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Superficial angiomyxoma on the vulva of an adolescent
Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 104e106
Contents lists available at ScienceDirect
Taiwanese Journal of Obstetrics & Gynecology journal homepage: www.tjog-online.com
Research Letter
Superficial angiomyxoma on the vulva of an adolescent Chia-Chieh Lee a, b, Yu-Li Chen a, b, Jau-Yu Liau c, Chi-An Chen a, Wen-Fang Cheng a, d, e, * a
Department of Obstetrics and Gynecology, College of Medicine, National Taiwan University, Taipei, Taiwan Gynecologic Cancer Center, Department of Obstetrics and Gynecology, Cathay General Hospital, Taipei, Taiwan c Department of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan d Graduate Institute of Oncology, College of Medicine, National Taiwan University, Taipei, Taiwan e Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan b
a r t i c l e i n f o Article history: Accepted 9 August 2013
Superficial angiomyxoma was first described as a cutaneous myxoma of Carney’s complex by Carney et al [1] in 1985. It was also reported to be an independent cutaneous tumor without the manifestations of Carney’s complex, including skin pigmentation or multiple myxomas over the heart, skin, or endocrine organs by Allen et al [2] in 1988 and Calonje et al [3] in 1999. It is a rare benign cutaneous tumor of soft tissue composed of prominent myxoid matrix and many blood vessels. Superficial angiomyxomas arise more commonly in males, with a peak incidence in the 4th decade of life [4,5]. Angiomyxoma tumors with a white, gray, or occasionally hemorrhagic appearance usually present as superficial, painless, and slow-growing nodules [2,3]. Grossly, the tumors arising from the overlying skin are moderately well-circumscribed, soft, and lobulated. These lesions are often located on the trunk, lower extremities, head and neck, and arms, and the cutting surface can be glistening, slimy, translucent, gelatinous, or colloid-like. Only a few cases of superficial angiomyxoma on the vulvar area have been reported in the literature [6]. Herein, we report a 13-year-old girl with a superficial angiomyxoma located on her left vulva with tumor bleeding. After the mass had been resected, the bleeding stopped and no recurrence was noted during a postoperative follow-up period of 1 year. A 13-year-old healthy female noted a mass on her left vulvar area 4 years previously. The vulvar mass gradually enlarged without any discomfort, but she did not seek medical advice. Eventually the tumor started to bleed and the frequency of bleeding increased. When massive vaginal bleeding from the tumor surface occurred, she visited our emergency department. A pedunculated Conflicts of interest: The authors have no conflicts of interest to declare. * Corresponding author. Department of Obstetrics and Gynecology, National Taiwan University Hospital, No. 8, Chung Shan S. Rd., Zhongzheng Dist., Taipei City 10041, Taiwan. E-mail address: [email protected] (W.-F. Cheng).
tumor measuring 7 cm 3 cm 2 cm in size with a brownish and eroded appearance was noted on the left vulvar area (Fig. 1A). The lesion was soft and elastic with a jelly-like composition on palpation. No associated pathological skin pigmentation or nodules were observed near the lesion. To investigate whether the lesion extended into the pelvic or abdominal cavity, computed tomography with contrast was performed. The image showed that the mass originated from the subcutaneous tissue without deep invasion through the perineal fascia into the pelvic cavity (Fig. 2). Vulva lipoma or other soft tissue mass was suspected, and excision of the vulvar mass was performed smoothly (Fig. 1B). Microscopically, the mass showed a lobulated tumor containing prominent myxoid stroma and thin walled vessels (Fig. 3A), and a higher power view showed spindle to stellate tumor cells. Diffused neutrophils were observed in the tumor (Fig. 3B); however, there was no nuclear pleomorphism or mitotic figures in the tumor cells. Based on the histological findings, a benign superficial angiomyxoma was diagnosed. The postoperative course was smooth, and she was discharged 1 day after surgery. No recurrence was noted during a postoperative follow-up period of 1 year. Diseases of the vulva constitute only a small fraction of those encountered in gynecological practice, and benign tumors of the vulva account for 22% of vulvar diseases. Superficial angiomyxoma is a rare benign soft tissue tumor rarely reported in the genital region, and to date only a few cases of superficial angiomyxoma on the vulva have been reported [6]. It is more commonly reported in males than in females [4,5]. No metastasis or malignancy of superficial angiomyxomas has been reported [7]. The lesion should be completely excised with a clear margin when possible. The rate of local recurrence is approximately 30e40%, and is associated with inadequate resection [2,3].
http://dx.doi.org/10.1016/j.tjog.2013.08.001 1028-4559/Copyright Ó 2014, Taiwan Association of Obstetrics & Gynecology. Published by Elsevier Taiwan LLC. All rights reserved.
C.-C. Lee et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 104e106
105
Fig. 1. (A) A pedunculated tumor arising from the left vulva with a brownish, elastic, and multilobular appearance. Some scale crust (black arrow) and erosive lesions (white arrow) were observed on this tumor. (B) The tumor, measuring 7 cm 3 cm 2 cm, was excised.
