CASE REPORT
Angiofibroma of the Vulva Hassan Ahmadnia, Ali Kamalati, Mahmood Dolati, Alireza Akhavan Rezayat, and Mehrdad Katebi Background: Cellular angiofibroma is a benign and rare tumor. It usually arises in middle-aged women and involves the vulva. Complete local excision of the tumor is the best cure, and, usually, there is no recurrence after surgery. Case Report: We describe a 20-year-old woman with a painless, growing vulvar mass who presented about 3 years ago. Her past medical history was negative for oral contraceptives, tobacco, and alcohol, and there were no similar lesions in her family history. A physical examination revealed two masses on the right and the left labia majora and similar lesions on the left axilla and both breasts. An uncomplicated simple resection of the vulvar masses was done in the operating room. There was no evidence of recurrence 12 months after surgery. Histopathologic examination revealed that the findings were consistent with the diagnosis of the cellular angiofibroma. Based on our knowledge, this patient is the youngest case of vulvar angiofibroma. The lesions were large and symmetrical (on both labia majora), although they differed in size. Extravulvar (breast and axilla) lesions were also noteworthy in this patient. Contexte: L9angiofibrome cellulaire est une tumeur be´nigne, rare, qui se de´veloppe habituellement chez les femmes d9aˆge muˆr et qui touche la vulve. Le meilleur traitement est l9exe´re`se locale et comple`te de la tumeur, et ge´ne´ralement il n9y a pas de re´cidive apre`s la chirurgie. Expose´ de cas: Sera de´crit le cas d9une femme de 20 ans, qui a consulte´ il y a environ 3 ans, pour une masse indolore et grossissante, a` la hauteur de la vulve. Elle n9avait aucun ante´ce´dent me´dical quant a` la prise de contraceptifs oraux, de tabagisme ou de consommation d9alcool, pas plus qu9elle n9avait d9ante´ce´dents familiaux au regard de le´sions similaires. L9examen physique a re´ve´le´ la pre´sence de deux masses sur les grandes le`vres droite et gauche de la vulve ainsi que de le´sions similaires a` l9aisselle gauche et aux deux seins. Nous avons proce´de´ a` l9exe´re`se simple, et sans complications, des masses vulvaires en salle d9ope´ration. Il n9y avait aucun signe de re´cidive 12 mois apre`s l9ope´ration. L9examen histopathologique a confirme´ le diagnostic d9angiofibrome cellulaire. Il s9agit, a` notre connaissance, du premier cas d9angiofibrome cellulaire enregistre´ chez une femme si jeune. Les le´sions e´taient grosses et syme´triques (sur les deux grandes le`vres), mais de taille diffe´rente. Autre point d9inte´reˆt: la pre´sence de le´sions extravulvaires (aux seins et a` l9aisselle) chez cette patiente.
OME BENIGN MESENCHYMAL TUMORS and tumor-like lesions occur in the vulva. These are usually divided into two groups. Soft tissue lesions occur at any site of the body, including the vulva, such as lipoma, leiomyoma, and hemangioma. The second group includes tumors such as angiomyofibroblastoma, aggressive angiomyxoma, fibroepithelial stromal polyp, lipoblastoma-like
S
From the Department of Urology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran; Department of Urology, Shahid Bahonar Hospital, Kerman University of Medical Sciences, Kerman, Iran; and Bentolhoda Hospital, Mashhad, Iran. Address reprint requests to: Hassan Ahmadnia, MD, Department of Urology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran; e-mail: [email protected].
DOI 10.2310/7750.2013.13130 # 2014 Canadian Dermatology Association
tumor of the vulva, superficial cervicovaginal myofibroblastoma, and cellular angiofibroma.1–4 Angiofibroma of the vulva is a rare benign mesenchymal tumor. It was initially described in 1997.5 This lesion usually involves the vulva, although it has been described in a woman involving the subcutaneous tissue of the chest wall.6 This tumor has been described as a lesion of middleaged women, but this report describes a case of vulvar and extravulvar angiofibroma in a 20-year-old woman.
Case Report A 20-year-old single woman, who had no history of sexually transmitted disease or genital warts, presented with a painless, growing vulvar mass about 3 years ago. The patient also had similar lesions on the left axilla and her breasts. She denied taking oral contraceptives, tobacco,
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
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Ahmadnia et al
Figure 3. Simple resection of vulvar masses in the operating room.
Figure 1. Vulvar masses: the larger mass on the right labium majus and the smaller mass on the left labium majus.
or alcohol. There was no family history of breast or any other gynecologic cancer. A physical examination revealed a 4 cm, mobile, hard, and nontender mass involving the right labium majus and a smaller similar lesion on the left labium majus (Figure 1). Also, the patient had similar lesions on the left axilla and both breasts (Figure 2). Breast lesions in palpation were harder than the ones on the vulva. A gynecologic examination was normal, and the patient had a normal
Figure 2. Extravulvar masses on the left axilla and the left breast.
