Clinicopathological features of fibrin-associated diffuse large B-cell lymphoma: a report of six cases / 中华病理学杂志

Objective: To investigate the clinical, pathological and immunophenotypic features, molecular biology and prognosis of fibrin-associated large B-cell lymphoma (LBCL-FA) in various sites. Methods: Six cases of LBCL-FA diagnosed from April 2016 to November 2021 at the Beijing Friendship Hospital, Capital Medical University, Beijing, China and the First Affiliated Hospital, Wenzhou Medical University, Wenzhou, China were collected. The cases were divided into atrial myxoma and cyst-related groups. Clinical characteristics, pathological morphology, immunophenotype, Epstein Barr virus infection status, B-cell gene rearrangement and fluorescence in situ hybridization of MYC, bcl-2, bcl-6 were summarized. Results: The patients' mean age was 60 years. All of them were male. Three cases occurred in atrial myxoma background, while the others were in cyst-related background, including adrenal gland, abdominal cavity and subdura. All cases showed tumor cells located in pink fibrin clot. However, three cyst-related cases showed the cyst wall with obviously fibrosis and inflammatory cells. All cases tested were non germinal center B cell origin, positive for PD-L1, EBER and EBNA2, and were negative for MYC, bcl-2 and bcl-6 rearrangements, except one case with MYC, bcl-2 and bcl-6 amplification. All of the 5 cases showed monoclonal rearrangement of the Ig gene using PCR based analysis. The patients had detailed follow-ups of 9-120 months, were treated surgically without radiotherapy or chemotherapy, and had long-term disease-free survivals. Conclusions: LBCL-FA is a group of rare diseases occurring in various sites, with predilection in the context of atrial myxoma and cyst-related lesions. Cyst-related lesions with obvious chronic inflammatory background show more scarcity of lymphoid cells and obvious degeneration, which are easy to be missed or misdiagnosed. LBCL-FA overall has a good prognosis with the potential for cure by surgery alone and postoperative chemotherapy may not be necessary.

Angiomixoma agresivo pélvico: reporte de caso y revisión / Aggressive pelvic angiomyxoma: case report and revision / Angiomixoma agressivo: relato de caso e revisão

El angiomixoma agresivo pélvico es un tumor de partes blandas extremadamente raro, del cual se han reportado alrededor de 350 casos hasta la fecha. A continuación se reporta el caso clínico de una paciente que presentó dicho tumor y se describe el proceso diagnóstico y su tratamiento quirúrgico. Por otro lado, se realiza una breve revisión de la literatura disponible hasta el momento sobre el tema.

Aggressive pelvic angiomyxoma is an extremely rare soft tissue tumor, of which around 350 cases have been reported to date. This article aims at reporting the case of a patient presenting said tumor, as well as describing its diagnostic approach and its surgical treatment. On the other hand, it aims at briefly reviewing the available literature on the subject.

O angiomixoma pélvico agressivo é um tumor extremamente raro dos tecidos moles, tendo sido descritos até à data cerca de 350 casos. Relatamos o caso de uma doente que apresentava este tumor e descrevemos o processo de diagnóstico e tratamento cirúrgico. É também feita uma breve revisão da literatura disponível até à data sobre o assunto.

Tratamento cirúrgico do mixoma odontogênico sem ressecção maxilar: relato de caso / Surgical Treatment od Odontogenic Mixoma Without Maxillary Resection: Case Report / Tratamiento Quirúrgico del Mixoma Odontogénico Sin Resección Maxilar: Informe de Caso

Mixoma Odontogênico é um tumor de origem mesenquimal raro, de desenvolvimento lento e agressivo que acomete indivíduos entre os 10 e 40 anos de idade e principalmente, do gênero feminino. Este estudo teve como principal objetivo descrever um caso clinico de tratamento cirúrgico do mixoma odontogênico sem ressecção maxilar em uma paciente do gênero feminino que compareceu ao Ambulatório de Patologia Oral e Maxilo Facial, da Faculdade de Odontologia da UNIRG, na cidade de Gurupi-TO - Brasil. A paciente foi submetida ao tratamento cirúrgico conservador, através da curetagem e enucleação total do tumor. A proservação foi realizada em períodos de 12 meses, 24 meses e 48 meses aonde pode-se observar a sequencial e completam reparação óssea, inclusive a permanência dos dentes envolvidos que foram submetidos a tratamento endodôntico com total remodelação da lâmina dura e do ligamento periodontal... (AU)

