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An angiomyofibroblastoma is a benign tumor that may present itself in the genital tract of a reproductive age woman. Despite it being a benign neoplasia, unable to be invasive, it can reach large sizes, producing discomfort, pain, and anatomical deformation. A late diagnosis may result in increased surgical difficulty for resection. The following is the clinical case of a woman with a vulvo-perineal tumor of 6 years of evolution, with a final diagnosis of angiomyofibroblastoma, in which complete resection of the lesion was achieved with good cosmetic results. The highlight of this tumor is its slow, silent, but progressive growth, leading to diagnoses that are often confused and late, with a consequent delay in treatment. Among the common differential diagnoses are the Bartholin gland cyst and the aggressive angiomyxoma.

El angiomiofibroblastoma es un tumor benigno que puede presentarse en el tracto genital de la mujer en edad reproductiva. A pesar de ser una neoplasia benigna, sin capacidad invasora, puede alcanzar grandes tamaños, produciendo molestia, dolor y alteración importante de la anatomía. Si el diagnóstico es tardío, aumenta la dificultad quirúrgica en su resección. A continuación, se presenta el caso clínico de una mujer con un tumor vulvoperineal de 6 años de evolución, con diagnóstico final de angiomiofibroblastoma, en el que se logró la resección completa de la lesión con un buen resultado estético. La importancia de este tumor está dada por su crecimiento lento, silencioso, pero progresivo, llevando a que su diagnóstico sea en muchas ocasiones confuso y tardío, con el consecuente retraso en el tratamiento. Dentro de los diagnósticos diferenciales comunes se encuentran el quiste de la glándula de Bartholino y el angiomixoma agresivo.

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Angioleiomioma (AL) é um tumor benigno de origem perivascular que raramente é observado na cavidade oral, principalmente em localizações como a gengiva. Devido sua apresentação clínica inespecífica, os ALs podem mimetizar outras lesões orais, como tumores benignos de glândulas salivares e lesões reacionais, como o granuloma piogênico. O presente artigo objetiva relatar um caso raro de AL localizado em gengiva, em uma paciente de 19 anos. Clinicamente, a lesão apresentava-se como um tumor assintomático, oval, pedunculado, bem definido, com superfície lisa, consistência fibrosa e cor eritematosa, semelhante a um granuloma piogênico, Uma biópsia excisional foi realizada e o fragmento foi encaminhado para análise histopatológica, que revelou uma proliferação vascular de diversos calibres, contendo paredes musculares espessas e proliferação muscular adjacente, além de infiltrado inflamatório, predominantemente crônico, hemácias extravasadas e área de ulceração, consistente com o diagnóstico de AL inflamado. A histopatologia desempenha um papel importante no diagnóstico final de lesões raras e com características clínicas inespecíficas. A excisão cirúrgica da lesão é o tratamento de escolha mais eficaz para os ALs orais. (AU)

Angioleiomyoma (AL) is a benign tumor of smooth muscle of perivascular origin that is rarely seen in the oral cavity, mainly in locations like the gingiva. Due to their nonspecific clinical presentation, ALs can mimic other oral lesions, such as benign salivary gland tumors and reactional lesions, as a pyogenic granuloma. We reported a case of an AL located in the gingiva in a 19-year-old female patient. In clinical terms, the lesion was presented as an asymptomatic, oval, pedunculated, well-defined nodule with a smooth surface, fibrous consistency and erythematous color, similar to a pyogenic granuloma. An excisional biopsy was performed and the fragment was sent for histopathological analysis that revealed a vascular proliferation of different calibers, containing thick muscle walls and adjacent muscle proliferation, in addition to an inflammatory infiltrate, predominantly chronic, extravasated red blood cells and an area of ulceration, consistent with the diagnosis of inflamed AL. The histopathology plays an important role in the final diagnosis of rare lesions and with nonspecific clinical characteristics. The surgical excision of the lesion is the most effective treatment of choice for oral ALs. (AU)

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Resumen El angioleiomioma es un tumor benigno perivascular que raramente se localiza en el útero. Se expone el caso de un angioleiomioma de gran tamaño en una mujer de 30 años con sangrado menstrual abundante y masa abdominal palpable. La paciente fue sometida a miomectomía y diagnosticada de angioleiomioma por el estudio histológico. Ante síntomas persistentes, la angiomiomectomía o la histerectomía simple son eficaces.

Abstract Angioleiomyoma is a benign perivascular tumor that is rarely located in the uterus. This paper presents a case of a large angioleiomyoma in a 30-year-old woman with heavy menstrual bleeding and a palpable abdominal mass. The patient underwent myomectomy and was diagnosed with angioleiomyoma by histological examination. For persistent symptoms, angiomyomectomy or simple hysterectomy are effective.

