tumors with low-grade malignancy can be controlled, according to their location and size, by complete resection; while high-grade tumors may recur and give rise to metastases. Our patient had histopathologic features with capsular invasion, which resulted in an increased risk of local recurrence. For this reason, adjuvant treatment with radiotherapy was decided to be applied. The patient remains without local or distant tumor recurrence after 9 years of medical surveillance(AU)

Corioangioma en paciente multípara. Caso clínico / Chorioangioma in multiparous aged. Clinical case

Resumen ANTECEDENTES: El corioangioma es un tumor vascular de origen placentario perteneciente al grupo de los hemangiomas vasculares. Es el tumor placentario primario no trofoblástico más frecuente de origen vascular. CASO CLÍNICO: Paciente de 37 años con antecedentes de: menarquia a los 12 años, inicio de la vida sexual activa a los 13 años, tres embarazos y dos partos. Del embarazo actual solo había tenido dos consultas prenatales. El ultrasonido reportó 35 semanas de embarazo con feto de 2250 g. La paciente tuvo dolor de origen obstétrico y trabajo de parto en fase latente. El parto fue eutócico, sin complicaciones. Se observó una tumoración placentaria. CONCLUSIONES: La valoración placentaria mediante ultrasonido-Doppler de control es importante para detectar tumoraciones y evitar que, cuando son pequeñas, pasen inadvertidas, como los corioangiomas.

Abstract BACKGROUND: Chorioangioma is a vascular tumor of placental origin belonging to the group of vascular hemangiomas. It is the most frequent non-trophoblastic primary placental tumor of vascular origin. CLINICAL CASE: 37-year-old patient with a history of: menarche at 12 years of age, beginning of active sexual life at 13 years of age, three pregnancies and two deliveries. She had only had two prenatal visits for the current pregnancy. The ultrasound reported 35 weeks of pregnancy with a 2250 g fetus. The patient had pain of obstetric origin and labor in the latent phase. The delivery was euthecological, without complications. A placental tumor was noted. CONCLUSIONS: Placental assessment by control ultrasound-Doppler is important to detect tumors and to avoid that, when they are small, they go unnoticed, such as chorangiomas.

PET/MR: primary inferior vena cava leiomyosarcoma.

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 de coroide e o desafio do diagnóstico diferencial / Choroidal hemangioma and the challenge of differential diagnosis

Resumo O hemangioma de coroide é um tumor benigno relativamente raro, que se apresenta de forma circunscrita ou difusa, sendo esta última normalmente associada à Síndrome de Sturge-Weber. Os tumores circunscritos manifestam-se de forma insidiosa, com o diagnóstico realizado comumente após o aparecimento de sintomas secundários. Apresentam como diagnóstico diferencial lesões graves e potencialmente letais, como melanoma de coroide e doença metastática. Neste relato descrevemos o caso de um hemangioma intraocular nodular avançado associado a descolamento hemorrágico da retina, evidenciando o desafio do diagnóstico diferencial devido às semelhanças clínicas e radiológicas compartilhadas pelos tumores.

Abstract Choroidal hemangioma is a fairly rare benign vascular tumor that can manifest in either circumscribed or diffuse type; the latter one is usually related to Sturge-Weber Syndrome. The circumscribed tumors have an insidious presentation and diagnosis is commonly made after the onset of secondary symptoms. Serious and potentially lethal lesions, such as choroidal melanoma and metastatic disease, may represent a differential diagnosis. In this report, we describe an advanced case of nodular hemangioma associated with hemorrhagic retinal detachment. This case highlights the challenge of differential diagnosis in intraocular tumors, due to their similar clinical and radiologic features.

Colorimetric analysis of images in the follow-up of infantile hemangiomas.

