Aggressive angiomyxoma of the female pelvis and perineum. Report of two cases and review of the literature

Simó, Manuel Romero; Zapata, Carlos; Esquius, J.; Domingo, J. Puig

doi:10.1111/j.1471-0528.1992.tb14446.x

Cited by 42 publications

(19 citation statements)

References 7 publications

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“…Dentro de los tumores malignos se debe imponer diagnóstico diferencial con tumores tales como liposarcoma mixoide, histiocitoma fibroso maligno mixoide y rabdomiosarcoma embrionario 3, 16,18. El tratamiento universalmente aceptado es la cirugía. En 1992 Simo et al concluyen que "una exéresis quirúrgica alargada basada en un correcto diagnóstico histopatológico es la base del tratamiento curativo" 20 . Aunque múltiples autores defiendan exéresis ampliadas, no siempre es factible realizar este tipo de gestos sin inherente morbilidad major asociada, dada la proximidad de estructuras genitourinarias y ano-réctales.…”

Section: Discussionunclassified

Angiomixoma agresivo penoescrotal

Xambre

Cerqueira

Guimarães³

et al. 2009

Actas Urológicas Españolas

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E l angiomixoma agresivo designa una neoplasia mesenquimatosa extremadamente rara, perfectamente individualizada en lo que respecta a crité-rios de diagnóstico histológico. Se caracteriza por un comportamiento localmente infiltrativo, alcanzando habitualmente grandes dimensiones, aunque muy raramente metastize. Hasta el momento sólo hay descritas escasas decenas de casos en el sexo masculino, con afectación casi exclusiva de los genitales externos o cordones espermáticos.Los autores presentan un caso adicional de este tumor, exuberante, en un paciente joven de sexo masculino. En el tratamiento fueron usados procedimientos reconstructivos haciendo uso de colgajos y el uso de injertos, antes nunca descritos en el tratamiento de estas situaciones. CASO CLÍNICOPaciente de sexo masculino, 18 años de edad, presentando antecedentes de atraso del desarrollo psico-motor, trombocitopenia auto-inmune, neumonía linfocítica granulomatosa difusa, síndrome anti-fosfolipídeo, esplenomegalia, obesidad central, soriasis inversa y psicosis. Medicado con alendronato de sodio, haloperidol, lorazepam, trihexifenidilo, omeprazol, carbonato de calcio y hierro oral. Enviado a la consulta de urología por aumento del volumen progresivo de los genitales externos. Refería tumefacción generalizada y difusa de la piel escrotal, condicionando dificultad miccional, de la marcha e higiene deficiente. Negaba antecedentes quirúrgicos genitales, inguinales o pélvicos. Sin antecedentes de radioterapia inguinal o pélvi- Nota clínica Angiomixoma agresivo penoescrotalLuís Xambre*, Manuel Cerqueira**, Isabel Guimaraes***, Fernando Carreira**, Teresa Galán**** ResumenAngiomixoma agresivo designa una neoplasia mesenquimatosa extremadamente rara. Afecta casi exclusivamente estructuras genitales, pélvicas o perineales de pacientes de sexo femenino, siendo muy raros los casos que envuelven el sexo masculino. Se trata de neoformaciones bien caracterizadas desde el punto de vista histológico y de comportamiento benigno.Los autores presentan un caso clínico de un tumor de este tipo en un adulto joven de sexo masculino con envolvimiento penoescrotal de grandes dimensiones implicando exéresis y reconstrucción compleja, con recurso a colgajos e injertos cutáneos.Es igualmente realizada una revisión bibliográfica exhaustiva sobre el tema englobando aspectos etiopatogénicos, clí-nicos, de imagen, anatomo-patológicos y de diagnóstico diferencial, así como terapéuticos y de pronóstico.Palabras clave: Angiomixoma agresivo. Escroto. Tumor mesenquimatoso. Penoscrotal agressive angiomyxoma AbstractAggressive angiomyxoma denotes an extremely infrequent mesenchymal tumour. In virtually every case it involves genital, pelvic or perineal female structures. Cases involving male patients are extremely rare. It is a distinctive tumour with a characteristic clinical course and specific and well characterized microscopic features.The authors report an additional clinical case in a young male patient with massive scrotal and penile involvement, necessitating exeresis followed by c...

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Section: Discussionunclassified

Angiomixoma agresivo penoescrotal

Xambre

Cerqueira

Guimarães³

et al. 2009

Actas Urológicas Españolas

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“…[1][2][3][4] The diagnosis is based on histopathological and immunohistochemical studies. In this case, the finger-like projections into surrounding tissues was not seen, [1][2][3][4][5][6][7][8] but the low cellularity in the myxoid stroma with numerous vessels of varying wall thickness along with the spindle-shaped and stellate-shaped cells with regular nuclei and absence of mitotic figures, added to the Immunohistochemical features, are all typical for AAM. [1][2][3][4][5][6][7][8] The documented studies for immunohistochemical data in two series showed nearly 97% positive response to desmin, 94% for smooth muscle actin, 93 and 90% for oestrogen and progesterone receptors, respectively, but 0% for S100 protein.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Its radiological features have been described, being a displacing mass rather than infiltrating, with high signal intensity on T2-weighted images, 1,14,15 but these features can be attributed to the myxoid component of the tumour, shown by many other soft tissue tumours of mesenchymal origin. On gross examination, it was different in being well circumscribed non-lobulated and encapsulated, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] but the gelatinous appearance, greyish discoloration on cross section and the large size of the tumour are common features of AAMs. These findings are attributed to the myxoid stroma that is rich in collagen fibres.…”

Section: Discussionmentioning

confidence: 99%

“…4,18 Complete excision is the treatment of choice. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] We believe that 1-2 yearly intervals of follow-up with alternate MRI and sonography is sufficient in view of the slow growing and nonmetastasising nature of this tumour.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5][6][7][8] It affects women 7 times as often as men. 9,10 It was also reported in children as young as 11 years old of both sexes.…”

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confidence: 99%

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A solitary encapsulated pelvic aggressive angiomyxoma

Adwan

Patel²,

Kamel³

et al. 2004

Ann R Coll Surg Engl

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Aggressive angiomyxoma (AAM) was first reported in 1983 as a distinct, slow growing, benign but locally infiltrative, soft tissue tumour. It usually arises in the pelvic and perineal organs, mostly in women. A 47-year-old woman was found to have a large encapsulated retroperitoneal aggressive angiomyxoma. The mass was completely excised via abdomino-perineal approach, and no recurrence noted on MRI at 19 months' follow-up. The encapsulation of this tumour together with other reported rare presentations, suggest an isolated mesenchymal cell origin. A review of the literature is provided.

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