Odontogenic Myxoma (OM) is a rare tumor of mesenchymal origin, of slow and aggressive development that affects individuals between 10 and 40 years of age and mainly female. This study aimed to describe a clinical case of surgical treatment of odontogenic myxoma with out maxillary resection in a female patient who attended the Outpa tient Clinic of Oral Pathology and Facial Maxillo, of UNIRG Dental School, in the city of Gurupi-TO - Brazil. The patient underwent con servative surgical treatment through curettage and total enucleation of the tumor. Proservation was carried out in periods of 12 months, 24 months and 48 months where it was possible to observe the sequential and complete bone repair including the permanence of the involved teeth that underwent endodontic treatment with total remodeling of hard blade and of the periodontal ligament... (AU)

El mixoma odontogénico es un tumor de origen mesenquimal poco frecuente, de desarrollo lento y agresivo que afecta a individuos entre 10 y 40 años de edad y principalmente mujeres. El objetivo principal de este estudio fue describir un caso clínico de tratamiento quirúrgico de mixoma odontogénico sin resección maxilar en una paciente femenina que asistió a la Clínica Ambulatoria de Patología Oral y Maxilo Facial, de la Facultad de Odontología de UNIRG, en la ciudad de Gurupi-TO - Brasil. El paciente se sometió a tratamiento quirúrgico conservador mediante legrado y enucleación tumoral total. La conservación se realizó en periodos de 12 meses, 24 meses y 48 meses donde es posible observar reparación ósea secuencial y completa, incluyendo la permanencia de los dientes implicados que fueron sometidos a tratamiento endodóntico con remodelación total de la durancia y ligamento periodontal... (AU)

Mixoma auricular gigante como diagnóstico diferencial de disnea: a propósito de un caso y revisión de la literatura / Giant atrial myxoma as differential diagnosis of dyspnea: a case report and literature review

Cardiac myxomas are the most common benign primary tumors and are most often located in the left atrium at the level of the interatrial septum, with the characteristic of being pedunculated and highly mobile, which is why they sometimes interfere with the functioning of the mitral valve, generating variable degrees of stenosis and mitral insufficiency. Diagnosis is by echocardiography and treatment is surgical resection. We present the case of a patient with a large atrial myxoma and severe double mitral lesion.

Síncope por mixoma auricular: a propósito de un caso clínico / Syncope due to atrial myxoma: presentation of a clinical case / Síncope por mixoma atrial: a propósito de um caso clínico

Las neoplasias cardíacas se dividen en tumores primarios y secundarios; estos últimos son 30 veces más frecuentes. La mayoría de las neoplasias cardíacas son benignas y dentro de este grupo destaca el mixoma auricular. La presentación clínica es muy variable en relación con su ubicación, tamaño y movilidad, y los hallazgos a nivel de las pruebas complementarias son inespecíficos. Dado que su historia natural muchas veces pasa inadvertida, el mixoma puede culminar en la obstrucción auriculoventricular, obstrucción del tracto de salida ventricular o incluso embolias sistémicas; de esta forma, es el causante de sintomatología severa como síncope, insuficiencia cardíaca y muerte súbita cardíaca. Su manifestación clínica como un síncope es bastante frecuente. Se expone el caso de un paciente de sexo masculino de 81 años que consultó en el servicio de urgencias por un síncope. Durante su valoración, se identificó el mixoma auricular como etiología de este.

Cardiac neoplasms are divided into primary and secondary tumors, the latter are 30 times more frequent. Most are benign and within this group the atrial myxoma stands out. The clinical presentation is very variable in relation to its location, size and mobility, and the findings in the complementary tests are nonspecific. Since its natural history often goes unnoticed, the myxoma can culminate in atrial ventricular obstruction, ventricular outflow tract obstruction or even systemic embolism, causing severe symptoms such as syncope, heart failure and sudden cardiac death. Its clinical manifestation as syncope is quite common. The case of an 81-year-old male patient who consulted at the emergency department for syncope is presented, in which the atrial myxoma was subsequently identified as the etiology of the event during the further evaluation.

