A Rare Case of Vulvar Superficial Angiomyxoma in a Pediatric Patient - 04/12/20
, Nahir Cortes-Santiago, MD, PhD 2, Ninad Mohan Patil, MD 2, Jennifer Lyrah Bercaw-Pratt, MD 1Abstract |
Background |
Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor. Originally described as a component of Carney complex, it is now recognized as a sporadic condition.
Case |
A 7-year-old girl was referred for management of a 2.5-cm mass arising from the right labia majora. Key pathologic features included lobules of spindle-shaped cells in a myxoid matrix and prominent neutrophilic infiltrate. The cells were positive for CD34 and negative for desmin, progesterone receptor, and estrogen receptor staining.
Summary and Conclusion |
This case is that of the youngest described patient with vulvar SAM. Patients should be carefully examined for manifestations of Carney complex to avoid potentially life-threatening complications. It is critical to distinguish SAM from aggressive angiomyxoma. The patient was referred to genetics and will be followed for local recurrence.
Le texte complet de cet article est disponible en PDF.Key Words : Superficial angiomyxoma, Myxoma, Carney complex, Vulvar neoplasms, Pediatric gynecology
Plan
| This work was presented as a poster presentation at the North American Society for Pediatric and Adolescent Gynecology (NASPAG) Annual Meeting, New Orleans, LA, April 11, 2019. |
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| There were no sources of funding for this work. |
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| The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article. |
Vol 33 - N° 6
P. 727-729 - décembre 2020 Retour au numéro
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