204
menstrual history. No lymph nodes were palpated on the groin examination. An uncomplicated simple resection of the vulvar masses was done in the operating room (Figure 3). Simple biopsy of the lesions of the breasts was also carried out. There were no postoperative complications, and there was no evidence of tumor recurrence 12 months after surgery. Informed consent was obtained prior to reporting the case.
Pathology On macroscopic examination, the larger mass size was 4 3 3 3 2 cm and the smaller one was 2 3 2 3 1 cm, firm, uniform, and white and was surrounded by a rim of normal tissue (Figure 4).
Figure 4. Macroscopic picture of the specimen.
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
Angiofibroma of the Vulva
relatively abundant small or medium-sized rounded vessels. Mast cells and adipocytes may also be present. Based on our knowledge, this patient is the youngest reported case of vulvar angiofibroma. The lesions were some of the largest ones and also symmetrical (on both labia majora), although they differed in size. Extravulvar (breast and axilla) lesions were also noteworthy in this patient.
Acknowledgment Financial disclosure of authors and reviewers: None reported.
References Figure 5. High-power view showing spindle- and oval-shaped stromal cells with bland vesicular nuclei and eosinophilic cytoplasm. Within these cells, many blood vessels with thick hyalinized walls were detected.
Histology of the biopsy showed spindle- and ovalshaped stromal cells with bland vesicular nuclei and eosinophilic cytoplasm. Within these cells, many blood vessels were detected with thick hyalinized walls (Figure 5). These findings are consistent with the diagnosis of cellular angiofibroma.
Discussion Vulvar angiofibroma is one of the benign mesenchymal tumors of the vulva and usually occurs in menopausal and middle-aged women with a mean age of 48 (range 37–77) years.5,7 Differential diagnosis of vulvar angiofibroma includes Bartholin gland, labial, and submucosal cysts.5 The best treatment choice is local excision with negative margins. Usually, this tumor does not recur, although it was once reported in a 49-year-old woman undergoing a simple excision of a 4 cm tumor. She subsequently developed a recurrent swelling at the site of the previous excision.8 Vulvar angiofibroma often arises in the labial region of the vulva and clitoris, but other sites, such as the perineum and breasts, have been reported.5,8–10 Vulvar angiofibroma usually occurs as a small lesion (usually less than 4 cm) with well-circumscribed margins. Histologically, angiofibroma consists of spindle-shaped cells lying between bands of collagen. It mixes up with
1. Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumors: update and review. Histopathology 2000;36:97–108, doi:10.1046/j.13652559.2000.00865.x. 2. Nielsen GP, Young RH. Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities. Int J Gynecol Pathol 2001;20:105–27, doi:10.1097/00004347-200104000-00002. 3. Laskin WB, Fetsch JF, Tavassoli FA. Superficial cervicovaginal myofibroblastoma: fourteen cases of a distinctive mesenchymal tumor arising from the specialized subepithelial stroma of the lower female genital tract. Hum Pathol 2001;32:715–25, doi:10.1053/hupa.2001.25588. 4. Lae MF, Pereira PF, Keeney GL, Nascimento AG. Lipoblastoma-like tumor of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation. Histopathology 2002;40: 505–14, doi:10.1046/j.1365-2559.2002.01409.x. 5. Nucci MR, Granter SR, Fletcher CDM. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol 1997;21:636–44, doi:10. 1097/00000478-199706000-00002. 6. Garijo MF, Val-Bernal JF. Extravulvar subcutaneous cellular angiofibroma. J Cutan Pathol 1998;25:327–32, doi:10.1111/j. 1600-0560.1998.tb01754.x. 7. Dargent JL, De Saint Aubain N, Galdon MG, et al. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of literature. J Cutan Pathol 2003;30:405–11, doi:10.1034/j.16000560.2003.00085.x. 8. McCluggage WG, Perenyei M, Irwin ST. Recurrent cellular angiofibroma of the vulva. J Clin Pathol 2002;55:477–80, doi:10. 1136/jcp.55.6.477-b. 9. Colombat M, Liard-Meilon ME, de Saint-Mour P, et al. L’angiofibrome cellularie, une tumeur vulvarie rare: a person d’un cas Ann Pathol 2001;21:145. 10. Curry JL, Olejnik JL, Wojeik EM. Cellular angiofibroma of the vulva with DNA ploidy analysis. Int Gynecol Pathol 2001;20:200–3, doi:10.1097/00004347-200104000-00015.
