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Vol. 69, No. 3, 2010
Issue release date: April 2010
Gynecol Obstet Invest 2010;69:212–216

Vulvar Lymphoedematous Pseudotumours Mistaken for Aggressive Angiomyxoma: Report of Two Cases

D’Antonio A. · Caleo A. · Boscaino A. · Mossetti G. · Iannantuoni N.
aUnit of Pathologic Anatomy, A.U.O. San Giovanni di Dio e Ruggi d’Aragona, Salerno, bUnit of Pathologic Anatomy, Casa di Cura Maria Rosaria, Pompei, cUnit of Pathologic Anatomy, A.O.R.N. Cardarelli, Naples, and dDepartment of Gynecology and Obstetrics, San Giuliano Hospital, Giugliano, Italy

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Background: We describe 2 cases of vulvar pseudotumour due to lymphatic obstruction with chronic lymphoedema of unknown cause that presented as a solitary mass that mimicked aggressive angiomyxoma. Material and Methods: Both patients presented with a vulvar mass without medical history of trauma, surgery in the anogenital region or skin diseases. One patient was overweight (BMI = 26). Both surgically resected vulvar specimens were represented by a polypoid mass with a soft and a gelatinous cut surface. Results: Histologically, the presence of an abundant oedematous stroma with spindle-shaped cells and numerous thin-walled small-to-medium vessels may be confused with an aggressive angiomyxoma. The diagnostic key was represented by the massive oedema, rather than myxoid stroma, with the presence of dilated, tortuous lymphatic channels (some surrounded by clusters of lymphocytes) in the dermis. Conclusion: The recognition of these lesions is important because they may be the cause of problems in differential diagnosis and therapeutic management. In fact, such lesions can be mistaken from both the clinical and histological perspective as a primitive tumour of the vulva-like aggressive angiomyxoma. However, these lesions are not true neoplasms and are likely due to lymphatic obstruction with lymphoedema. A simple surgical excision with vulvoplasty is curative.

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    External Resources

  2. Lu S, Tran TA, Jones DM, Meyer DR, Ross JS, Fisher HA, Carlson JA: Localized lymphedema (elephantiasis): a case series and review of the literature. J Cutan Pathol 2009;36:1–20.
  3. Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindlom JM: Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer 1996;78:79–90.
  4. McCluggage WG, Nielsen GP, Young RH: Massive vulvar edema secondary to obesity and immobilization: a potential mimic of aggressive angiomyxoma. Intern J Gynecol Pathol 2008;27:447–452.
  5. Vang R, Connelly JH, Hammill HA, Shannon RL: Vulvar hypertrophy with lymphedema. Arch Pathol Lab Med 2000;124:1697–1699.

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