BACKGROUND/OBJECTIVES: Infantile hemangiomas (IH) are common vascular tumors that appear early in life, have a rapid proliferative phase and slowly involute. There are no standardized ways to evaluate the regression of these lesions. We propose a colorimetric analysis of photographs to allow a more precise determination of IH treatment response and involution. METHODS: Patients 1-10 months of age with superficial or mixed IH were included. The lesions were managed with 0.5% topical timolol ophthalmic solution. Patients were followed for 16 weeks with 6 evaluations each. Photographic images were taken with a red and green circle placed beside each hemangioma. The photographs were treated as to equalize the size, color, and brightness among them based on the colors of the two circles. A grading scale was established based on the color of the patient skin (0) and the color of the hemangioma at the beginning of treatment (100) by pixel analysis using Adobe Photoshop® software. RESULTS: A total of 17 patients 1 to 10 months of age were included, of whom 16 were girls (94%). Fourteen lesions were superficial, and 3 were mixed IH. The median time prior to initiation of treatment was 105 days (44-232). All lesions showed some degree of clearing. The mean of lightening of color intensity observed was of 45% (17%-74%) over the period of follow-up. CONCLUSIONS: The colorimetric analysis of the digital images allowed an accurate and objective evaluation of IH clearing.

Epithelioid hemangioendothelioma of the parotid gland: A case report in an unusual location with a review of the literature.

Epithelioid hemangioendothelioma (EHE) is a malignant vascular tumor that usually affects the liver, lung, bone and deep soft tissues of the extremities or trunk. To our knowledge, only 3 cases in the parotid gland have been reported to date. We report a case of a 62-year-old woman who presented with a 1-year history of a slow-growing, painless mass over the left mandibular angle. Imaging studies showed a 2cm mass over the left parotid gland with peripheral calcifications. The patient underwent a superficial parotidectomy. Sections displayed neoplastic epithelioid cells with cytoplasmic vacuoles containing erythrocytes, surrounded by a myxohyaline stroma. Immunohistochemistry was positive for CD31, CD34, ERG, and factor VIII, but negative for cytokeratin AE1/AE3, CK7, EMA, SMA, and S100. The findings were those of an EHE involving the parotid gland. This case demonstrates an EHE in a rare location and emphasizes the need to consider this tumor when diagnosing uncommon soft tissue tumors of salivary glands.

Hiperplasia angiolinfoide con eosinofilia / Angiolymphoid Hyperplasia with eosinophilia

La hiperplasia angiolinfoide con eosinofilia es una enfermedad vascular o tumor vascular, infrecuente, tumoral o reactiva, con carácter inflamatorio, benigna y de etiología desconocida. Los hallazgos histológicos consisten en la proliferación vascular de células endoteliales prominentes y un infiltrado inflamatorio intersticial crónico de leucocitos, histiocitos y eosinófilos. Afecta frecuentemente a mujeres de la tercera y cuarta década de la vida. Se han descrito múltiples variantes de tratamiento pero es frecuente su recurrencia. Clínicamente se presenta como pápulas o nódulos subcutáneos, limitados, únicos o múltiples, color rojo, marrón o violáceo, que se localizan con frecuencia a nivel de la cabeza, cuello; en ocasiones se asocia a prurito y dolor. El interés en presentar este caso radica en su rara localización, su presentación en el sexo masculino; el tamaño, la extensión y la infrecuencia de la lesión. Se presenta el caso de un paciente masculino de 42 años de edad con múltiples lesiones en el eje longitudinal del miembro superior izquierdo, la mayor de 15 x 15 cm, localizada en la cara póstero-interna del brazo en sus dos tercios dístales, limitada, móvil, no dolorosa, con desplazamiento del tejido muscular, y deformidad estructural, con diagnóstico, después de unabiopsia posquirúrgica,de hiperplasia angiolinfoide con eosinofilia según histología La evolución del paciente fue satisfactoria y se mantiene seguimiento médico para comprobarposibles recidivas(AU)

The angiolymphoid hyperplasia with eosinophilia is a vascular disease (or vascular tumor) uncommon, tumoral or reactive, with inflammatory characteristics, benign of unknown etiologic. The histological finds consist on the vascular proliferation of endotelial prominent cells and a chronic inflammatory interstitial infiltrator of leukocytes, histiocytes and eosinophils. It frequently affect to elder women in the third and fourth ages. Clinically, it is presented as papules or subcutaneous nodules, limited, only one or multiple nodules; in red, brown or purplish color that are frequently located at the level of the head, neck; in occasions, it is associated with pruritus and pain. The interest in presenting this case is due to its rare localization, its appearance in the male sex, the size, extension and infrequency of the lesion. The case consists in a 42 year-old masculine patient with multiple lesions in the left superior member's longitudinal axis. The biggest lesion is of 15 x 15 cm, located in the postero-internal part of the arm in its distals two thirds, limited, movable, not painful, with displacement of the muscular tissue, and structural deformity, with a diagnostic of angiolymphoid hyperplasia with eosinophilia according to histology (post-surgical biopsy).The evolution of the patient was satisfactory, and remains medically followed-up in case of possible relapses(AU)

