Journal of Obstetrics and Gynaecology, August 2008; 28(6): 563566

Leiomyoma and rhabdomyoma of the vagina Vaginal myoma

A. IMAI1, T. FURUI1, Y. HATANO1, M. SUZUKI1, N. SUZUKI1 & S. GOSHIMA2

Departments of 1Obstetrics and Gynecology and 2Radiology, Gifu University School of Medicine, Yanagido, Gifu, Japan

Summary Vaginal bromyomas (leiomyomas and rhabdomyomas) are rare; approximately 300 cases have been reported in the literature. They usually present as a mass per vaginum or dyspareunia or pressure symptoms on the urinary tract. However, they sometimes have an unusual presentation that is largely responsible for the relative difculty in preoperative diagnosis. Preoperative imaging and careful examination may help to rule out malignancy. Recurrence occurs infrequently but the practical approach entails immediate careful excision. Surgical excision through the vaginal route has been the traditional approach, but abdominoperineal route may be necessary for huge tumours. Keywords Genital bleeding, rhabdomyoma, vaginal leiomyoma, vulval tumour

Introduction
Fibromyomas are the most common tumours in the uterus. They can be in various anatomical locations and in various sizes. The vagina is a rare site, and in a survey of solid tumour occurring in the vagina, 4.5% are bromyomas (Liu 1988; Sangwan et al. 1996). Less than 300 cases have been reported in the world literature (Cobanoglu et al. 1996). They have varied clinical presentation, the most common being vaginal mass. An unusual presentation may create a clinical dilemma in diagnosis (Gowri et al. 2003). Fibromyomas of the vagina may be confused with a variety of benign or malignant vaginal tumours, because or their infrequency (Liu 1988; Cobanoglu et al. 1996; Sangwan et al. 1996; Gowri et al. 2003). Thereby vaginal bromyoma should always be considered in the differential diagnosis of vaginal tumours.

General considerations
The estimated number of the leiomyomas will probably not exceed 250 cases (Liu 1988). Leiomyoma of the vagina occurs most frequently between the ages of 3848 years and predominates in White Women, whereas leiomyoma of the uterus predominates in Black women (Sangwan et al. 1996). Less than 30 cases of rhabdomyoma of the vagina have previously been reported (Suarez Vilela et al. 1990; Lopez et al. 1994).

Symptom
Their size varies from 0.5 to 15 cm; the majority of these tumours measure approximately 34 cm in diameter (Sangwan et al. 1996). The largest vaginal wall leiomyoma

recorded measured 20 cm in diameter (Moghissi 1960). These tumours are usually localised and vary from solid to cystic in consistency (Liu 1988). They are usually rm and can undergo degenerative changes and feel soft. They manifest in adult women by the appearance of a mass that can produce signs of compression, dyspareunia, and urinary difculties. Fibromyomas have the characteristics of slow growth and small size. Therefore, it does not produce symptoms at the onset, and cannot make themselves evident in a large number of patients. Patients may primarily have vaginal mass-related symptoms of urinary bladder outlet obstruction (Nel and Tiltman 1978; Njeh et al. 1993; Ruggieri et al. 1996; Sangwan et al. 1996). Shaw (Shaw 1989) reported a case of sub-urethral myoma who presented with pelvic pain and had cystitis cystica. Presentation is usually at about 40 years of age and may be delayed because the tumour is slow-growing and the vagina very distensible. The initial lack of symptoms is due to the elasticity of the vagina. Tumours with slow growth and of small size may not produce any symptoms and regress after menopause. After attaining a certain size (46 cm in diameter) and according to their location and weight, vaginal bromyoma can cause a dragging down sensation, urinary symptoms, constipation, dyspareunia, leucorrhoea and protrusion of the mass from the vagina. Ulceration of the overlying mucosa with subsequent necrosis can produce purulent discharge. In a number of reported cases, ulceration of the overlying epithelium has been noted with subsequent necrosis, purulent discharge and bleeding (Sangwan et al. 1996; Sesti et al. 2004). A huge bromyoma occupying both the ischiorectal fossae has been reported in a pregnant patient (Sadan et al. 1987). She had presented with a vaginal mass and the tumour was found to be adherent to the