As neoplasias cardíacas dividem-se em tumores primários e secundários, estes últimos são 30 vezes mais frequentes. A maioria são benignas e dentro deste grupo destaca-se o mixoma auricular. A apresentação clínica é muito variável em relação com sua localização, tamanho e mobilidade, e os achados a nível dos testes complementares são inespecíficos. Dado que a sua história natural muitas vezes passa despercebida, o mixoma pode culminar na obstrução aurículo-ventricular, obstrução do tracto de saída ventricular ou mesmo embolias sistémicas, sendo assim o causador de sintomatologia severa como síncope, insuficiência cardíaca e morte súbita cardíaca. Sua manifestação clínica como síncope é bastante comum. Apresenta-se o caso de um paciente de sexo masculino de 81 anos que consultou no serviço de urgências para um síncope. Durante a sua avaliação, se identificou mixoma auricular como etiologia deste

Tumores cardíacos pediátricos: características de imagens ecocardiográficas / Pediatric cardiac tumors: echocardiographic imaging features

Tumores cardíacos (TC) em crianças são lesões muito raras, mas seu diagnóstico é fundamental para a conduta adotada para o paciente. A ecocardiografia é a modalidade de imagem cardiovascular mais utilizada na prática clínica para o diagnóstico inicial de TC em pacientes pediátricos. Conhecer as características ecocardiográficas das TCs pode possibilitar um diagnóstico cada vez mais precoce e a identificação de sua etiologia mais provável. Os TCs primários e benignos são os mais frequentes na população pediátrica. Entre os TCs benignos, os mais frequentes em fetos e neonatos são rabdomiomas e teratomas. Em crianças e adolescentes, rabdomiomas e fibromas são os mais comuns. Neste artigo, descrevemos as características ecocardiográficas dos principais TCs em idades pediátricas.(AU)

Cardiac tumors (CTs) in children are very rare, but their diagnosis is crucial for patient management. Echocardiography is the most commonly used cardiovascular imaging modality in clinical practice for the initial diagnosis of CTs in pediatric patients. Knowing the echocardiographic characteristics of CTs can enable an increasingly early diagnosis and the identification of its most likely etiology. Primary and benign CTs are the most frequent types in the pediatric population. Among benign CTs, the most frequent in fetuses and neonates are rhabdomyomas and teratomas. In children and adolescents, rhabdomyomas and fibromas are more common. Here we describe the echocardiographic characteristics of the most common CTs in pediatric patients.(AU)

Fast growth rate of a right atrial myxoma

ABSTRACT Primary cardiac tumors are rare, with an incidence between 0.0017 and 0.19%, and are asymptomatic in up to 72% of cases. Approximately 75% of tumors are benign, and nearly 50% of these are myxomas. Concerning location, 75% of myxomas are in the left atrium, 15 to 20% in the right atrium, and more rarely in the ventricles. The finding of cardiac myxomas usually implies immediate surgical excision to prevent embolic events and sudden cardiac death. Reports with documented growth rate are rare, and the actual growth rate remains a controversial issue. We report the rapid growth rate of a right atrial myxoma in an oligosymptomatic 69-year-old patient, with negative previous echocardiographic history in the last two years, who refused surgery upon diagnosis, enabling monitoring of myxoma growth.

Ultrasonographic features of cutaneous myxomas in a patient with Carney complex

Abstract Carney complex is a rare genodermatosis characterized by cardiac and cutaneous myxomas, among other tumors. In the majority of cases, cutaneous myxomas precede the diagnosis of cardiac myxomas, which are the main cause of death in these patients. Despite the fact that the diagnosis of cutaneous myxomas is histopathological, high-frequency ultrasonography plays an essential role in the differential diagnosis with other cutaneous and subcutaneous tumors. The authors of the present study describe, for the first time in the literature, the ultrasonographic features of both variants of cutaneous myxomas, superficial and subcutaneous, in a patient with a Carney complex.

