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Hemangioma of the Clitoris Presenting as Clitoromegaly: A Case Report
J Pediatr Adolesc Gynecol (2009) 22:e137ee138
Case Report Hemangioma of the Clitoris Presenting as Clitoromegaly: A Case Report Vincenzina Bruni1, Valentina Pontello1, Metella Dei1, Marco Alessandrini2, Vincenzo Li Marzi2, and Giulio Nicita2 1
Department of Gynecology, Perinatology and Human Reproduction; 2Clinica Urologica II, University of Florence, Italy
Abstract. A 20-year-old woman with massive clitoral enlargement is presented to discuss the differential diagnosis and the treatment of this condition.
Key Words. Clitoromegaly—Clitoral enlargement— Hemangioma
Introduction Clitoromegaly is defined as a measure of the clitoral index (width length in mm) more than 15 mm2 in the newborn and more than 21 mm2 in the adult woman. This condition can be of congenital or acquired nature, and the most frequent causes are hormonal, i.e. related to hyperandrogenism, but can very rarely be non-hormonal, due to clitoral or prepucial masses. We present a case report of clitoromegaly, due to a cavernous hemangioma of the clitoris. Case Presentation A 20-year-old woman presented at our clinic complaining that a massive clitoral enlargement appeared three years before. Family history was negative, and medical history showed no significant pathology, except for mild allergic asthma. The patient reported age at menarche at 11 years and oligomenorrhea (cycle length 40e45 days), for which she had used oral contraceptives from 15 to 17 years of age. Address correspondence to: Vincenzina Bruni, Department of Gynecology, Perinatology and Human Reproduction, Ospedale di Careggi, Viale Morgagni 85, 50134 Firenze, Italy; E-mail: [email protected] Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
External genitalia examination showed a clitoral mass of 5020mm (5clitoral index 1000 mm2) and pelvic visit was normal (Fig. 1). Interestingly, the patient presented no clinical sign of hyperandrogenism, and biochemical analysis of follicular phase gonadotropins and androgens was normal, including 17OH progesterone, testosterone, D4 androstenedione, and DHEAS. The next diagnostic step consisted of the performance of a vulvar ultrasound, which showed a nodular mass (measuring 461926mm) in the thickness of the right labia minora, displacing the clitoris in the opposite direction. The ultrasound appearance of the mass was non-homogeneous and solid with small hypoechoic areas. The patient was referred for surgery, which was performed under general anesthesia. The prepuce of the clitoris and the right labia minora were incised for 3 cm, and the mass, which appeared adherent to the body and glans of the clitoris, was dissected from superficial and deep tissues using scissors and the electrosurgical knife. The subsequent removal was executed with preservation of the corpora cavernosa and the neurovascular bundle of the clitoris (Fig. 2). The duration of the operation was 45 minutes. Histologic examination of the mass showed a cavernous hemangioma. The urinary Foley catheter was removed on first postoperative day, and on the second day the patient was discharged. Follow-up visits at 1, 3, and 6 months showed a recovery of normal anatomical features and no signs of recurrence appeared. Clitoral sensitivity was reported by the patient as reduced at 3e6 months, and normal at 9 months from surgery. Discussion Clitoromegaly is a condition usually associated with sustained high androgen serum levels (Table 1). 1083-3188/09/$36.00 doi:10.1016/j.jpag.2008.11.002
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Bruni et al: Clitoral Hemangioma Presenting as Clitoromegaly Table 1. Causes for Clitoromegaly Congenital Congenital adrenal hyperplasia, classical Ambiguous genitalia, isolated or in syndromic conditions Acquired Hormonal: Congenital adrenal hyperplasia, late onset Ovarian or adrenal tumours (androgen secreting) Iatrogenic androgen exposure Non hormonal Neurofibromatosis Epidermoid cyst (spontaneous or traumatic e female genital mutilation -) Hemangioma of the clitoris or the prepuce Metastatic infiltration Idiopatic
Fig. 1. Appearance of the external genitalia before surgery.