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BACKGROUND: A uterine neoplasm was observed, as an incidental finding, during post-mortem examination of a 26-year-old female multiparous African green monkey (Chlorocebus aethiops sabaeus). The intramural, expansile, 2 to 3 cm well-demarcated, dark-red, nodular neoplasm was located on the anterior uterine body (corpus) wall. METHODS: The mass was examined by light microscopy and by immunohistochemistry. RESULTS: The mass was confirmed as a cavernous uterine angioleiomyoma (syn. vascular leiomyoma) characterized by abundant intratumoural vasculature lined by Factor VIII-positive endothelial cells and surrounded by smooth muscle actin-positive cell proliferations. CONCLUSION: Angioleiomyoma sharing the characteristics of intramural human cavernous uterine angioleiomyoma should be considered in the differential diagnosis of uterine tumours in non-human primates.

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Congenital cutaneous angioleiomyoma is an extremely rare benign smooth muscle tumor. We present a case of a firm, painful subcutaneous mass noticed at birth on the left leg that on surgical excision proved to be an angioleiomyoma. Prognosis is good, and recurrences are uncommon. To our knowledge, this is the second report of a congenital angioleiomyoma.

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Se presenta un caso de dolor de cara anterior de rodilla de causa turnoral de partes blandas en el alerón patelar medial, en un paciente de 50 años. Se realizó biopsia por resección de la turnoración. El estudio anatomo patológico concluye el diagnóstico de angioleiomioma. No se han hallado citas comunicando dicha localización. A pesar de su limitada dimensión, se presumió la etiología al configurarse el síndrome: dolor más masa palpable.(AU)

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Se presenta un caso de dolor de cara anterior de rodilla de causa turnoral de partes blandas en el alerón patelar medial, en un paciente de 50 años. Se realizó biopsia por resección de la turnoración. El estudio anatomo patológico concluye el diagnóstico de angioleiomioma. No se han hallado citas comunicando dicha localización. A pesar de su limitada dimensión, se presumió la etiología al configurarse el síndrome: dolor más masa palpable.

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O Hamartoma Nodal Angiomiomatoso (HNA) é uma doença benigna rara, descrita pela primeira vez em 1992 por Chan et al. Apresenta-se como um tumor vascular primário dos linfonodos que ocorre, na maioria das vezes, exclusivamente nos linfonodos inguinais e femoral, e está ocasionalmente associado a edema de membro inferior ipsilateral. Relata-se o caso de uma paciente de 74 anos, do sexo feminino, com HNA envolvendo linfonodos inguinais direitos; descrevendo seus aspectos patogênicos, clínicos, diagnósticos e terapêuticos. Enfatiza-se a importância do reconhecimento desta patologia para adequado diagnóstico e tratamento. Juntamente com o relato de caso, será realizada breve revisão da literatura (AU)

Angiomyomatous Hamartoma (AH) is a rare benign disease which presents itself as a primary vascular tumor of the lymph nodes, primarily found in the inguinal and femoral lymph nodes and occasionally associated with edema of the ipsilateral limb. Here we report the case of a female patient with AH involving the right inguinal lymph nodes. In addition to the case report, a brief literature review is presented describing the pathogenic, clinical, diagnostic and therapeutic aspects of the condition. The importance of proper recognition of the disease is emphasized for adequate diagnosis and treatment (AU)

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Liomyomas of the nasal cavity and paranasal sinuses are rare. They make up less than 1% of all leiomyomas in the human body. This is due to the paucity of smooth muscle in the nose. They are classified in three groups: leiomyoma, angiomyoma and epithelioid leiomyoma. Only 15 cases of vascular leiomyomas have been found in the literature. The treatment of choice is surgical excision. Hereby we present a new case and review the literature.

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Leiomioma de cavidade nasal e seios paranasais é raro. Ele constitui menos de 1 por cento de todos os leiomiomas do corpo humano. Isto se deve à escassez de células musculares no nariz. Estas neoplasias podem ser classificadas em três grupos: leiomioma, angiomioma e leiomioma epitelióide. Somente 15 casos de angiomioma foram encontrados na literatura. O tratamento de escolha é a excisão cirúrgica. Um novo caso e a revisão da literatura são apresentados.

Liomyomas of the nasal cavity and paranasal sinuses are rare. They make up less than 1 percent of all leiomyomas in the human body. This is due to the paucity of smooth muscle in the nose. They are classified in three groups: leiomyoma, angiomyoma and epithelioid leiomyoma. Only 15 cases of vascular leiomyomas have been found in the literature. The treatment of choice is surgical excision. Hereby we present a new case and review the literature.

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INTRODUÇÃO: Os leiomiomas são neoplasias benignas do músculo liso que ocorrem mais comumente nos tratos geniturinário e gastrintestinal, entretanto são raros na cavidade bucal, na qual provavelmente a maioria desses tumores tem sua origem a partir de músculo liso vascular. OBJETIVOS: Relatar dois casos com história clínica semelhante, confrontando seus aspectos com aqueles encontrados na literatura. RELATO DOS CASOS: Dois casos de mulheres na quinta e sexta décadas de vida apresentando nódulo submucoso em ventre lingual. Após exame microscópico que revelou numerosos vasos sangüíneos entremeados a células fusiformes, com núcleos ovalados ou alongados, e exame imuno-histoquímico com positividade para actina de músculo liso nos dois casos, o diagnóstico final foi leiomioma vascular. CONCLUSÃO: O estudo do leiomioma vascular bucal é de extrema importância devido à raridade e semelhança desse com outras lesões da cavidade bucal. A análise imuno-histoquímica é importante para o diagnóstico final do leiomioma.