Sarcoma de Kaposi clásico variedad linfangiomatosa tratada con criocirugía: a propósito de un caso / Classic Kaposi sarcoma lynphangiomatous variety treated with cryosurgery: a case report

El sarcoma de Kaposi es un tumor vascular que afecta la piel y otros órganos. Actualmente existen diversas opciones terapéuticas para tratar esta condición, sin embargo, la terapia óptima aún no está bien establecida. La crioterapia es una alternativa que ofrece buenos resultados y escasas complicaciones. Se trata de un paciente masculino de 90 años de edad, acude con un cuadro clínico de aproximadamente 12 meses de evolución, con presencia de "manchas café" en ambos pies, que se exacerban con calor y disminuyen con frío, asintomáticas y sin tratamiento previo.

Kaposi's sarcoma is a vascular tumor that affects the skin and other organs. Currently there are several therapeutic options to treat this condition, however, optimal therapy is not yet well established. Cryotherapy is an alternative that offers good results and few complications. It's a case of a 90-year-old male patient, with a clinical picture of approximately 12 months of evolution, with the presence of "brown spots" on both feet, which are exacerbated with heat and decrease with cold, asymptomatic and without previous treatment.

Nasopharyngeal Angiofibroma: A Clinical, Histopathological and Immunohistochemical Study of 42 Cases with Emphasis on Stromal Features.

Nasopharyngeal angiofibroma is a benign but aggressive tumor of unknown etiology, typically occurring in adolescent males. It is described as a rare neoplasm; however, the prevalence seems to have geographic differences. All cases referred to our head and neck clinical and pathology service were reviewed. Most of the patients presented at an advanced stage. The clinical and radiographic features are presented and discussed. Histologically, the tumor shows a highly vascular fibrous proliferation with characteristic plump, angulated and stellate cells, categorized as fibroblasts. Immunohistochemistry was performed on 42 cases to further elucidate the nature of these cells. The stromal cells expressed vimentin and factor XIIIa, the latter expressed most commonly in the giant stellate cells. Inflammation was almost exclusively present in peripheral subepithelial areas. Mast cells were abundant, even in the absence of other inflammatory cells. Lymphatics were observed principally in peripheral regions. Proliferating cells (Ki-67 reactive) were restricted to endothelial cells.

Angioma en penacho: A propósito de un caso clínico / Tufted angioma: A case report

Se presenta el caso de una paciente de un año de edad, con una lesión tumoral congénita en dorso, eritemato-violácea. Se evidencia al cabo de un año durante el control evolutivo, aumento del volumen y cambio de coloración, por lo que se decide su exéresis completa. El examen histopatológico de la pieza concluyó con el diagnóstico de angioma en penacho (AP). El AP es un tumor vascular benigno, poco frecuente. Aparece sin predilección racial y es igual en ambos sexos. Puede ser congénito o adquirido en la primera infancia, ocasionalmente se presenta en la edad adulta. Su patogenia está poco dilucidada.

Presented the case of a patient of one year old with a congenital, erythematous-violaceous tumoral lesion on back. During the control evolutionary is evidence after a year increase of the volume and change of coloration by what is decides his removal complete. The histopathological examination of the piece ended with a diagnosis of tufted angioma (TA). The TA is a rare, benign vascular tumor. Appears no predilection racial and is equal in both sexes. It can be congenital or acquired in early childhood, it occurs occasionally in adulthood. Its pathogenesis is shortly to become.