Non-hormonal causes are exceptionally rare: in the literature there are a few cases in neurofibromatosis patients,1 and epidermoid cysts of the clitoris are a known complication of female genital mutilation, but these can also occur spontaneously.2 Hemangioma of the clitoris or the prepuce has so far been reported in 4 cases worldwide.3e6 Only one case of clitoral metastatic infiltration has been described in a postmenopausal patient, affected by bladder carcinosarcoma.7 Therefore, the first diagnostic step in the approach to clitoromegaly is to exclude hormonal causes, especially congenital adrenal hyperplasia, through general examination and the investigation of biochemical markers of hyperandrogenism, in particular 17OH progesterone. A serum 17OH progesterone that exceeds 2 ng/ml in the follicular phase of a menstruating young woman raises the suspect of congenital adrenal hyperplasia, and therefore represents a indication to perform an ACTH test. When the biochemical tests are normal, the next diagnostic step can be imaging techniques. Ultrasound
Fig. 2. Intraoperative view of the hemangioma.
is a highly informative and low cost modality, according to the experience of other authors.8 Indeed, in our case, an MRI was performed, without any additional findings. In conclusion, the correct diagnosis of clitoromegaly is essential to assure that the appropriate therapy is undertaken, which is surgical in non-hormonal cases. References 1. Sutphen R, Galan-Gomez E, Koussef BG: Clitoromegaly in neurofibromatosis. Am J Med Genet 1995; 55:325 2. Rouzi AA, Sindi O, Radhan B, et al: Epidermal clitoral inclusion cyst after type I female genital mutilation. Am J Obstet Gynecol 2001; 185:569 3. Kaufman-Friedman K: Hemangioma of clitoris, confused with adrenogenital syndrome: case report. Plast Reconstr Surg 1978; 62:452 4. Ishizu K, Nakamura K, Baba Y, et al: [Clitoral enlargment caused by prepucial hemangioma: a case report.] Hinyokika Kiyo 1991; 37:1563 5. Strayer SA, Yum MN, Sutton GP: Epithelioid hemangioendothelioma of the clitoris: a case report with immunohistochemical and ultrastructural findings. Int J Gynecol Pathol 1992; 11:234 6. Haritharan T, Islah M, Zulfiqar A, et al: Solitary vascular malformation of the clitoris. Med J Malaysia 2006; 61:258 7. Langenstroer P, Romanas M, Damjanov I: Clitoromegaly caused by metastatic carcinosarcoma of the urinary bladder. Arch Pathol Lab Med 2003; 127:505 8. Robin G, Marcelli F, Agberta N, et al: Contribution of ultrasonography to the diagnosis of non-hormonal acquired clitoromegalia: a case report. Ann Endocrinol (Paris) 2006; 67: 613e6
Case Report Hemangioma of the Clitoris Presenting as Clitoromegaly: A Case Report Vincenzina Bruni1, Valentina Pontello1, Metella Dei1, Marco Alessandrini2, Vincenzo Li Marzi2, and Giulio Nicita2 1
Department of Gynecology, Perinatology and Human Reproduction; 2Clinica Urologica II, University of Florence, Italy
Abstract. A 20-year-old woman with massive clitoral enlargement is presented to discuss the differential diagnosis and the treatment of this condition.
Key Words. Clitoromegaly—Clitoral enlargement— Hemangioma
Introduction Clitoromegaly is defined as a measure of the clitoral index (width length in mm) more than 15 mm2 in the newborn and more than 21 mm2 in the adult woman. This condition can be of congenital or acquired nature, and the most frequent causes are hormonal, i.e. related to hyperandrogenism, but can very rarely be non-hormonal, due to clitoral or prepucial masses. We present a case report of clitoromegaly, due to a cavernous hemangioma of the clitoris. Case Presentation A 20-year-old woman presented at our clinic complaining that a massive clitoral enlargement appeared three years before. Family history was negative, and medical history showed no significant pathology, except for mild allergic asthma. The patient reported age at menarche at 11 years and oligomenorrhea (cycle length 40e45 days), for which she had used oral contraceptives from 15 to 17 years of age. Address correspondence to: Vincenzina Bruni, Department of Gynecology, Perinatology and Human Reproduction, Ospedale di Careggi, Viale Morgagni 85, 50134 Firenze, Italy; E-mail: [email protected] Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