The diagnosis of superficial angiomyxoma is not usually made by clinicians, but rather by pathologists [7]. In microscopic findings, superficial angiomyxomas are well-circumscribed but unencapsulated multilobulated lesions, which frequently extend to the subcutaneous fat. The tumors consist of lobules of spindle-shaped or satellite fibroblast-like tumor cells with an abundance of myxoid stroma containing numerous thin-walled, small-to-medium-sized blood vessels and inflammatory cells [8]. The spindle cells of superficial angiomyxomas have been reported to be negative for actin, S100, and desmin [7]. In addition, nuclear pleomorphism and mitoses are uncommon in superficial angiomyxomas. Superficial angiomyxoma was first described with Carney’s complex syndrome by Carney et al [1] in 1985. In 1988, 28 cases of superficial angiomyxomas without other evidence of Carney’s complex were reported by Allen et al [2]. In 1999, Calonje et al [3] also reported the clinicopathological and immunohistochemical features of superficial angiomyxomas as an independent disease entity in 39 patients. Carney’s syndrome, a rare autosomal dominant disorder, is characterized by neoplasia involving the heart,
Fig. 2. Coronal view computed tomography. A subcutaneous vulvar mass with a welldefined margin was observed (star). The tumor did not invade through the deep perineal fascia (arrow) into the pelvic cavity.
central nervous system, and endocrine organs [1]. Cardiac myxomas can lead to sudden death, and the genetic defect responsible for this complex may be caused by mutations of the PRKAR1A gene on chromosome 17q. The presence of pigmented skin and mucosal lesions is an important hallmark of this syndrome. The association between Carney’s syndrome and superficial angiomyxomas arising in the genital region is unclear. However, when cutaneous myxomas are noted in multiple sites, Carney’s syndrome should be highly suspected [7]. Superficial angiomyxoma arises more commonly in males, with an age distribution from birth to 82 years and a peak incidence in the 4th decade of life [4e6]. These lesions can arise anywhere in the superficial tissue, but are most commonly found on the trunk, lower extremities, head and neck, and arms. Only a few cases of superficial angiomyxomas in the genital region have been reported [3,6,8]. In male patients, the tumors usually involve the scrotum. As with our patient, the superficial angiomyxomas previously reported in females occurred over the vulva, groin, or mons pubis with sizes ranging from 0.6 cm to 12.5 cm. However, the age of our patient was younger than the mean age (23 years) noted in other studies [3,6,8]. Our patient did not have associated manifestations of Carney’s syndrome including skin pigmentation, mucosal disorder, or other abnormal tumors. She also had no family history of myxomas. Therefore, we highly suspected that the superficial angiomyxoma was an independent lesion. However, a superficial angiomyxoma may be the earliest presentation of Carney’s syndrome in some cases [1]. Therefore, regular follow-up was still arranged for our patient. Superficial angiomyxoma should also be distinguished from aggressive angiomyxoma. Aggressive angiomyxoma occurs commonly in the soft tissue of the pelvic and peritoneal region. It occurs mainly in females and rarely in males; however, it is not easy to distinguish superficial angiomyxoma from aggressive angiomyxoma by clinical characteristics alone [6]. Histologically, a poorly circumscribed and infiltrative margin is seen with aggressive angiomyxomas. In addition, compared with the variable thin-wall vessels in superficial angiomyxomas, diffused median-sized thickwall blood vessels are noted in aggressive angiomyxomas [7]. Angiomyofibroblastomas should also be distinguished from superficial angiomyxomas. Clinically, angiomyofibroblastomas almost always occur in the vulvovaginal region of females, but also occasionally occur in the inguinoscrotal region of males. It is usually a cystic lesion, such as Bartholin’s duck cyst. Histologically, angiomyofibroblastomas show a well-circumscribed margin and are composed of alternating hypercellular and hypocellular areas [7]. The tumor contains numerous thin-walled, capillary-like vessels
106
C.-C. Lee et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 104e106
Fig. 3. (A) The lobulated tumor contained prominent myxoid stroma and thin-walled vessels. Cystic and basaloid epithelial components are observed. Original magnification: 40. (B) Thin-walled vessels and spindle to stellate tumor cells are observed. The presence of neutrophils is also characteristic of this tumor. Original magnification: 200. (C) Overlying normal vulvar skin histology: 100.
surrounded with epithelioid or plasmacytoid tumor cells. These characteristics can be used to distinguish angiomyofibroblastomas from superficial angiomyxomas. In conclusion, superficial angiomyxoma is a rare benign soft tissue tumor, rarely arising in the genital region. These tumors can be independent lesions or the earliest presentation of Carney’s syndrome. Local recurrence is noted even after excision, and regular follow-up is recommended for these patients. References [1] Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985;64:270e83.