Hemangiossarcoma ocular em um equino: relato de caso / Equine ocular hemangiosarcoma: case report

O hemangiossarcoma ocular na espécie equina é um tumor maligno, raro e agressivo, de origem vascular endotelial. No presente trabalho, descreve-se um caso de hemangiossarcoma ocular em uma égua de 10 anos que apresentava secreção serossanguinolenta advinda de uma massa, acometendo a conjuntiva bulbar e a terceira pálpebra do olho direito. O diagnóstico foi realizado com base na avaliação histopatológica e na imuno-histoquímica. Foi realizada a enucleação, assim como a completa excisão cirúrgica do tecido acometido, não sendo observada, após seis meses da terapia, a recidiva ou a metástase da lesão.(AU)

Equine ocular hemangiosarcoma is a rare and aggressive malignant tumor of vascular endothelial origin. We describe a case of ocular hemangiosarcoma in a 10-year-old mare with serosanguineous secretion arising from a mass involving the bulbar conjunctiva and third eyelid of the right eye. The diagnosis was based on histopathological evaluation and immunohistochemistry. Enucleation was performed as complete surgical excision of the affected tissue, with no recurrence or metastasis of the lesion being observed after six months of.(AU)

Hemangiossarcoma ocular em um equino: relato de caso / Equine ocular hemangiosarcoma: case report

O hemangiossarcoma ocular na espécie equina é um tumor maligno, raro e agressivo, de origem vascular endotelial. No presente trabalho, descreve-se um caso de hemangiossarcoma ocular em uma égua de 10 anos que apresentava secreção serossanguinolenta advinda de uma massa, acometendo a conjuntiva bulbar e a terceira pálpebra do olho direito. O diagnóstico foi realizado com base na avaliação histopatológica e na imuno-histoquímica. Foi realizada a enucleação, assim como a completa excisão cirúrgica do tecido acometido, não sendo observada, após seis meses da terapia, a recidiva ou a metástase da lesão.

Equine ocular hemangiosarcoma is a rare and aggressive malignant tumor of vascular endothelial origin. We describe a case of ocular hemangiosarcoma in a 10-year-old mare with serosanguineous secretion arising from a mass involving the bulbar conjunctiva and third eyelid of the right eye. The diagnosis was based on histopathological evaluation and immunohistochemistry. Enucleation was performed as complete surgical excision of the affected tissue, with no recurrence or metastasis of the lesion being observed after six months of.

Granuloma piogénico gigante / Giant pyogenic granuloma: Case report and literature review

El granuloma piogénico es una lesión vascular adquirida, frecuente en niños y adultos jóvenes, que afecta piel y mucosas. Se presenta como un nódulo, único, de rápido crecimiento, con fácil sangrado frente a traumatismos. Su patogenia aún no está aclarada y existen variantes clínicas, dentro de ellas el granuloma piogénico gigante. Se presenta el caso de una paciente con un cuadro clínico e histológico de granuloma piogénico gigante, tratándose de una variante poco frecuente, la cual plantea diagnósticos diferenciales con otros tumores como melanoma amelanótico.

Pyogenic granuloma is an acquired vascular lesion affecting skin and mucous membranes, mostly in children and young adults. It presents as an unique nodule with fast growing and easy bleeding after minimal trauma. Its pathogenesis is still not clear and there are clinical variants, among them the giant pyogenic granuloma. A female patient with a clinical and histological giant pyogenic granuloma is reported. It is a rare entity which presents differential diagnosis with other tumors such as amelanotic melanoma.

Hemangiopericitoma: reporte de 3 casos / Hemangiopericytoma: a report of 3 cases and a literature review

Introduction: The hemangiopericytoma (HPC) is a rare vascular tumor that can be potentially malignant. It can be found in any part of the body but usually in the lower extremities or the retroperitoneum. Because its potential malign nature, it's necessary to perform oncological resections when are operating on. Methods: Three cases are presented. Results: One of them was located at the sacrococcigeal space, being the second reported case in the international literature. The other cases were in cervical and adrenal localization.

Introducción: El hemangiopericitoma (HPC) es un tumor vascular raro y potencialmente maligno que puede localizarse en cualquier parte del cuerpo, con mayor frecuencia en extremidades inferiores y retrope-ritoneo. Su potencial malignidad da la necesidad de realizar resecciones oncológicas al operarlos. Material y Método: Se presentan tres casos tratados por nuestro grupo. Resultados: Uno de ellos tiene localización sacra siendo, hasta donde sabemos, el segundo en ser publicado. Otro es de localización suprarrenal y el tercero es cervical.