External genitalia examination showed a clitoral mass of 5020mm (5clitoral index 1000 mm2) and pelvic visit was normal (Fig. 1). Interestingly, the patient presented no clinical sign of hyperandrogenism, and biochemical analysis of follicular phase gonadotropins and androgens was normal, including 17OH progesterone, testosterone, D4 androstenedione, and DHEAS. The next diagnostic step consisted of the performance of a vulvar ultrasound, which showed a nodular mass (measuring 461926mm) in the thickness of the right labia minora, displacing the clitoris in the opposite direction. The ultrasound appearance of the mass was non-homogeneous and solid with small hypoechoic areas. The patient was referred for surgery, which was performed under general anesthesia. The prepuce of the clitoris and the right labia minora were incised for 3 cm, and the mass, which appeared adherent to the body and glans of the clitoris, was dissected from superficial and deep tissues using scissors and the electrosurgical knife. The subsequent removal was executed with preservation of the corpora cavernosa and the neurovascular bundle of the clitoris (Fig. 2). The duration of the operation was 45 minutes. Histologic examination of the mass showed a cavernous hemangioma. The urinary Foley catheter was removed on first postoperative day, and on the second day the patient was discharged. Follow-up visits at 1, 3, and 6 months showed a recovery of normal anatomical features and no signs of recurrence appeared. Clitoral sensitivity was reported by the patient as reduced at 3e6 months, and normal at 9 months from surgery. Discussion Clitoromegaly is a condition usually associated with sustained high androgen serum levels (Table 1). 1083-3188/09/$36.00 doi:10.1016/j.jpag.2008.11.002
e138
Bruni et al: Clitoral Hemangioma Presenting as Clitoromegaly Table 1. Causes for Clitoromegaly Congenital Congenital adrenal hyperplasia, classical Ambiguous genitalia, isolated or in syndromic conditions Acquired Hormonal: Congenital adrenal hyperplasia, late onset Ovarian or adrenal tumours (androgen secreting) Iatrogenic androgen exposure Non hormonal Neurofibromatosis Epidermoid cyst (spontaneous or traumatic e female genital mutilation -) Hemangioma of the clitoris or the prepuce Metastatic infiltration Idiopatic
Fig. 1. Appearance of the external genitalia before surgery.
Non-hormonal causes are exceptionally rare: in the literature there are a few cases in neurofibromatosis patients,1 and epidermoid cysts of the clitoris are a known complication of female genital mutilation, but these can also occur spontaneously.2 Hemangioma of the clitoris or the prepuce has so far been reported in 4 cases worldwide.3e6 Only one case of clitoral metastatic infiltration has been described in a postmenopausal patient, affected by bladder carcinosarcoma.7 Therefore, the first diagnostic step in the approach to clitoromegaly is to exclude hormonal causes, especially congenital adrenal hyperplasia, through general examination and the investigation of biochemical markers of hyperandrogenism, in particular 17OH progesterone. A serum 17OH progesterone that exceeds 2 ng/ml in the follicular phase of a menstruating young woman raises the suspect of congenital adrenal hyperplasia, and therefore represents a indication to perform an ACTH test. When the biochemical tests are normal, the next diagnostic step can be imaging techniques. Ultrasound
Fig. 2. Intraoperative view of the hemangioma.
is a highly informative and low cost modality, according to the experience of other authors.8 Indeed, in our case, an MRI was performed, without any additional findings. In conclusion, the correct diagnosis of clitoromegaly is essential to assure that the appropriate therapy is undertaken, which is surgical in non-hormonal cases. References 1. Sutphen R, Galan-Gomez E, Koussef BG: Clitoromegaly in neurofibromatosis. Am J Med Genet 1995; 55:325 2. Rouzi AA, Sindi O, Radhan B, et al: Epidermal clitoral inclusion cyst after type I female genital mutilation. Am J Obstet Gynecol 2001; 185:569 3. Kaufman-Friedman K: Hemangioma of clitoris, confused with adrenogenital syndrome: case report. Plast Reconstr Surg 1978; 62:452 4. Ishizu K, Nakamura K, Baba Y, et al: [Clitoral enlargment caused by prepucial hemangioma: a case report.] Hinyokika Kiyo 1991; 37:1563 5. Strayer SA, Yum MN, Sutton GP: Epithelioid hemangioendothelioma of the clitoris: a case report with immunohistochemical and ultrastructural findings. Int J Gynecol Pathol 1992; 11:234 6. Haritharan T, Islah M, Zulfiqar A, et al: Solitary vascular malformation of the clitoris. Med J Malaysia 2006; 61:258 7. Langenstroer P, Romanas M, Damjanov I: Clitoromegaly caused by metastatic carcinosarcoma of the urinary bladder. Arch Pathol Lab Med 2003; 127:505 8. Robin G, Marcelli F, Agberta N, et al: Contribution of ultrasonography to the diagnosis of non-hormonal acquired clitoromegalia: a case report. Ann Endocrinol (Paris) 2006; 67: 613e6