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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.
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.
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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.
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The administration of nanoparticles (NPs) first faces the challenges of evading renal filtration and clearance of reticuloendothelial system (RES). After that, NPs infiltrate through the expanded endothelial space and penetrated the dense stroma of tumor microenvironment to tumor cells. As long as possible to prolong the time of NPs remaining in tumor tissue, NPs release active agent and induce pharmacological action. This review provides a comprehensive summary of the physical and chemical properties of NPs and the influence of various biological factors in tumor microenvironment, and discusses how to improve the final efficacy through adjusting the characteristics and structure of NPs. Perspectives and future directions are also provided.
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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.
Subject(s)
Humans , Male , Adult , Retinal Detachment/diagnosis , Glaucoma, Neovascular/diagnosis , Choroid Neoplasms/diagnosis , Choroid Neoplasms/pathology , Hemangioma/diagnosis , Hemangioma/pathology , Eye Enucleation , Diagnosis, DifferentialABSTRACT
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)
Subject(s)
Humans , Male , Adult , Vascular Diseases/diagnosis , Angiolymphoid Hyperplasia with Eosinophilia/etiologyABSTRACT
Objective To explore the effect of invasion and migration of hepatocellular carcinoma (HCC) endothelial cells (TECs) affected by overexpression of microRNA-3178 (miR-3178) through the transfection of miR-3178 mimic.Methods Real-time polymerase chain reaction (Real-time PCR) was used to identify differential expression of miR-3178 in normal hepatic sinusoidal endothelial cells (HSECs) and HCC TECs.Furthermore,HCC TECs were divided into 3 groups:control (CON) group,miRNA-3178 upregulation (Mimics,up-regulation of miR-3178 expression was achieved using miR-3178 mimics transfected into HCC TECs) group and negative control (NC,negative control sequence was transfected into HCC TECs) group.RT-PCR was used to detect expression of miR-3178 in HCC TECs before and after transfection.Transfection efficiency was observed by using an inverted fluorescence microscope.HCC TECs invasionand migration were measured by matrigel invasion and transwell migration assay.EGR3 protein expression of HCC TECs were identified by Western blotting analysis.EGR3 mRNA expression of HCC TECs were identified by RT-PCR analysis.Results The results of RT-PCR showed that miR-3178 was significantly down-regulated in HCC TECs compared to HSECs (P ＜0.05),and expression of miR-3178 was significantly increased after the transcienttransfection (P ＜ 0.05).The transfection efficiency in HCC TECs was morethan 90％.Number of migrated and invaded cells and in miR-3178 group was significantly less than those in other groups.Target gene prediction software showed EGR3 was a possible candidate target.Transfection of miR-3178 mimic significantly decreased the mRNA and protein expression levels of EGR3.Conclusion MiR-3178 was downregulated in HCC TECs and overexpression of miR-3178 can specifically inhibit migration and invasion of HCC TECsin vitro through inhibiting EGR3 expression,thus,miR-3178 might be a critical targeted therapy strategv for HCC.
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AIM:To investigate the role of NFATc1 in vascular generation in the nude mice transplanted with human ovarian cancer SKOV3 cells.METHODS: NFATc1 expression was silenced by siRNA in SKOV3 cells.Human ovarian cancer transplantation nude mouse model was established by transplanting with SKOV3 cells in which the NFATc1 gene was silenced by siRNA technique.The expression of NFATc1, CXCR2, FGF-2 and PDGF-BB at mRNA and protein levels was determined by RT-PCR, Western blotting and immunohistochemical staining.The tumor growth, angiogenesis and lymphangiogenesis were also observed.RESULTS:Over-expression of NFATc1 was observed in human ovarian cancer tissues.The silencing of NFATc1 expression by siRNA decreased tumorigenesis of transplanted ovarian cancer cells in the nude mice, reduced tumor vascular generation and inhibited the expression of CXCR2, FGF-2 and PDGF-BB at mRNA and protein levels.CONCLUSION:NFATc1 is overexpressed in ovarian cancer.NFATc1 silencing regulates the tumor vascu-lar generation.NFATc1 thus has potential as a therapeutic target and for use in the diagnosis and evaluating prognosis of epithelial ovarian cancer.