Correspondence: A. Imai, Department of Obstetrics and Gynecology, Gifu University School of Medicine, Yanagido, Gifu 5011194, Japan. E-mail: atsushi@gifu-u.ac.jp ISSN 0144-3615 print/ISSN 1364-6893 online 2008 Informa Healthcare USA, Inc. DOI: 10.1080/01443610802310333

564

A. Imai et al.
The possibility that a leiomyoma may actually be a smooth muscle tumour of uncertain malignant potential or a leiomyosarcoma often dictates the clinical management or rapid growing broids (Schwartz and Kelly 2006). Based on the degree of cytological atypia, mitotic activity, and other features, uterine smooth muscle tumours have historically been grouped into two classes: benign leiomyomas and malignant leiomyosarcomas (Berretta et al. 2007). However, this separation holds true more in principle than in practice because the tumours biological potential may not always be determined with certainty, complicating diagnosis, and therapy. The clinician must be aware that the infrequent occurrence of extrauterine leiomyosarcomas makes it difcult to establish absolutely rm recommendations for the diagnosis and management of this disease, particularly with regard to fertility preservation. A malignant tumour, such as aggressive angiomyxoma, Gartner duct cyst, endometriosis, cystocele or uterine prolapse must be considered in the differential diagnosis. Fibromyomas reveal an ellipse of semisolid, smoothsurfaced mass occupying the vaginal cavity. However, aggressive angiomyxoma are most often not capsulated, a non-rubbery mass, and a non-ulcerative surface (Gungor et al. 2004). On CT, the malignant tumours such as aggressive angiomyxoma are hypodense relative to the muscle (Wiser et al. 2006). On T2-weighted MRI, it has high signal intensities. These appearances likely relate to the loose myxoid matrix and high water content of angiomyxoma. A distinctive appearance of swirled strands of lower density on CT and lower signal intensity on MRI within the tumour have also been described (Wiser et al. 2006). CT and MRI can accurately reveal extension of the tumour from the vagina into the perineum and the pelvis (Wiser et al. 2006) (Figure 1). When the mass is not well dened or has atypical behaviour, it seems to us that preoperative MRI might raise the suspicion for tumour, improve preparation before surgery, and direct the surgeon toward complete excision. Extracardiac rhabdomyomas are very infrequent tumours. Lopez et al. (1992, 1994) present a case of a pedunculated polyp originated from the anterior wall of the vagina. They concluded that vaginal rhabdomyoma should be included in the differential diagnosis of vaginal polyps in which atypical

rectum at the time of operation. Gowri et al. (2003) report a case of myoma from the lateral vaginal wall that presented as a gluteal swelling with pus discharging per vagina. In genital bleeding during childhood, two sources of bleeding should be suspected: a local vulval or vaginal lesion and the endometrium as a manifestation of precocious puberty (Imai 1998; Imai et al. 1994). Sarcoma botryoides and endodermal sinus tumour of the vagina occur in children below the age of 10. In early childhood, patients with such malignant tumours in the vagina develop bright red spotting on their nappies only. Thus, careful examination may be the mainstay of evaluation to exclude the presence of the tumours.

Diagnosis
They are usually located in the anterior wall (Gowri et al. 2003) and rarely in the lateral wall (Kaufman and Gardner 1965) and fornix (Figure 1). The tumour is covered with an ellipse of mucosa on the surface. Fibromyomas of the vagina are usually rm but since they may undergo the same degenerative changes as those of the uterus, i.e. hyalinisation, calcication, liquefaction, necrosis, cystic degeneration, etc. they may vary in consistency from rm to soft (Sangwan et al. 1996). Oruc et al. (2004) report an interesting case of prolapsed cervical myoma with endometrium on the surface. In their case, a prolapsed, pedunculated cervical myoma was detected along with pregnancy complications, pre-term premature rupture of membrane and fetal death. The cause-and-effect relationship between the prolapsed myoma and membrane rupture is unknown. Vaginal bromyomas may be confused with a variety of benign vaginal tumours. A preoperative diagnosis is seldom made. To discriminate between benign and malignant tumour and to assess surrounding structures, ultrasound, biopsy and CT scans have been recommended (Bolt and Schutter 1998). Leiomyoma with variable cell size and shape, hyperchromatic and multilobulated nuclei but without marginal inltration or mitotic activity has been called bizarre leiomyoma. Only one such case has been reported so far (Biankin et al. 2000). The benign behaviour of these bizarre leiomyoma has been rmly established (Downes and Hart 1997).

