Cellular angiofibroma: A rare vulvar tumor case report angiofibroma celular: Um caso raro de tumor vulvar

Abstract Cellular angiofibroma (CA)is a rare benign mesenchymal tumor. In women, it occurs mainly in the vulvovaginal region, with vulvar location in 70% of the cases. Its clinical presentation is nonspecific and similar to several other vulvar tumors of different cellular origins. Thus, its histological and immunohistochemical features allow distinction fromother tumors. Cellular angiofibromas have good prognosis, despite some risk of relapse. The authors present the case of a 49-year-old woman with a bulky right vulvar lesion, for which the preoperative diagnosis was a Bartholin cyst, but the histological and immunohistochemical evaluation yielded a CA.

Resumo O angiofibroma celular é um tumor mesenquimatoso benigno e raro. Nasmulheres, surge principalmente na região vulvo-vaginal, com localização vulvar em 70% dos casos. A sua apresentação clínica é inespecífica e semelhante a vários outros tumores vulvares de diferentes origens celulares. Assim, são as suas características histológicas e imunohistoquímicas que permitem a diferenciação entre eles. Os angiofibromas celulares têm bom prognóstico,embora apresentemalgumrisco de recidiva. Os autores apresentamo caso de umamulher de 49 anos de idade comuma lesão vulvar direita volumosa, cujo diagnóstico pré-operatório era de quisto da glândula de Bartholin, mas cujo exame histológico e imunohistoquímico revelaram tratar-se de um angiofibroma celular.

Angiofibroma celular mesenquimatoso vulvar: reporte de un caso y revisión bibliográfica / Vulvar mesenchymal cell angiofibroma: A case report and literature review

Resumen: ANTECEDENTES: El angiofibroma celular (neoplasia mesenquitamosa) es un tumor poco frecuente conformado por células fusiformes y estructuras vasculares. Los angiofibromas celulares son neoplasias pequeñas, delimitadas, que pueden aparecer en mujeres de 40 a 50 años. CASO CLÍNICO: Paciente de 15 años, sin antecedentes patológicos de importancia para el padecimiento actual, que acudió al servicio médico debido a la aparición de una masa vulvar de crecimiento rápido, de 1 año de evolución. En la exploración física se observó una tumoración vulvar de 10 x 4 cm, en forma de herradura y en el contorno de la vulva se visualizó otra tumoración dependiente de la anterior, en el clítoris, de 7 x 5 cm, que simulaba un falo; ambas estaban adheridas a los planos profundos, sin causar dolor a la palpación, móviles, circunscritas, de consistencia blanda, con crecimiento de vello corporal en posición androgénica. Se llevó a cabo la resección del tumor; el procedimiento se inició con el acceso al labio mayor, sin afectar el meato uretral y el clítoris; se observó aumento de la vascularidad y la tumoración se disecó en su totalidad. El reporte histopatológico fue de angiofibroma celular. La evolución de la paciente fue favorable. CONCLUSIÓN: El angiofibroma celular de la vulva es una neoplasia mesenquimatosa con características clínicas, morfológicas e inmunohistoquímicas propias. El caso aquí expuesto es el primero reportado en una paciente adolescente.

Abstract: BACKGROUND: Cellular angiofibroma is a very rare tumor, it was described as a mesenchymal neoplasm, mainly made up of two components: spindle cells and vascular structures. Cellular angiofibromas are small-delimited malignancies and characteristically occur in women between 40 to 50 years of age. CLINICAL CASE: A 15-year-old female patient, with no pathological history of importance for the current condition, went to the medical unit due to the presence of a 1-year evolution vulvar mass, which was growing rapidly, 10 x 4 cm vulvar mass, horseshoe-shaped bearing the contour of the vulva, another tumor dependent on the previous in the clitoris area (7 x 5 cm), simulating a phallus, fixed to deep planes, not painful on palpation, mobile, circumscribed, of soft consistency, with growth of body hair in an androgenic position. In the surgical event, a tumor resection was performed, beginning an approach by the external part of the majors labia, respecting the urethral meatus and clitoris, an increase in vascularity was observed, dissecting the tumor in its entirety. The histopathological report was of cellular angiofibroma. The evolution of the patient was favorable. CONCLUSION: The cellular angiofibroma of the vulva is a mesenchymal neoplasm with the own clinical, morphological and immunohistochemical characteristics. The case presented is the first identity developed in an adolescent patient.

Angiomiofibroblastoma-like, un tumor excepcional: presentación de un caso clínico y diagnóstico diferencial / Angiomyofibroblastoma-like, an exceptional tumor: presentation of a clinical case and differential diagnosis

Presentamos un caso de Angiomiofibroblastoma-like (AML), en el que la ecografía fue importante para determinar la detección, localización y extensión local. Hallazgos: La ecografía demostró una lesión paratesticular sólida hipoecogénica de bordes bien definidos lo que sugirió lesión benigna extratesticular. La histología evidenció una neoplasia mesenquimal benigna tipo mixoide en cuyo diagnóstico diferencial se incluyen el Angiomixoma superficial (AMS), Angiomixoma agresivo (AMA), Angiomiofibroblastoma (AMF) y el AML. En base a estos hallazgos se realizó una revisión de tumores de similares características en la literatura, llegando finalmente al diagnóstico de AML cuya localización paratesticular sólo ha sido descrita en muy pocos casos hasta la fecha. Conclusión: La ecografía es útil para localizar los tumores paratesticulares y determinar su extensión local. No obstante, para llegar al diagnóstico definitivo es necesario realizar un estudio histológico e inmunohistoquímico de la tumoración.

We present a case of a paratesticular Angiomiofibroblastoma-like (AML) tumor in which ultrasound was important to give the specific location and local extension of the lesion. Findings: Ultrasound revealed a hypoechoic paratesticular lesion with well-defined borders, suggesting an extratesticular benign lesion. The histology showed a benign mesenchymal myxoid-type neoplasm. The differential diagnosis included superficial Angiomyxoma (AMS), Aggressive angiomyxoma (AMA), Angiomyofibroblastoma (AMF) and AML. Based on these findings, a review of similar tumors was carried out and ultimately led to the diagnosis of paratesticular AML. This location has been described only in a few cases in the literature. Conclusion: Ultrasound is useful to locate paratesticular tumors and determine their local extension. However, a definitive diagnosis still requires a histological and immunohistochemical study.

Cellular Angiofibroma of the Perianal Space: MR Imaging and Pathologic Correlation

A variety of benign and malignant tumor can occur in the perianal space. Among them, cellular angiofibroma is a rare tumor that belongs to fibroblastic/myofibroblastic tumors of the WHO classification, sometimes called as "angiomyofibroblastoma-like tumor". Cellular angiofibroma can be found at various anatomic sites such as the vulva, perineum, genital tract, and inguinal regions with approximately equal gender ratio. This tumor is usually a well-circumscribed mass showing isosignal intensity to muscle on T1-weighted images. On T2-weighted images, cellular angiofibroma may show as a mass with inhomogeneous signal intensity depending on amount of composed spindle cell, collagenous stroma, myxoid matrix, and fat tissue; however it is characterized by low signal intensity due to fibrous tissues. This highly vascular mass shows strong enhancement on post contrast images. Recommended treatment is simple local excision and no recurrence or metastasis have been reported up to date.