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Objetivo: analizar la importancia de una metodología protocolizada para el diagnóstico y tratamiento de patologías de baja frecuencia, como el tumor fibroso solitario, y la del diagnóstico diferencial con otras entidades. Caso clínico: se trata de una paciente con lesión subcutánea de 13 cm x 3,5 cm en región geniana izquierda. Tras efectuar estudios clínicos por imágenes y anatomopatológicos, cuyo diagnóstico presuntivo era tumor de tejido blando vascularizado, se realizó la resección de la lesión y la reconstrucción de la zona intervenida, mediante un colgajo local. El diagnóstico definitivo fue tumor fibroso solitario dérmico. Conclusiones: los datos clínicos orientan las conductas diagnósticas complementarias. Los estudios por imágenes muestran las características de la lesión, pero no la definen. La planificación del tratamiento implica la preparación del paciente, la elección de la conducta terapéutica, la evaluación de las posibles complicaciones y el seguimiento del caso.
Subject(s)
Humans , Female , Middle Aged , Hemangiopericytoma/surgery , Hemangiopericytoma/classification , Hemangiopericytoma/diagnosis , Age and Sex Distribution , Argentina , Dental Service, Hospital , Diagnosis, Differential , Follow-Up Studies , Hemangiopericytoma , Magnetic Resonance Imaging/methods , Oral Surgical Procedures/methods , Plastic Surgery Procedures/methods , Surgical FlapsABSTRACT
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.
Subject(s)
Humans , Female , Child , Granuloma, Pyogenic/classification , Granuloma, Pyogenic/etiology , Cryotherapy , Electrocoagulation , LasersABSTRACT
El angioma en penacho (AP) o tufted angioma es un tumor vascular benigno poco frecuente. Aparece principalmente durante el primer año de vida y muy rara vez es congénito. Comunicamos el caso de una niña de 13 años de edad que presenta un tumor vascular congénito a nivel de la región preesternal. La biopsia confirmó un AP...
Tufted angioma is a rare benign vascular tumor. It usually appears in infancy and early childhood, however, rare cases of TA presenting at birth have been published. We report a case of a 13-year-old girl with a congenital vascular tumor on the upper trunk. A skin biopsy confirmed a tufted angioma. Key words: congenital tufted angioma; tufted angioma; vascular tumors...
Subject(s)
Humans , Adolescent , Female , Hemangioma , Skin Neoplasms , Congenital AbnormalitiesABSTRACT
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.
Subject(s)
Humans , Male , Female , Adult , Aged , Hemangiopericytoma/surgery , Hemangiopericytoma/diagnosis , Vascular Neoplasms/surgery , Vascular Neoplasms/diagnosis , Adrenal Glands , Hemangiopericytoma/pathology , Neck , Vascular Neoplasms/pathology , Sacrococcygeal RegionABSTRACT
Objective To investigate whether recombinant human endostatin can create a time window of vascular normalization prior to vascular pruning to alleviate hypoxia in Lewis lung carcinoma in mice. Methods Kinetic changes in morphology of tumor vasculature in response to recombinant human endostatin were detected under a confocal microscope with immunofluorescent staining in Lewis lung carcinomas in mice. The hypoxic cell fraction of different time was assessed with immunohistochemical staining . Effects on tumor growth were monitored as indicated in the growth curve of tumors . Results Compared with the control group vascularity of the tumors was reduced over time by recombinant human endostatin treatment and significantly regressed for 9 days. During the treatment, pericyte coverage increased at day 3, increased markedly at day 5, and fell again at day 7. The vascular basement membrane was thin and closely associated with endothelial cells after recombinant human endostatin treatment, but appeared thickened, loosely associated with endothelial cells in control tumors. The decrease in hypoxic cell fraction at day 5 after treatment was also found. Tumor growth was not accelerated 5 days after recombinant human endostatin treatment. Conclusions Recombinant human endostatin can normalize tumor vasculature within day 3 to 7, leading to improved tumor oxygenation. The results provide important experimental basis for combining recombinant human endostatin with radiation therapy in human tumors.