Figure 1. A case of vaginal myoma. A 47-year-old generally healthy woman presented with a mans st sized, well-circumscribed mass from her vagina. Clinical examination revealed an ellipse of semi-solid, smooth-surfaced mass occupying the vaginal cavity. Sagittal T2-weighted MRI (a) showed 9 6 5 6 11 cm pedunculated mass from the posterior vaginal fornix. Feeder vessels are detected within the stalk (transverse T2-weighted MRI (b)). The resected tumour measured 10 6 6 6 10 cm with an ellipse of mucosa on the surface, and muscle-like, white, elastic and homogenous appearance was observed on cut section (c). On pathological evaluation, it was reported as myoma not associated with uterine leiomyomas.

Vaginal myoma
multinucleated stromal cells, somewhat resembling rhabdomyoblasts, are occasionally seen (Abdul-Karim and Cohen 1990; Suarez Vilela et al. 1990). Their light and ultrastructural characteristics have already been reported (Lopez et al. 1994). Immunohistochemistry has been also applied both in vaginal location (Suarez Vilela et al. 1990) and in the soft tissues (Eusebi et al. 1988). Malignant tumours seen in very young girls (53 years old) are often not palpable per rectum and are frequently not detected in cytology. The failure of early detection may carry a dismal prognosis, demanding a prompt and precise location and extent of the lesion. Thus, careful examination with MRI may be the mainstay of evaluation to exclude the presence of the tumours (Imai 1998; Imai et al. 1994).

565

Treatment
The practical approach to such a vaginal mass entails immediate careful excision. Surgery through the vaginal route has been reported as the treatment of choice for these tumours (Gowri et al. 2003). Huge tumours may require combined abdominal and perineal approach and may necessitate hysterectomy. Although morcellation of the tumour might render the surgery through the vaginal route easier, recurrence of the tumour is of concern (Gowri et al. 2003). Dhaliwal et al. (1992) have specically recommended removal of the tumour en bloc to avoid any possible recurrence. Vaginal leiomyoma, especially the borderline variety may undergo sarcomatous change (Miyakawa et al. 1985; Cobanoglu et al. 1996). Liu (1988) reported an incidence of 9.1% for malignant change in his series. Hence these tumours should be operated as soon as they are recognised. To reserve fertility, vaginal myomectomy is recommended as the initial treatment of choice for prolapsed, pedunculated myomas, except when other indications necessitate as abdominal approach (Phelan 1995). Surgical resection of myomas should be reserved for pregnant women with symptomatic, pedunculated myomas with a small stalk and for those with a pregnancy complication from myoma. Resection should be considered prior to the next pregnancy (Oruc et al. 2004). At present, no nal consensus has been reached on the choice of the best strategy for surgery and adjuvant therapy.

Conclusion
Despite their rarity, vaginal bromyomas should be included in the differential diagnosis of vaginal masses. Recurrence is rare but the practical approach entails immediate careful abdomenoperineal approach and/or hysterectomy for better surgical access. Declaration of interest: The authors report no conicts of interest. The authors alone are responsible for the content and writing of the paper.

References
Abdul-Karim F, Cohen R. 1990. Atypical stromal cells of lower female genital tract. Histopathology 17:249253. Berretta R, Rolla M, Merisio C, Giordano G, Nardelli G. 2007. Uterine smooth muscle tumor of uncertain malignant potential: a three-case report. International Journal of Gynecological Cancer 6:563569.

