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Malignant Transformation of Pleomorphic Adenoma of Vulva: Report of a Rare Case and Review of Literature

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Abstract

Introduction

Pleomorphic adenomas are mixed tumors with epithelial and stromal elements and commonly arise in the salivary glands, particularly in the parotid. Pleomorphic adenomas of the vulva are very rare, and less than ten cases have been reported in the literature. We report a rare case of carcinoma expleomorphic adenoma, which we believe is the second such reported case in the world.

Case report

A 65-year-old postmenopausal woman presented with vulval mass arising from mons pubis involving the left labia. Preoperative biopsy showed features of adenocarcinoma. She underwent a radical vulvectomy with bilateral groin node dissection. Histology revealed a low-grade epithelial–myoepithelial carcinoma expleomorphic adenoma. No adjuvant therapy was given. Patient is disease free at 6-months of follow-up.

Discussion

Carcinoma expleomorphic adenoma is defined as a pleomorphic adenoma in which an epithelial malignancy is derived. It commonly arises in the salivary glands, mainly the parotid. The most important factor predisposing to recurrence is positive surgical margins. Hence treatment is wide excision aiming at negative margins to avoid both the risk of recurrence and malignant transformation. Available evidence from the treatment of salivary tumors suggests that adjuvant radiotherapy may improve local control in high-risk cases.

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Correspondence to Anbukkani Subbian.

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The authors declare that they have no conflict of interest.

Ethical Approval

All procedures performed in this patient were in accordance with the ethical standards of the institutional research committee and were done after multidisciplinary tumor board discussion and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from the patient included in the case report.

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Subbian, A., Uthamalingam, P., Bag, M. et al. Malignant Transformation of Pleomorphic Adenoma of Vulva: Report of a Rare Case and Review of Literature. Indian J Gynecol Oncolog 16, 35 (2018). https://doi.org/10.1007/s40944-018-0209-6

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  • DOI: https://doi.org/10.1007/s40944-018-0209-6

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