INTRODUCTION: The leiomyomas are benign tumors of smooth muscle origin, which are more frequently found in genitourinary and gastrointestinal tracts. However, leiomyomas rarely arise in the oral cavity, and when it happens, the majority of them is originated by smooth muscle of blood vessels. AIMS: Report two cases of oral vascular leiomyoma with similar clinical characteristics, and discuss their aspects with the literature. CASE REPORT: Two cases of vascular leiomyoma in women that presented a nodular mass in the tongue ventral surface were described. The microscopic analysis revealed numerous blood vessels surrounded by spindle cells with ovoid or elongated nuclei. The two cases presented immunopositivity for smooth muscle actin, confirming the diagnosis. CONCLUSION: The study of vascular leiomyoma is required due to its rarity and similar aspects with other oral common lesions. The immunohistochemical analysis is a fundamental tool for the final diagnosis of leiomyoma.

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OBJECTIVE AND IMPORTANCE: Angioleiomyomas (ALMs) are relatively rare, benign, vascular soft tissue tumors that occur most frequently in the extremities of middle-aged individuals. To date, only two cases of intracranial ALMs have been described, both with little emphasis on the clinical, surgical, and radiological aspects. Neither of these reported cases of ALM involved the cavernous sinus. Furthermore, there is no previous intracranial ALM magnetic resonance imaging scan described in the literature. This report presents the first case of cavernous sinus ALM, emphasizing the clinical, radiological, and surgical aspects. CLINICAL PRESENTATION: A 52-year-old man had a 2-year history of horizontal diplopia and frontal headache. Facial numbness and impaired visual acuity in the previous 6 months were also reported. Physical examination revealed paralysis of right Cranial Nerves III, IV, and VI. A decrease in optical acuity was also noted. Computed tomographic and magnetic resonance imaging scans demonstrated a mass lesion located in the right cavernous sinus, which enhanced homogeneously with administration of intravenous contrast medium. INTERVENTION: A total resection was performed via a right frontotemporal craniotomy and a pretemporal approach with peeling of the middle fossa. The postoperative course was uneventful. Histological examination identified the ALM, with no recurrence noted during follow-up. CONCLUSION: It is unknown why intracranial ALMs have not been reported more frequently in the literature. Although ALMs are a rare occurrence, misinterpretation of this lesion may also have contributed to the lack of reported cases. Before surgery, ALMs can be distinguished from meningiomas and schwannomas but not from hemangiomas. The prognosis of intracranial ALM is good, as suggested in this case as well as the two previously reported cases.

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El angioleiomioma es un tumor benigno originado en el músculo liso de la pared de una vena. Es relativamente raro, se observa con mayor frecuencia en mujeres durante la cuarta década de la vida, y se localiza con preferencia en los miembros inferiores. Presentamos 50 casos de esta entidad con las siguientes ubicaciones: 39 (78 por ciento) en las extremidades inferiores, 3 en la cabeza, 2 en el abdomen, 2 en los miembros superiores y 1 en la mama; en 3 casos la localización no se informó. En el estudio histopatológico de la pieza quirúrgica se hallaron lesiones bien delimitadas que contienen gran cantidad de venas de diferente calibre, con pared de espesor variable. Se subdividieron en capilares o sólidos (33 casos), venosos (16 casos) y cavernosos (1 caso). Entre los diagnósticos histológicos debe considerarse el resto de los leiomiomas. Para algunos autores se trata de una malformación hamartomatosa debida a la presencia de células adiposas maduras en el interior del angioleiomioma, un hallazgo que nosotros observamos en sólo uno de nuestros pacientes (AU)

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Cutaneous smooth muscle is present in 3 separate locations: arrector pili muscles, blood vessel walls, and genital/areolar skin. Benign or malignant smooth muscle neoplasms may arise from each of these locations. This review discusses the pathogenesis, clinical manifestations, histologic findings, prognosis, treatment options, and controversial areas of cutaneous smooth muscle neoplasms. ( J Am Acad Dermatol 2002;46:477-90.) Learning objective: At the completion of this learning activity, participants should be able to discuss the pathogenesis, clinical manifestations, histologic findings, prognosis, and treatment options of cutaneous smooth muscle neoplasms.

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A case of angiomyofibroblastoma of the vulva in a 23-year-old woman is presented. Clinical, histological and immunohistochemical features as well as the differential diagnosis and prognosis of this rare but distinct entity are discussed. Only about 65 cases are documented in the literature and to our knowledge this is the first case to be reported from the Caribbean.

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Presentamos el caso de un angioleiomioma en una paciente de 50 años de edad localizado en tobillo izquierdo, de 7 meses de evolución, asociado a miomas uterinos y cáncer de mama. Se realiza una revisión bibliográfica del tema teniendo en cuenta sus aspectos clínicos, histológicos, diagnósticos diferenciales, tratamiento y sus asociaciones (AU)

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