Mixofibroma odontogénico, reporte de un caso con seguimiento a cinco años / Odontogenic myxofibroma, case report with 5 years of follow-up

El mixoma odontogénico es una neoplasia de origen mesenquimal, con crecimiento lento pero localmente invasivo y agresivo, que cuenta con un aspecto histológico característico. Radiográficamente, la mayoría de las lesiones tienen un aspecto radiolúcido multilocular, descrito en la literatura como «panal de abejas¼ o «burbujas de jabón¼, que ocasionan desplazamiento dental y expansión corticales. En el ámbito histopatológico, se caracteriza por células estelares o fusiformes con prolongaciones citoplasmáticas, en una sustancia mucoide o mixoide con presencia o no de colágena, cuando contiene grandes cantidades de tejido fibroso celular maduro se le llama mixofibroma. Se presenta un caso clínico de hombre de 38 años, que acude al Hospital General de Uruapan, Michoacán con aumento de volumen en región maxilar derecha; se toma biopsia incisional y se emite el diagnóstico histopatológico de mixofibroma odontogénico, 33.6% de los casos se presenta en el maxilar, con una distribución uniforme por todas las áreas llegando a erosionar el seno maxilar. Tiene una tasa de recurrencia de 10 a 33%, y el tratamiento ideal es la resección en bloque con márgenes de seguridad (AU)

Odontogenic myxoma is a slow-growing locally invasive intraosseous lesion with defined microscopic appearance. Radiographic appearance, is described as multilocular radiolucent lesion, which is reported in scientific literature as «honeycomb¼ or «soap bubbles¼ appearance most of the time; it exhibits dental displacement and cortical expansion. Histopathologically, stellate and spindle cells with cytoplasmic processes are observed in a mucoid or myxoid substance, with or without collagen; when it contains large amounts of mature cellular fibrous tissue, it is called myxofibroma. The present article presents the case of a 38 year old male, who attended the General Hospital in Uruapan, Michoacán due to an increased volume in the right maxillary region. Incisional biopsy was performed and the histopathological diagnosis of odontogenic myxofibroma was emitted. 33.6% of cases occur in the maxilla with a uniform distribution throughout the areas, and can erode the maxillary sinus. It has a recurrence rate of 10 to 33%, therefore ideal treatment is bloc resection leaving safety margins (AU)

A novel mutation in PRKAR1A gene in a patient with Carney complex presenting with pituitary macroadenoma, acromegaly, Cushing's syndrome and recurrent atrial myxoma

SUMMARY Carney complex (CNC) is a rare syndrome of multiple endocrine and non-endocrine tumors. In this paper we present a 23-year-old Iranian woman with CNC who harbored a novel mutation (c.642dupT) in PRKAR1A gene. This patient presented with pituitary macroadenoma, acromegaly, recurrent atrial myxoma, Cushing's syndrome secondary to primary pigmented nodular adrenocortical disease and pigmented schwanoma of the skin. PRKAR1A gene was PCR amplified using genomic DNA and analyzed for sequence variants which revealed the novel mutation resulting in substitution of amino acid cysteine instead of the naturally occurring valine in the peptide chain and a premature stop codon at position 18 (V215CfsX18). This change leads to development of tumors in different organs due to lack of tumor suppressive activity secondary to failure of synthesis of the related protein.

Mixoma odontogênico de mandíbula tratado por curetagem, osteotomia periférica e uso de solução de carnoy: relato de caso mandibular / Odontogenic myxoma treated by curettage, peripheral osteotomy and uso of carnoy's solution: case report

O mixoma odontogênico é um tumor benigno que acomete os maxilares, de rara ocorrência que tem origem a partir do componente ectomesenquimatoso de um germe dentário, seja ao nível da papila dentária, ao nível do folículo ou ainda ao nível do ligamento periodontal. Tem predileção pelo sexo feminino, podendo comprometer com mais significância a faixa etária da segunda à quarta década de vida. O tratamento dos mixomas odontogênicos pode ser conservador ou radical. Com relação à escolha do tratamento, deve o cirurgião levar em consideração principalmente a extensão do envolvimento da lesão. O tratamento conservador pode ser considerado como primeira escolha, evitando efeitos associados a morbidade, comprometimento de estruturas anatômicas nobres e diminuição da qualidade de vida. Por se tratar de lesão recidivante, métodos complementares de tratamento têm sido empregados, tais como osteotomia periférica, uso do nitrogênio líquido e a solução de Carnoy. Este trabalho teve como objetivo relatar um caso de mixoma odontogênico em corpo mandibular de paciente do sexo feminino, leucoderma, 25 anos, solteira, natural de Recife-PE- Brasil, tratado de forma conservadora através de curetagem, osteotomia periférica e uso de solução de Carnoy, que até o presente momento não ocorreu sinais de recidiva... (AU)