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
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Copyright of Journal of Cutaneous Medicine & Surgery is the property of Decker Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Angiofibroma of the Vulva Hassan Ahmadnia, Ali Kamalati, Mahmood Dolati, Alireza Akhavan Rezayat, and Mehrdad Katebi Background: Cellular angiofibroma is a benign and rare tumor. It usually arises in middle-aged women and involves the vulva. Complete local excision of the tumor is the best cure, and, usually, there is no recurrence after surgery. Case Report: We describe a 20-year-old woman with a painless, growing vulvar mass who presented about 3 years ago. Her past medical history was negative for oral contraceptives, tobacco, and alcohol, and there were no similar lesions in her family history. A physical examination revealed two masses on the right and the left labia majora and similar lesions on the left axilla and both breasts. An uncomplicated simple resection of the vulvar masses was done in the operating room. There was no evidence of recurrence 12 months after surgery. Histopathologic examination revealed that the findings were consistent with the diagnosis of the cellular angiofibroma. Based on our knowledge, this patient is the youngest case of vulvar angiofibroma. The lesions were large and symmetrical (on both labia majora), although they differed in size. Extravulvar (breast and axilla) lesions were also noteworthy in this patient. Contexte: L9angiofibrome cellulaire est une tumeur be´nigne, rare, qui se de´veloppe habituellement chez les femmes d9aˆge muˆr et qui touche la vulve. Le meilleur traitement est l9exe´re`se locale et comple`te de la tumeur, et ge´ne´ralement il n9y a pas de re´cidive apre`s la chirurgie. Expose´ de cas: Sera de´crit le cas d9une femme de 20 ans, qui a consulte´ il y a environ 3 ans, pour une masse indolore et grossissante, a` la hauteur de la vulve. Elle n9avait aucun ante´ce´dent me´dical quant a` la prise de contraceptifs oraux, de tabagisme ou de consommation d9alcool, pas plus qu9elle n9avait d9ante´ce´dents familiaux au regard de le´sions similaires. L9examen physique a re´ve´le´ la pre´sence de deux masses sur les grandes le`vres droite et gauche de la vulve ainsi que de le´sions similaires a` l9aisselle gauche et aux deux seins. Nous avons proce´de´ a` l9exe´re`se simple, et sans complications, des masses vulvaires en salle d9ope´ration. Il n9y avait aucun signe de re´cidive 12 mois apre`s l9ope´ration. L9examen histopathologique a confirme´ le diagnostic d9angiofibrome cellulaire. Il s9agit, a` notre connaissance, du premier cas d9angiofibrome cellulaire enregistre´ chez une femme si jeune. Les le´sions e´taient grosses et syme´triques (sur les deux grandes le`vres), mais de taille diffe´rente. Autre point d9inte´reˆt: la pre´sence de le´sions extravulvaires (aux seins et a` l9aisselle) chez cette patiente.
OME BENIGN MESENCHYMAL TUMORS and tumor-like lesions occur in the vulva. These are usually divided into two groups. Soft tissue lesions occur at any site of the body, including the vulva, such as lipoma, leiomyoma, and hemangioma. The second group includes tumors such as angiomyofibroblastoma, aggressive angiomyxoma, fibroepithelial stromal polyp, lipoblastoma-like
S
From the Department of Urology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran; Department of Urology, Shahid Bahonar Hospital, Kerman University of Medical Sciences, Kerman, Iran; and Bentolhoda Hospital, Mashhad, Iran. Address reprint requests to: Hassan Ahmadnia, MD, Department of Urology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran; e-mail: [email protected].
DOI 10.2310/7750.2013.13130 # 2014 Canadian Dermatology Association
tumor of the vulva, superficial cervicovaginal myofibroblastoma, and cellular angiofibroma.1–4 Angiofibroma of the vulva is a rare benign mesenchymal tumor. It was initially described in 1997.5 This lesion usually involves the vulva, although it has been described in a woman involving the subcutaneous tissue of the chest wall.6 This tumor has been described as a lesion of middleaged women, but this report describes a case of vulvar and extravulvar angiofibroma in a 20-year-old woman.
Case Report A 20-year-old single woman, who had no history of sexually transmitted disease or genital warts, presented with a painless, growing vulvar mass about 3 years ago. The patient also had similar lesions on the left axilla and her breasts. She denied taking oral contraceptives, tobacco,
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
203
Ahmadnia et al
Figure 3. Simple resection of vulvar masses in the operating room.
Figure 1. Vulvar masses: the larger mass on the right labium majus and the smaller mass on the left labium majus.
or alcohol. There was no family history of breast or any other gynecologic cancer. A physical examination revealed a 4 cm, mobile, hard, and nontender mass involving the right labium majus and a smaller similar lesion on the left labium majus (Figure 1). Also, the patient had similar lesions on the left axilla and both breasts (Figure 2). Breast lesions in palpation were harder than the ones on the vulva. A gynecologic examination was normal, and the patient had a normal
Figure 2. Extravulvar masses on the left axilla and the left breast.
