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Bilateral Fibroepithelial Polyps of The Lower Female Genital Tract With Atypical Stromal Cells (Pseudosarcoma Botryoides): A Case Reports Fazlı DEMİRTÜRK1 , Engin ŞENER2, Ahmet Cantuğ ÇALIŞKAN1 , Hakan AYTAN1 Tokat-Turkey A case of female genital tract f ibroepithelial polyps containing atypical stromal cells arising from the v ulva is described.Fibroepithelial stromal polyps of the vulv avaginal lesion are benign lesions that, when bland or hy pocellular, are readily recognized. The tumor was multipl and the highest one measured 4 centimeter in diameter. Cytologic atypia has been a previously recognized f eature in these lesions; howev er fibroepithelial polyps with stromal aty pia are an uncommon tumor of the v ulva, are rarely bilateral and combination of these f eatures has only rarely been documented. These tumors must be diff erentiated f rom sarcoma botry oides, which resemble both grossly and microscopically. (Gynecol Obstet Reprod Med 2006; 12:000-000) Key Words: Fibroepithelial polyps, Atypical stromal cells, Female genital tract Fibroepithelial stromal polyps are benign lesions that occur in a wide variety of sites including the lower female genital tract.Polypoid or grape-like masses of the vagina or cervix are always worrisome because they raise the suspicion of sarcoma botryoides.More recently, larger series showed that reported lesions in the vulva remain an atypical site for these diseases. 1,2 Although they are found at any age, about one third have been reported in pregnancy. They have a gross appearance of soft, translucent pedunculated mucosal lesions. These lesions could contain atypical stromal cells and the presence of such cells should not be mistaken for malignancy.3,4 Both are rare and it is important to distinguish between them. We describe a vulvar example of the condition together with atypical stromal cells. The appearance of an identical tumor on the contralateral labia compounded the diffi culty in both clinical and pathological diagnosis. At operation, the polypoid tumor was attached to the left and right labium majus by a broad base. The lower genital tract was otherwise normal and the inguinal nodes were not enlarged. None of the lesions showed locally aggressive behavior. The tumors were removed by local excision and submitted for pathological assessment. Pathologic Findings Macros copic features: The lesions ranged in size from 1 to 4 cm in m aximum dimension. The fresh specimen was described as polypoid or pedunculated in appearance whi ch had either smooth or fissured surfaces.The cut surface was oedematous, uniform and soft.They were described as multipl cysts that were gelatinous in appearance (Figure 1). Case Report A 18 year-old unmarri ed girl, fi rst present ed in 1994 with polypoid swelling of the right and left labium, which had been presented for 5 years. They were excised and no further treatment instituted.The pathological material submitted for assesment.She returned in 2005 with large polipoid lumps on both sides o f clitoris and lower portion o f labium majus. The clinical examination revealed to be circumscri bed tumor mass of the vulva. 1 Gaziosmanpaşa University Medical Faculty, Obstetrics and Gynecology Department, 2Gaziosmanpaşa University Medicine Faculty,Dermatology Department, Tokat-Turkey Address of Correspondence Ahmet Cantuğ Çalışkan Gaziosmanpaşa University Medicine FacultyObstetrics and Gynecology Department 60100 Tokat-Turkey Submitted for Publication: 11.06.2005 Accepted for Publication: 11.06.2005 62 Figure 1. Fibroepithelial sessile vulvar polyps with fissured surfaces. Microscopic features: The histologic features revealed loose, acellular myxoid stroma, with irregular dilated blood vessels, covered by normal squamous epithelium. On high power we obs erved pleomorphic fibroblast-like cells, with hyperchromatic nuclei and elongated cytoplasmic process es and sparse multinucleated giant cells (Figure 2).The micros- Gynecology Obstetric & Reproductive Medicine 2006; 12:62-63 copic features of the right labial lesions were identical to those of the original left labial tumor. 