Odontogenic myxoma is a rare benign tumor that affects the jaws. It´s originates from the ectomesenchymatous component of a dental germ, either at the level of the dental papilla, at the level of the follicle or at the level of the periodontal ligament. It has a predilection for the female sex, being able to compromise with more significance the age group from the 2nd to the 4th. decade of life. The treatment of odontogenic myxomas can be conservative or radical. Regarding the choice of treatment, the surgeon must take into account mainly the extent of the lesion's involvement. Conservative treatment can be considered as the first choice, avoiding effects associated with morbidity, impairment of noble anatomical structures and decreased quality of life. As it is a recurrent lesion, complementary treatment methods have been used, such as peripheral osteotomy, use of liquid nitrogen and Carnoy's solution. This study aimed to report a case of odontogenic myxoma in the mandibular body of a female patient, leucoderma, 25 years old, single, born in Recife-PE- Brazil, treated conservatively through curettage, peripheral osteotomy and use of a solution of Carnoy, that until now there have been no signs of recurrence... (AU)

Mixoma auricular izquierdo gigante que causa obstrucción valvular mitral, hipertensión pulmonar y tromboembolia pulmonar / Giant atrial myxoma causing mitral valve obstruction, pulmonary hypertension and pulmonary thromboembolism

Resumen: Los tumores cardíacos pueden ser primarios o, más frecuentemente secundarios o metastásicos. Entre los tumores primarios es más frecuente el mixoma, cuya ubicación más común es en la aurícula izquierda. Las manifestaciones clínicas son diversas, producidas principalmente por obstrucción mecánica, embolizaciones, y manifestaciones constitucionales. Se comunica el caso de un paciente de 32 años, con cuadro clínico de insuficiencia cardíaca, hipertensión pulmonar severa y tromboembolismo pulmonar bilateral. Se hizo el diagnóstico de mixoma auricular izquierdo. Se resecó el tumor y se manejó la hipertensión pulmonar desde el ingreso al hospital con inhibidores de la fosfodiesterasa asociado a anticoagulación. Se discute el tema dando énfasis a aspectos fisiopatológicos involucrados tanto en la hipertensión pulmonar como en la presencia de tromboembolia pulmonar.

Abstract: Cardiac tumors may be primary or, more frequently secondary or associated to metastasis. Atril myxoma es the most frequent primary tumor, usually located in the left atrium. Clinical manifestations include those due to mitral valve occlusión, emboli and general non spedific symptoms and signs. Herein we report the clinical case of a 32 year old patient with severe pulmonary hypertension and bilateral pulmonary embolism. The tumor was extirpated, and he received phosphoro-diesterase inhiborts and anticoagulants. Subsequent clinical course was satisfactory. A brief discussion of this condicion is included.

Multidisciplinary approach to right ventricular myxoma

Abstract Right ventricular (RV) myxoma that obstructs the RV outflow tract is rare. Multimodality imaging is crucial due to the curved and triangular shape of the RV anatomy. Incomplete resection by the right atrial approach in cardiac myxomas may be prevented by preoperative imaging with echocardiography, computed tomography and magnetic resonance imaging to provide detailed visualization. Right ventriculotomy may be an alternative approach to the isolated atrial approach to get complete resection of RV myxoma in suitable patients. The preferred surgical treatment is not well defined for ventricular myxomas and careful preoperative planning is essential. Surgical resection should be performed as soon as possible to avoid outflow tract obstruction, which might result in sudden death. The collaboration between cardiologist and heart surgeon and the effective use of imaging tools are essential for successful treatment. In this article, diagnosis and treatment and the heart team approach to RV myxoma are discussed with a demonstrative patient.

