Giant angiomyofibroblastoma of the vulva.

Ugo Indraccolo a, MD, PhD; Enzo De Sanctis b, MD; Fabrizio Liberati c, MD; Mario Ippolito b, MD; Salvatore Renato Indraccolo d, MD, Prof. 

a Department of Surgical Sciences, Institution of Obstetrics and Gynecology, University of Foggia.

b Division of Surgery, Hospital of Dubbo.

c Division of Pathology, Hospital of Dubbo.

d Department of Gynecology, Perinatology and Child welfare, II Faculty of Medicine and Surgery, “La Sapienza” University of Rome.

 Correspnding author:Dr. Ugo Indraccolo, MD, PhD. Loc. Montagnano 16 62032 Camerino (MC) – Italy. Tel. 39 328 6180677 Fax 39 0737 636668 

e-mail: ugo.indraccolo@libero.it

  

Abstract.

Background. Angiomyofibroblastoma is a rare tumour which generally arises in women in fertile age upon the cutis of the perineal or vulvar region and usually concerning a size of just a few cm in diameter. Case. In this case report, it is described the unusual occurrence of a giant angiomyofibroblastoma of the vulva (weighing 4,800 g) which has prevent the patient for walking. Conclusion. We should reckon that angiomyofibroblastoma may grow up to a very large size. 

Key words: angiomyofibroblastoma, vulvar mass, diagnosis, surgical treatment, pathologic features.

 

Background.

Angiomyofibroblastoma is a rare usually benign tumor which generally arises in women in fertile age upon the cutis of the perineal or vulvar region and usually concerning a size of just a few cm in diameter (1). Its texture is rubbery or smooth and looks oedematous when cut. As a rule, it shows spindle-shaped cells with production of collagen fibres and alternates hypercellular areas, particularly around the vessels, and hypocellular areas. Sometimes we can find mature adipocytes in the tumoral tissue (1). It would originate from an immature multipotent cell of the connective tissue, situated around the vessels and being capable of differentiating into similar tumoral varieties, with which the giant angiomyofibroblastoma enters into differential diagnosis (1,2). In this short report, we present the unusual clinical case of a giant angiomyofibroblastoma which has required a wide surgical excision.

 

Case.

The 40-year-old patient went to the hospital of Dubbo (Wolayta, Ethiopia), reporting impossibility to walk and vulvar pain in the standing position due to the appearance and growth of a formation starting from the right labium major. It was not possible to understand through the case-history within what lapse of time such neoformation had been growing. During the gynaecological examination we detected a big dark brown coloured mass over 25 cm wide in main diameter with a hard texture and irregular contour, painless to palpation and without any evidence of ulcerated areas. Such neoformation originated from the right labium major, to which it was connected by a large and oedematous peduncle. Within the normal range the other vulvar and perineal structures as well as the inner genitalia. The patient was submitted to right hemivulvectomy. She was discharged three days after the surgery in good health conditions. It was not possible to perform any follow-up. 

At the macroscopic examination (Figure 1), the mass removed weighed 4,800 g and was hard when cut, compact and showing a whitish surface. At the microscopic examination, the tumour appeared well-circumscribed and constituted by cells ranging from spindle- to oval-shaped, without cytological atypias, with sizeable areas rich in collagen and numerous capillary-like vessels. The cells showed a bland nucleus and did not contain any mitosis (Figure 2). Inside the lesions there were some mature adipocytes. The above-mentioned features match with the diagnosis of angiomyofibroblastoma, as reported by  McCluggage (1).  

 

Figure 1. The excised and cut giant mass.

 

Discussion.

Angiomyofibroblastoma is a usually benign lesion which does not exceed 12 cm in diameter (1). However, recently a case of vulvar angiomyofibroblastoma 13 cm wide in main diameter (3) has been reported as well. It appears as a pedunculate mass originating from one of the labia majora, whose growth times have not been well determined and can vary from months up to years (4). It is often confused with other benign and more usual pathologies of the vulva, such as the Bartholin’s gland cysts or lipomata (4,5), although it must be suspected in case a pedunculate mass originating from a labium major becomes objective (4). As far as we are concerned, so far no possibility has ever been reported for an angiomyofibroblastoma to grow up to over 13 cm in main diameter, probably because it is always excised before. In this case, due to cultural difficulties present in this region of Africa, the patient went to the hospital only when the situation had already become extreme. Therefore, we should reckon that angiomyofibroblastoma may grow, when not treated, up to such a volume as to prevent the patient from walking.

 

References.

  1. McCluggage WG. A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol. 2005; 24: 26-38.

  2. Alameda F, Munne A, Baro T, Iglesias M, Condom E, Lloreta-Trull J, et al. Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study. Ultrastruct Pathol. 2006; 30: 193-205.

  3. Hsu IH, Chang TC, Wu CT, Chen RJ, Chow SN. Angiomyofibroblastoma of the vulva. J Formos Med Assoc. 2004; 103: 467-471. 

  4. Omori M, Toyoda H, Hirai T, Ogino T, Okada S. Angiomyofibroblastoma of the vulva: a large pedunculated mass formation. Acta Med Okayama. 2006; 60: 237-242. 

  5. Wang J, Sheng W, Tu X, Shi D, Zhu X, Zhang R. Clinicopathologic analysis of angiomyofibroblastoma of the female genital tract. Chin Med J (Engl). 2000; 113: 1036-1039.

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