204
menstrual history. No lymph nodes were palpated on the groin examination. An uncomplicated simple resection of the vulvar masses was done in the operating room (Figure 3). Simple biopsy of the lesions of the breasts was also carried out. There were no postoperative complications, and there was no evidence of tumor recurrence 12 months after surgery. Informed consent was obtained prior to reporting the case.
Pathology On macroscopic examination, the larger mass size was 4 3 3 3 2 cm and the smaller one was 2 3 2 3 1 cm, firm, uniform, and white and was surrounded by a rim of normal tissue (Figure 4).
Figure 4. Macroscopic picture of the specimen.
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
Angiofibroma of the Vulva
relatively abundant small or medium-sized rounded vessels. Mast cells and adipocytes may also be present. Based on our knowledge, this patient is the youngest reported case of vulvar angiofibroma. The lesions were some of the largest ones and also symmetrical (on both labia majora), although they differed in size. Extravulvar (breast and axilla) lesions were also noteworthy in this patient.
Acknowledgment Financial disclosure of authors and reviewers: None reported.
References Figure 5. High-power view showing spindle- and oval-shaped stromal cells with bland vesicular nuclei and eosinophilic cytoplasm. Within these cells, many blood vessels with thick hyalinized walls were detected.
Histology of the biopsy showed spindle- and ovalshaped stromal cells with bland vesicular nuclei and eosinophilic cytoplasm. Within these cells, many blood vessels were detected with thick hyalinized walls (Figure 5). These findings are consistent with the diagnosis of cellular angiofibroma.
Discussion Vulvar angiofibroma is one of the benign mesenchymal tumors of the vulva and usually occurs in menopausal and middle-aged women with a mean age of 48 (range 37–77) years.5,7 Differential diagnosis of vulvar angiofibroma includes Bartholin gland, labial, and submucosal cysts.5 The best treatment choice is local excision with negative margins. Usually, this tumor does not recur, although it was once reported in a 49-year-old woman undergoing a simple excision of a 4 cm tumor. She subsequently developed a recurrent swelling at the site of the previous excision.8 Vulvar angiofibroma often arises in the labial region of the vulva and clitoris, but other sites, such as the perineum and breasts, have been reported.5,8–10 Vulvar angiofibroma usually occurs as a small lesion (usually less than 4 cm) with well-circumscribed margins. Histologically, angiofibroma consists of spindle-shaped cells lying between bands of collagen. It mixes up with
1. Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumors: update and review. Histopathology 2000;36:97–108, doi:10.1046/j.13652559.2000.00865.x. 2. Nielsen GP, Young RH. Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities. Int J Gynecol Pathol 2001;20:105–27, doi:10.1097/00004347-200104000-00002. 3. Laskin WB, Fetsch JF, Tavassoli FA. Superficial cervicovaginal myofibroblastoma: fourteen cases of a distinctive mesenchymal tumor arising from the specialized subepithelial stroma of the lower female genital tract. Hum Pathol 2001;32:715–25, doi:10.1053/hupa.2001.25588. 4. Lae MF, Pereira PF, Keeney GL, Nascimento AG. Lipoblastoma-like tumor of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation. Histopathology 2002;40: 505–14, doi:10.1046/j.1365-2559.2002.01409.x. 5. Nucci MR, Granter SR, Fletcher CDM. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol 1997;21:636–44, doi:10. 1097/00000478-199706000-00002. 6. Garijo MF, Val-Bernal JF. Extravulvar subcutaneous cellular angiofibroma. J Cutan Pathol 1998;25:327–32, doi:10.1111/j. 1600-0560.1998.tb01754.x. 7. Dargent JL, De Saint Aubain N, Galdon MG, et al. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of literature. J Cutan Pathol 2003;30:405–11, doi:10.1034/j.16000560.2003.00085.x. 8. McCluggage WG, Perenyei M, Irwin ST. Recurrent cellular angiofibroma of the vulva. J Clin Pathol 2002;55:477–80, doi:10. 1136/jcp.55.6.477-b. 9. Colombat M, Liard-Meilon ME, de Saint-Mour P, et al. L’angiofibrome cellularie, une tumeur vulvarie rare: a person d’un cas Ann Pathol 2001;21:145. 10. Curry JL, Olejnik JL, Wojeik EM. Cellular angiofibroma of the vulva with DNA ploidy analysis. Int Gynecol Pathol 2001;20:200–3, doi:10.1097/00004347-200104000-00015.
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 18, No 3 (May/June), 2014: pp 203–205
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Copyright of Journal of Cutaneous Medicine & Surgery is the property of Decker Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