Síndrome de Mazabraud: reporte de un caso / Mazabraud syndrome: A case report

El síndrome de Mazabraud es una rara asociación de lesiones del sistema musculoesquelético. Resulta de la combinación de uno o varios mixomas intramusculares y displasia fibrosa. Presentamos a un hombre de 46 años con lesiones óseas compatibles con displasia fibrosa asociada a mixoma intramuscular configurando un síndrome de Mazabraud y una revisión de la bibliografía. Nivel de Evidencia: IV

Mazabraud's Syndrome is a rare association of lesions to the musculoskeletal system. It results from the combination of one or more intramuscular myxomas and fibrous dysplasia. We present a case of a 46-year-old man with bone lesions compatible with fibrous dysplasia associated with intramuscular myxoma, configuring Mazabraud's Syndrome and a review of the literature. Level of Evidence: IV

Multiple cerebral aneurysms originating from previously resected cardiac myxoma

Primary cardiac tumors are very rare, accounting for 0,001 ­0,03% in autopsy series, cardiac myxoma (CM) account for approximately 50% of them. Association between CM and cerebral aneurysm is well documented in this condition but the occurrence of cerebral aneurysm after resection of CM is very rare, with only 40 reports in medical literature. We present a case of a 45 years old female patient with multiple cerebral aneurysms 2 years after a successful resection of cardiac myxoma, submitted to radiosurgery for 2 bigger aneurysms with good results. This report ads to literature new clinical findings and a hypothesis of radiosurgery as an effective option of treatment for cerebral aneurysms with origin in CM. Keywords: Cardiac Myxoma; Cerebral aneurysm; Radiosurgery; Neurosurgery.

Mixoma auricular izquierdo como causa de accidente cerebral isquémico transitorio e infarto agudo de miocardio con elevación del ST / Left atrial myxoma as a cause of a transient ischaemic stroke and acute myocardial infarction with ST elevation

Resumen Los mixomas son los tumores cardíacos primarios más comunes. La mayoría tiene curso benigno y pueden tratarse de hallazgos incidentales o ser casi asintomáticos. Los síntomas dependen de su ubicación, en cuyo caso la más frecuente es la aurícula izquierda, de ahí que el riesgo embólico sea elevado. El principal lugar de embolia es el sistema nervioso central; sin embargo, en ocasiones puede afectar los sistemas respiratorio, periférico o coronario(1). Se expone el caso de una paciente con mixoma auricular izquierdo, el cual se manifiesta con accidente cerebral isquémico transitorio, seguido de infarto agudo de miocardio con elevación del segmento ST en la pared ínfero-posterior.

Abstract The myxomas are the most common primary cardiac tumours. The majority of them are benign and can be an incidental finding or be almost asymptomatic. The symptoms depend on their location, with the most common being the left atrium, which carries an increased risk of clots. The main effects of the clots are on the central nervous system, but occasionally they can involve the respiratory, peripheral, or coronary system. The case is presented of a patient with left atrial myxoma, as well as a transient ischaemic stroke and acute myocardial infarction with ST elevation in the inferior-posterior wall.

Masa auricular derecha: la importancia del ecocardiograma al momento del diagnóstico de fibrilación auricular en el enfoque terapéutico / Right atrial mass: The importance of cardiac ultrasound at the time of diagnosis of atrial fibrillation in the therapeutic approach

Resumen Se presenta el caso de un varón de 70 años de edad, quien debutó con fibrilación auricular no valvular, en quien, dos meses después y mediante ecocardiografía, se objetivaron dos masas contiguas en la aurícula derecha. Las masas eran dependientes de la válvula de Eustaquio, alcanzaban el septo interauricular y se asemejaban a un mixoma. Ambas fueron resecadas mediante cirugía, sin complicaciones intraoperatorias ni postoperatorias. El estudio anatomo-patológico concluyó que eran compatibles con trombos sin componente tumoral. Durante el seguimiento permaneció asintomático y en ritmo de fibrilación auricular.