63 In summary,pseudosarcom a botryoides of the lower female genital tract can exhibit a spectrum of a worrisome morphologic features, with polyps occuring during pregnancy being especially pseudosarcomatous. Cytologic atypia has been a recognized feature in these lesions.Patients with a combination of atypical features may have greater recurrent potential.The treatment of choice is local excision. Recurren ce may occur i f resection is incomplete.3 To ensure completeness of surgical removal, it is urged that pathologists pay special attention to excision margins. References 1. Chirayil SJ, Tobon H.Polyps of the vagina: A clinicopathological study of 18 cases. Cancer 1981; 47:2904-7. Figure 2. High power of stroma with hyperchromatic fibroblast-like cells and multinucleated giant cell. (Hematoxylin and eosin x40). Discussion Pseudosarcoma botyroides of the female genital tract are benign lesions that occur generally in young to middle age women in their reproductive years. 5,6,7 The term is not reserved for embryonal rhabdomyosarcoma, a highly malignant tumour with poor prognosis. The term “ pseudosarcoma botryoides” was applied by Eliott et al. 8 to emphasize its gross and microscopic res emblance to the malignant tumor and the fact that it has been confus ed with latter.They occur most commonly in the vagina-the site of their original descriptionbut they can also occur in the vulva2 and less commonly the cervix. 9 Fibroepithelial stromal polyps can occasionally recur, sometimes more than once.3 The pathogenesis of fibroepithelial polyps is poorly understood.These tumors are composed of an intimate ad mixture of myxoid stroma and vessels reminiscent of the submucosa of the normal uterin cervix, vagina and vulva. 10 The occurence of morphologically similar cells in normal tissue and reactive processes at other anatomic locations such as breast, bladder, nasal polyps and polyps and ulcers of the gastrointestinal tract add additional support to the likely reactive nature of pseudos arcom a botryoides of the lower female genital tract.The stromal cells o f fib roepithelial polyps can express estrogen and progesteron receptors, which suggests that hormonal influences may potentially play a role in the pathogenesis of these lesions. Approximately one-third of the cas es of fibroepithelial polyps occur in pregnant women and another third in those who have t aken sex hormone preparations.It may be that such a hormonal milieu predisposes to their formation. 2. Ostor AG, Fortune DW, Riley CB. Fibroepithelial polyps with atypical stromal cells (pseudosarcoma botryoides) of vulva and vagina. Int J Gynecol Pathol 1988; 7:351-60. 3. Carter J, Elliot P, Russel P. Bilateral fibroepithelial polyp of l abium minus with atypical stromal cells. Pathology 1992; 24:37-9. 4. Halvorsen TB, Johannesen E.Fibroepithelial polyp of vagina: are they old granul ation tissue polyps? J Clin Pathol 1992; 45:235-40. 5. Hartmann C-A, Sperling M, Stein H. So called fibroepithelial polyps of the vagina exhibiting an unusual but uniform antigen profil e characterized by expression of desmin and steroid hormone receptors but no musclespeci fic actin or macrophage markers. Am J Clin Pathol 1990; 93:604-8. 6. Mucitelli DR, Charles EZ, Kraus FT. Vulvavaginal polyps. Histologic appearance, ultrastructure, immunocytochemical characteristics,and clinicopathologic correlation.Int J Gynecol Pathol 1990; 9:20-40. 7. Nucci MR, Fletcher CDM. Fibroepithelial stromal polyps of vulvavaginal tissue. From the banal to bizarre. Pathol Case Rev 1998; 3:151-7. 8. Elliot GB, Reynolds HA, Fidler HK.Pseudosarcoma botryoides of cervix and vagina in pregnancy. J Obstet Gynaecol Br Cwlth 1967; 74:728-33. 9. Cachaza JA, Caballero JJL, Fernandez JA, Salido E. Endocervi cal polyp with ps eudosarcomatous pattern and cytoplasmic inclusions:an electron microscopic study. Am J Clin Pathol 1986; 85:633-5. 10. Al-Nafussi AI, Rebello G, Hughes D, Blessing K. Benign vaginal polyp: a histological, histochemical and immunohistochemical study of 20 polyps with comparison to normal vaginal subepithelial layer. Histopathology 1992; 20:145-50.