Biankin S, OToole V, Fung C, Russell P. 2000. Bizarre leiomyoma of the vagina: report of a case. International Journal of Gynecological Pathology 19:186187. Bolt J, Schutter E. 1998. A tumor in the paracolpium. A case report. European Journal of Obstetrics, Gynecology and Reproductive Biology 76:233238. Cobanoglu O, Gurkan Zorlu C, Ergun Y, Kutluay L. 1996. Leiomyosarcoma of the vagina. European Journal of Obstetrics, Gynecology and Reproductive Biology 70:205207. Dhaliwal L, Das I, Gopalan S. 1992. Recurrent leiomyoma of the vagina. International Journal of Gynaecology and Obstetrics 37:281283. Downes K, Hart W. 1997. Bizarre leiomyomas of the uterus: a comprehensive pathologic study of 24 cases with long-term followup. American Journal of Surgical Pathology 21:12611270. Eusebi V, Ceccarelli C, Daniele E, Collina G, Viale G, Mancini A. 1988. Extracardiac rhabdomyoma: an immunocytochemical study and review of the literature. Applied Pathology 6:197207. Gowri R, Soundararaghavan S, Oumachigui A, Sistla S, Iyengar K. 2003. Leiomyoma of the vagina: an unusual presentation. Journal of Obstetric and Gynaecological Research 29:395398. Gungor T, Zengeroglu S, Kaleli A, Kuzey G. 2004. Aggressive angiomyxoma of the vulva and vagina. A common problem: misdiagnosis. European Journal of Obstetrics, Gynecology and Reproductive Biology 112:114116. Imai A. 1998. Genital tumours in girls and place of surgery. In: Dramusic A, Ratnam SS, editors. Clinical approach to paediatric and adolescent gynaecology. Singapore: Oxford University Press. pp 203212. Imai A, Furui T, Tamaya T. 1994. Gynecologic tumors and symptoms in childhood and adolescence; 10-years experience. International Journal of Gynaecology and Obstetrics 45:227234. Kaufman R, Gardner H. 1965. Benign mesodermal tumors. Clinical Obstetrics and Gynecology 8:978. Liu M. 1988. Fibromyoma of the vagina. European Journal of Obstetrics, Gynecology and Reproductive Biology 29:321328. Lopez J, Brouard I, Eizaguirre B. 1992. Rhabdomyoma of the vagina. European Journal of Obstetrics, Gynecology and Reproductive Biology 45:147148. Lopez V, Lopez M, La Cruz P. 1994. Vaginal rhabdomyomas. International Journal of Gynaecology and Obstetrics 47:169170. Miyakawa I, Yasuda H, Taniyama K, Mori N, Uehara Y, Sumiyoshi A. 1985. Leiomyosarcoma of the vagina. International Journal of Gynaecology and Obstetrics 23:213216. Moghissi K. 1960. Myoma of the vagina: report of a case and review of literature. Obstetrics and Gynecology 15:235236. Nel C, Tiltman A. 1978. Leiomyoma of the vagina. South African Medical Journal 54:816817. Njeh M, Barkia A, Jemni M, Zermani R, Ben Milad K, Ayed M. 1993. Vaginal leiomyoma; the female prostate. Acta Urologica Belgica 61:3132. Oruc S, Karaer O, Kurtul O. 2004. Coexistence of a prolapsed, pedunculated cervical myoma and pregnancy complications: a case report. Journal of Reproductive Medicine 49:575577. Phelan J. 1995. Myomas and pregnancy. Obstetric and Gynecologic Clinics of North America 22:801805. Ruggieri A, Brody J, Curhan R. 1996. Vaginal leiomyoma. A case report with imaging ndings. Journal of Reproductive Medicine 41:875877. Sadan O, Kruger S, van Iddekinge B. 1987. Vaginal tumors in pregnancy. Case report and review of the literature. Acta Obstetricia et Gynecologica Scandinavica 66:559562. Sangwan K, Khosla A, Hazra P. 1996. Leiomyoma of the vagina. Australia and New Zealand Journal of Obstetrics and Gynaecology 36:494495. Schwartz P, Kelly M. 2006. Malignant transformation of myomas: myth or reality? Obstetrics and Gynecology Clinics of North America 33:183198. Sesti F, La Marca L, Pietropolli A, Piccione E. 2004. Multiple leiomyomas of the vagina in a premenopausal woman. Archives of Gynecology and Obstetrics 270:131132.

566

A. Imai et al.
Wiser A, Korach J, Gotlieb W, Fridman E, Apter S, BenBaruch G. 2006. Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: report of three cases and review of the literature. Abdominal Imaging 31:383386.

Shaw C. 1989. Vaginal leiomyoma as a cause of pelvic pain and cystitis cystica. Journal of the American Osteopath Association 89:13301331. Suarez Vilela D, Gimenez Pizarro A, Rio Suarez M. 1990. Vaginal rhabdomyoma and adenosis. Histopathology 16:393394.