Sarcoma de Kaposi. Una revisión / Kaposi's sarcoma

El Sarcoma de Kaposi es un tumor vascular de la piel, más frecuente en hombres mayores de 50 años, de larga evolución y baja mortalidad. Cada día cobran mayor interés los elementos epidemiológicos, como su relación con el SIDA (aumento de incidencia en jóvenes y niños, con una agresividad no habitual) y con la inmunosupresión en pacientes transplantados. Se atribuye una fuerte relación del Herpesvirus 8 como uno de los factores etiopatogénicos, y el tratamiento se ha reorientado en base a estos, pero aún no existe una terapia 100 por ciento eficaz.

A bibliographic review was done on Kaposi's sarcoma with the purpose of updating this topic, mainly in relation to its etiopathogenic aspects and therapeutic behavior. Kaposi's sarcoma is a vascular tumor of the skin, most frequently seen in men over 50 years, which develops throughout many years, rarely causing death. Its epidemiological elements have greater significance each day, such as its relation to AIDS and immunosuppresion in kidney transplants, as well as other organs.In the past years it has been observed worldwide in young patients, even children, with an unusual aggressiveness, more lesions and dissemination in the body, due to its relation to AIDS. A strong relation to Herpesvirus 8 is considered as one of the etiopathogenic factors, and treatment has been changed accordingly, but there is no 100 percent effective therapy.

Hemangioma cavernoso gigante de seno maxilar: reporte de un caso y revisión de la literatura / Giant cavernous hemangioma of the maxillary sinus: case report and literature review

Los hemangiomas son neoplasias vasculares benignas que se presentan de preferencia en la región cervicofacial, siendo raros en las fosas nasales y excepcionales en los senos paranasales, con pocos casos reportados. Se presenta un paciente de sexo masculino y 33 años de edad, que consultó por obstrucción nasal, aumento de volumen maxilar y exoftalmo izquierdo de 2 años de evolución. La tomografía computada con contraste mostraba una masa hipodensa con realce anular del contraste en maxilar izquierdo, con extensión a la cavidad nasal, con destrucción del piso de la órbita y la pared anterior del seno maxilar. Se tomó biopsia que se informó como hemangioma cavernoso, por lo que fue manejado con embolización y escleroterapia arterial supraselectiva previa a la resección. Se realizó una maxilectomía subtotal por abordaje de Weber-Ferguson, sin recidiva al seguimiento a 15 meses. Además se comparan los hallazgos y el manejo de nuestro paciente con revisiones extranjeras.

Hemangiomas are benign vascular neoplasms that occur preferentially in the head and neck, being uncommon in the nasal cavities and exceptionally rare in the paranasal sinuses, with very few cases reported. We present the case of a 33 year old male, that consulted for nasal obstruction, maxillary enlargement and left exophthalmos of 2 years of evolution. Contrast enhanced computed tomography showed a hypódense mass with a ring enhanced lesion in the left maxilla, extending to the nasal cavity, with orbital floor and anterior wall of the maxillary sinus destruction. A biopsy sample was informed as cavernous hemangioma. Accordingly, it was treated by supraselective arterial embolization and sclerotherapy previous to surgical resection. A subtotal maxillectomy was performed following the Weber-Ferguson approach, with no recidives after a 15 month follow-up. In addition, we compare our findings and patient management with those reviewed in the literature.

Miopericitoma em fossa nasal / Myopericytoma in nasal cavity

Introdução: Os miopericitomas representam cerca de 1 % dos tumores vasculares, sendo relativamente comum na região de cabeça e pescoço, 25% dos casos, e raro nas cavidades nasais e paranasais. Objetivo: Descrever um caso de miopericitoma em fossa nasal. Relato do Caso: Apresentamos um caso de paciente adulta, do sexo feminino, com queixas de obstrução nasal, dor em região de fossas nasais e epistaxe eventual em fossa nasal direita, apresentando massa angiomatosa e facilmente sangrante, não pulsátil, ocupando toda fossa nasal direita. Comentários Finais: Os mopericitomas são tumores vasculares incomuns, raramente localizados na cavidade nasal e nos seios paranasais. Devem ser incluídos no diagnóstico diferencial das massas bem delimitadas, vasculares e de crescimento lento à tomografia computadorizada.

