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Giant Superficial Angiomyxoma of the Vulva – A Bizarre Presentation

  • Darshan Hosapatna Basavarajappa
    Affiliations
    Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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  • Shalini Gainder
    Correspondence
    Corresponding author. Dr. Shalini GAINDER, MD, Additional Professor, Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. Phone +91-98155-55732.
    Affiliations
    Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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  • Radhika Srinivasan
    Affiliations
    Department of Cytology and Gynecological Pathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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      A 25-year-old woman with a 2-week history of a huge, rapidly growing, foul-smelling genital growth presented with high-grade fever, dehydration, and features of sepsis. The 70-pound tumour, with skin ulcerations and purulent discharge, was carried over the abdomen. The pedunculated, twisted tumour from the left labia majora depicted in the Figure was multilobulated, the and initial differential diagnosis included myxoid sarcoma or a large condyloma with superadded bacterial infection. No other similar growth in the body was found, and magnetic resonance imaging revealed that the soft tissue lesion was confined only to the vulva with no bony or pelvic extensions. The growth after untwisting was seen to exclusively arise from the left labia majora. Wide local excision was performed, and the microscopic features suggested giant superficial angiomyxoma.
      The clinical presentation was highly suggestive of aggressive angiomyxoma, but this was not confirmed in view of thin interspersed large vessels, spindle-shaped fibroblasts with bland nuclei, and moderate eosinophilic cytoplasm. The tumour cells were positive for desmin and negative for ER, PR, and CD34 markers, strongly suggestive of giant superficial type. The tumour margins were free, and no local recurrence was seen with 24 months of follow-up.

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      • Angiomyxome superficiel géant de la vulve : une présentation inhabituelle
        Journal of Obstetrics and Gynaecology Canada Vol. 44Issue 8
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          Une femme de 25 ans a consulté en raison d’une énorme tumeur génitale nauséabonde et de croissance rapide apparue 2 semaines auparavant, accompagnée d’une forte fièvre, d’une déshydratation et de signes de sepsis. La tumeur de 70 livres, avec ulcérations cutanées et écoulement purulent, s’étendait jusqu’à l’abdomen. La tumeur pédiculée tordue de la grande lèvre gauche présentée dans la figure était multilobulée. Le diagnostic différentiel initial comprenait un sarcome myxoïde ou un condylome volumineux avec infection bactérienne surajoutée.
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