Abstract The case is presented on a 70 year-old male who debuted with non-valvular atrial fibrillation. Two months later, and using cardiac ultrasound, two contiguous masses were observed in the right atrium. The masses were hanging from the Eustachian (inferior vena cave) valve, reached the interatrial septum and resembled a myxoma. Both were surgically resected, with no intra- or post-operative complications. The patient remained asymptomatic and in atrial fibrillation rhythm during follow-up.

Aggressive angiomyxoma of the vagina: Case report and review of the literature / Angiomixoma agresivo de vagina: reporte de caso y revisión de la literatura

RESUMEN Objetivos: Presentar el caso de una paciente con diagnóstico de angiomixoma agresivo de vagina, y hacer una revisión de la literatura del diagnóstico, tratamiento y pronóstico. Materiales y métodos: Mujer de 46 años de edad, quien consulta por disuria y sensación de masa en vagina. Tras la exploración física y los estudios imagenológicos se observa tumoración en vagina con extensión hacia cavidad pélvica, la cual es extraída por cirugía vaginal. La patología quirúrgica mostró angiomixoma agresivo de vagina. La lesión presentó recurrencia al tercer mes posoperatorio requiriendo una nueva cirugía por vía retrorrectal. Se realizó una búsqueda en las bases de datos Medline vía PubMed, LILACS, SciELO y Google Scholar, con los términos: "Angiomixoma", "Agresivo" y "Vagina",incluyendo artículos de revisión, reportes y series de caso en inglés y español publicados desde 1995. Se extrajo información sobre el diagnóstico referente a síntomas, signos, pruebas de inmunohistoquímica e imágenes utilizadas, el tipo de tratamiento -quirúrgico o de otro tipo- y el pronóstico. Se hace descripción narrativa de los hallazgos Resultados: Se identificaron 23 títulos, de los cua- les 14 reportes de caso, 2 series de casos clínicos y un artículo de revisión cumplieron los criterios de inclusión. El 65 % de las pacientes tenía entre 30 y 50 años. El diagnóstico se hizo con estudios de inmunohistoquímica en 8 de los casos y se utilizaron imágenes diagnósticas en 12 casos; la tomografía mostró mejor delimitación de las lesiones. El trata- miento quirúrgico se aplicó en todos los reportes y se complementó con terapia hormonal en 4 casos. Se hizo seguimiento a 14 de los 17 casos reportados. Conclusión: El angiomixoma agresivo de vagina es una entidad poco frecuente. Es necesario evaluar la utilidad de las diferentes pruebas de inmunohistoquímica en los casos identificados como angiomixoma agresivo por histopatología. Se requiere evaluación del tratamiento hormonal como coadyuvante de la cirugía. El pronóstico es bueno.

ABSTRACT Objectives: To present the case of a patient diagnosed with aggressive angiomyxoma of the vagina and to conduct a review of the diagnosis, treatment and prognosis of this disease condition. Materials and Methods: A 46-year old female patient complaining of dysuria and vaginal mass sensation. Physical exploration and imaging studies revealed a tumor extending into the pelvic cavity. The mass was resected through a vaginal approach and pathology of the surgical specimen showed an aggressive angiomyxoma of the vagina. New retrorectar surgery was performed three months after the initial resection because of recurrence. A search was conducted in the Medline via PubMed, Lilacs, Scielo and Google Scholar databases using the terms "Angiomyxoma," "Aggressive" and "Vagina." The search included review articles, case reports and case series published in English and Spanish since 1995. The information extracted included diagnosis, symptoms, signs, immunohistochemistry and imaging studies used, type of treatment surgical or other - and prognosis. Findings are described in narrative form. Results: Overall, 23 titles were identified, of which 14 case reports, 2 clinical case series and 1 review article met the inclusion criteria. Sixty-five per cent of the patients were between 30 and 50 years of age. Diagnosis was made by immunohistochemistry in 8 cases, and diagnostic imaging was used in 12 cases. Computed tomography showed sharper contours of the lesions. Surgical treatment was applied in all reports, supplemented by hormonal therapy in 4 cases. There was follow-up in 14 of the 17 cases reported. Conclusion: Aggressive angiomyxoma of the vagina is rare. Assessment of the role of various immunohistochemical tests is needed in cases identified as aggressive angiomyxoma on histopathology. Evaluation of hormonal treatment as an adjunct to surgery is required. Prognosis is good.