Introduction: The myopericytomas represent about 1% of the vascular tumors, is relatively common in the region of head and neck, 25% of the cases, and uncommon in the nasal and paranasal cavities. Objective: To describe one case of myopericytoma in nasal cavity. Case Report: We present a case of an adult patient, of the female sex, with complaints of nasal obstruction, pain in the nasal cavities region and eventual epistaxis in the right nasal cavity, which present an angiomatous and easily bleeding, non-pulsatile mass occupying all the right nasal cavity. Final Considerations: The myopericytomas are uncommon vascular tumors, rarely located in the nasal cavity and in the paranasal sinuses. They must be included in the differential diagnosis of the well delimited, vascular and slow growth masses upon computed tomography.

Tratamiento con vincristina de tres tumores vasculares con fenómeno de Kasabach-Merritt / Vincristine treatment of three vascular tumors with Kasabach-Merritt phenomenon

El fenómeno de Kasabach-Merrit (FKM) es una coagulopatía por consumo que se presenta en las primeras semanas de la vida. Se caracteriza por presentar coagulopatía por consumo, con trombocitopenia y anemia hemolítica microangiopática, asociado a un tumor vascular. El objetivo de este trabajo es presentar tres pacientes con tumores vasculares que manifestaron FKM y fueron tratados con vincristina como droga de segunda línea, atendidos en el Servicio de Dermatología del Hospital de Pediatría J. P. Garrahan. Dos pacientes presentaron el tumor al nacimiento y otro a partir de los dos meses de vida. Dos fueron niñas y uno varón.Todos los pacientes tenían una trombocitopenia severa (3 000/mm_), bajos niveles de fibrinógeno y dimero D elevado. Los tumores estaban localizados en región proximal de miembro inferior, tronco y miembro superior, y región cervical. Ninguno de nuestros pacientes tuvo compromiso de órganos internos. El diagnóstico histológico en dos de ellos fue de hemangioendotelioma kaposiforme (HEK). Los corticoides fueron el tratamiento de primera linea: metilprednisona 3mg/kg/día por vía oral. En un paciente el tumor continuó creciendo a pesar de haber asociado interferón alfa 2 a la corticoterapia y realizarle secundariamente una embolización. Dada la falta de respuesta clínica y hematológica, se decidió iniciar tratamiento con vincristina 1mg/m2/dosis/IV semanal. Todos los pacientes normalizaron los parámetros hematológicos, con franca mejoría clínica, dos pacientes a la quinta y otro a la sexta dosis de vincristina, con involución gradual del tamaño del tumor. Ninguno de los pacientes presentó complicaciones secundarias al tratamiento ni recidivas de su enfermedad a la fecha. La duración promedio de tratamiento fue de 35±6 días. Podemos concluir que el uso de vincristina es considerado en la actualidad una droga de segunda línea en el tratamiento de tumores vasculares con FKM (AU)

Kasabach-Merritt phenomenon (KMP) is a consumptive coagulopathy that typicallly presents in the first few weeks of life. It is characterized by a triad of vascular tumor, thrombocytopenia and coagulopathy. We reviewed the clinical and hematologic data and response to therapeutic with vincristine in three patients who had a vascular tumor and KMP at the Dermatology Department of Hospital de Pediatría J. P. Garrahan.Tumors were present at birth in two patients and in one at two months old. Two were girls and one was a boy. All patients had severe thrombocytopenia (Lowest platelets count 3 000/mm_), consumption of fibrinogen and lower D-dimer levels.Tumors were localized on proximal lower limb, trunk and upper limb and cervical area. None of our patients had internal involvement. Histopathology finding in two of them was kaposiform hemangioendothelioma. First line of treatment was prednisolone 3-5mg/kg/day. In one patient the tumor size continued to increase in spite of simultaneous treatment with corticosteroid and interferon alfa-2a plus embolization. After corticosteroids treatment failure, correction of coagulopathy and tumor regression occurred in our three patients after 5 to 6 doses of vincristine 0.5-1mg/m_ IV weekly with almost complete tumor regression and correction of coagulopathy.The average duration of treatment was 35±6days. None of the patients developed complications due to this intervention nor experienced recurrence of the tumor.The use of vincristine is currently a second line treatment of vascular tumors with KMP (AU)