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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.
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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.
Subject(s)
Humans , Male , Adult , Hemangioma, Cavernous/surgery , Hemangioma, Cavernous/pathology , Hemangioma, Cavernous , Maxillary Sinus Neoplasms/surgery , Maxillary Sinus Neoplasms/pathology , Maxillary Sinus Neoplasms , Treatment OutcomeABSTRACT
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.
Subject(s)
Humans , Female , Aged , Biopsy , Hemangiopericytoma/diagnosis , Nose Neoplasms/diagnosis , Nasal Obstruction/etiologyABSTRACT
El hemangioendotelioma kaposiforme es un tumor vascular agresivo y raro que ocurre predominantemente en la infancia. Suele tener localización cutánea o retroperitoneal. Frecuentemente se asocia con el fenómeno de Kasabach-Merritt y ocasionalmente con linfangiomatosis. Las lesiones cutáneas deben ser diferenciadas clínicamente del hemangioma de la infancia, de los hemangiomas congénitos (RICH y NICH) y de otros tumores vasculares. Describimos un caso de hemangioendotelioma kaposiforme cutáneo en una niña de 10 años.
Kaposiform hemangioendothelioma is a rare aggressive vascular tumor that predominantly occurs at infancy and early childhood. It usually presents cutaneous or retroperitoneal location. It is frequently associated with Kasabach-Merritt phenomenon and occasionally with lymphangiomatosis. Cutaneous lesions must be clinically differentiated from infantile hemangiomas, congenital hemangiomas (RICH and NICH) and other vascular tumors. We present a case of cutaneous kaposiform hemangioendothelioma in a 10-year-old girl.
Subject(s)
Humans , Female , Child , Hemangioendothelioma , Neoplasms, Vascular Tissue , Diagnosis, DifferentialABSTRACT
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)
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Vincristine/therapeutic use , Kasabach-Merritt Syndrome/drug therapy , Hemangioendothelioma/drug therapyABSTRACT
El leiomiona vascular intranasal es extremadamente raro. Menos de 1% de todos los leiomiomas se localizan en cabeza y cuello y de éstos sólo 3% se encuentran en la cavidad nasal. Los síntomas clínicos más comunes son obstrucción nasal, epistaxis y dolor. Se informa el caso de un leiomioma vascular nasal en una mujer de 35 años cuyo tratamiento se hizo mediante cirugía endoscópica transnasal para resecar el leiomioma vascular nasal más grande hasta el momento se comunica en la literatura.
An intranasal vascular leiomyoma is extremely rare. Less than 1% of all leiomyomas originate in the head and neck area, and of these, only 3% are found in the nasal cavity. The most common symptoms are nasal obstruction, epistaxis and pain. A rare case of nasal vascular leiomyoma that developed in a 35 year old woman is reported. Transnasal endoscopic sinus surgery to perform a resection on the biggest nasal vascular leiomyoma ever found was made.
Subject(s)
Female , Leiomyoma , Neoplasms , Nose , Paranasal Sinuses/abnormalities , WomenABSTRACT
Masculino de 32 años de edad, con aumento de volumen progresivo en la cara lateral derecha del cuello, de 20 años de evolución. Se sometió a resolución quirúrgica sin complicaciones. La histopatología reveló un Hemangioma Cavernoso, un tumor vascular benigno poco común, que puede causar molestias estéticas y funcionales de acuerdo a su localización, así como complicaciones sistémicas relacionadas con síndromes asociados. Comúnmente aparece en etapa neonatal puede hacerlo en el adulto. No existe tendencia a la regresión. La cirugía es el tratamiento definitivo, siempre que la lesión sea circunscrita y no afecte a órganos vitales.