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.
Similar content being viewed by others
References
El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours. 4th ed. Chapter 8. 204–260 edn. IARC, Lyon; 2017.
Gnepp DR, Brandwein-Gensler MS, El-Naggar AK, Nagao T. Carcinoma ex pleomorphic adenoma. In: Barnes EL, Everson JW, El-Naggar AK, editors. WHO classification of tumors, vol. 9, 2005. p. 242–3.
Gnepp DR. Malignant mixed tumors of the salivary glands: a review. Pathol Annu. 1993;28(Pt 1):279–328.
Ellis GL, Auclair PL, Gnepp DR. Surgical pathology of salivary the glands. Philadelphia: Saunders; 1991. p. 350–68.
Redaelli de Zinis LO, Piccioni M, Antonelli AR, Nicolai P. Management and prognostic factors of recurrent pleomorphic adenoma of the parotid gland: personal experience and review of the literature. Eur Arch Otorhinolaryngol. 2008;265:447–52.
Wittekindt C, Streubel K, Arnold G, Stennert E, Guntinas-Lichius O. Recurrent pleomorphic adenoma of the parotid gland: analysis of 108 consecutive patients. Head Neck. 2007;29:822–8.
Barclay DL, Collins CG, Macey HB Jr. Cancer of the bartholin’s gland: a review and report of 8 cases. Obstet Gynecol. 1964;24:329–36.
Crossen RJ. Primary carcinoma of Bartholin’s gland. Am J Surg. 1948;75:597–600.
Ordonez NG, Manning JT, Luma MA. Mixed tumour of the vulva: a report of two cases probably arising in Bartholin’s gland. Cancer. 1981;48:181–6.
Wilson D, Woodger BA. Pleomorphic adenoma of the vulva. J Obstet Gynaecol Br Commonw. 1974;81:1000–2.
Chrome J, Giard R. Case report of an unusual tumor of the vulva; epithelioma of rearranged stroma or so-called mixed tumour. Bull Fed Soc Gynecol Obstet Lang Fr. 1956;16:721–5.
Dykgraaf RH, van Veen MM, van Bekkumde Jonge EE, et al. Pleomorphic adenoma of the vulva: a review illustrated by a clinical case. Int J Gynecol Cancer. 2006;16:920–3.
Rorat E, Wallach RC. Mixed tumors of the vulva: clinical outcome and pathology. Int J Gynecol Pathol. 1984;3(3):323–8.
Su A, Apple SK, Moatamed NA. Pleomorphic adenoma of the vulva, clinical reminder of a rare occurrence. Rare Tumors. 2012;4(1):e16.
Chen MA, Garcia J, Bucci K, Quivey JM, Eisele DW. The role of post operative radiation therapy in carcinoma ex pleomorphic adenoma of the parotid gland. Int J Radiat Oncol Biol Phys. 2007;67(1):138–43.
Lüers J-C, Wittekindt C, Streppel M, Guntinas-Lichius O. Carcinoma ex pleomorphic adenoma of the parotid gland. Study and implications for diagnostics and therapy. Acta Oncol. 2009;48(1):132–6.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
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.
Rights and permissions
About this article
Cite this article
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
Received:
Revised:
Accepted:
Published:
DOI: https://doi.org/10.1007/s40944-018-0209-6