[2] Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with or without epithelial components. Report of 30 tumors in 28 patients. Am J Surg Pathol 1988;12:519e30. [3] Calonje E, Guerin D, Mc Cormick D, Fletcher CD. Superficial angiomyxoma. Clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23: 910e7. [4] Basak S, Rogers S, Solomonsz AF. Superficial angiomyxoma of the vulva: a case report of a rare cutaneous tumour. J Obstet Gynaecol 2011;31:360e1. [5] Nakamura M, Tokura Y. Superficial angiomyxoma on the scrotum of a child. Pediatr Dermatol 2011;28:200e1. [6] Kim HS, Kim GY, Lim SJ, Ki KD, Kim HC. Giant superficial angiomyxoma of the vulva: a case report and review of the literature. J Cutan Pathol 2010;37:672e7. [7] Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumours: update and review. Histopathology 2000;36:97e108. [8] Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol 1997;16:325e34.
Contents lists available at ScienceDirect
Taiwanese Journal of Obstetrics & Gynecology journal homepage: www.tjog-online.com
Research Letter
Superficial angiomyxoma on the vulva of an adolescent Chia-Chieh Lee a, b, Yu-Li Chen a, b, Jau-Yu Liau c, Chi-An Chen a, Wen-Fang Cheng a, d, e, * a
Department of Obstetrics and Gynecology, College of Medicine, National Taiwan University, Taipei, Taiwan Gynecologic Cancer Center, Department of Obstetrics and Gynecology, Cathay General Hospital, Taipei, Taiwan c Department of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan d Graduate Institute of Oncology, College of Medicine, National Taiwan University, Taipei, Taiwan e Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan b
a r t i c l e i n f o Article history: Accepted 9 August 2013
Superficial angiomyxoma was first described as a cutaneous myxoma of Carney’s complex by Carney et al [1] in 1985. It was also reported to be an independent cutaneous tumor without the manifestations of Carney’s complex, including skin pigmentation or multiple myxomas over the heart, skin, or endocrine organs by Allen et al [2] in 1988 and Calonje et al [3] in 1999. It is a rare benign cutaneous tumor of soft tissue composed of prominent myxoid matrix and many blood vessels. Superficial angiomyxomas arise more commonly in males, with a peak incidence in the 4th decade of life [4,5]. Angiomyxoma tumors with a white, gray, or occasionally hemorrhagic appearance usually present as superficial, painless, and slow-growing nodules [2,3]. Grossly, the tumors arising from the overlying skin are moderately well-circumscribed, soft, and lobulated. These lesions are often located on the trunk, lower extremities, head and neck, and arms, and the cutting surface can be glistening, slimy, translucent, gelatinous, or colloid-like. Only a few cases of superficial angiomyxoma on the vulvar area have been reported in the literature [6]. Herein, we report a 13-year-old girl with a superficial angiomyxoma located on her left vulva with tumor bleeding. After the mass had been resected, the bleeding stopped and no recurrence was noted during a postoperative follow-up period of 1 year. A 13-year-old healthy female noted a mass on her left vulvar area 4 years previously. The vulvar mass gradually enlarged without any discomfort, but she did not seek medical advice. Eventually the tumor started to bleed and the frequency of bleeding increased. When massive vaginal bleeding from the tumor surface occurred, she visited our emergency department. A pedunculated Conflicts of interest: The authors have no conflicts of interest to declare. * Corresponding author. Department of Obstetrics and Gynecology, National Taiwan University Hospital, No. 8, Chung Shan S. Rd., Zhongzheng Dist., Taipei City 10041, Taiwan. E-mail address: [email protected] (W.-F. Cheng).
tumor measuring 7 cm 3 cm 2 cm in size with a brownish and eroded appearance was noted on the left vulvar area (Fig. 1A). The lesion was soft and elastic with a jelly-like composition on palpation. No associated pathological skin pigmentation or nodules were observed near the lesion. To investigate whether the lesion extended into the pelvic or abdominal cavity, computed tomography with contrast was performed. The image showed that the mass originated from the subcutaneous tissue without deep invasion through the perineal fascia into the pelvic cavity (Fig. 2). Vulva lipoma or other soft tissue mass was suspected, and excision of the vulvar mass was performed smoothly (Fig. 1B). Microscopically, the mass showed a lobulated tumor containing prominent myxoid stroma and thin walled vessels (Fig. 3A), and a higher power view showed spindle to stellate tumor cells. Diffused neutrophils were observed in the tumor (Fig. 3B); however, there was no nuclear pleomorphism or mitotic figures in the tumor cells. Based on the histological findings, a benign superficial angiomyxoma was diagnosed. The postoperative course was smooth, and she was discharged 1 day after surgery. No recurrence was noted during a postoperative follow-up period of 1 year. Diseases of the vulva constitute only a small fraction of those encountered in gynecological practice, and benign tumors of the vulva account for 22% of vulvar diseases. Superficial angiomyxoma is a rare benign soft tissue tumor rarely reported in the genital region, and to date only a few cases of superficial angiomyxoma on the vulva have been reported [6]. It is more commonly reported in males than in females [4,5]. No metastasis or malignancy of superficial angiomyxomas has been reported [7]. The lesion should be completely excised with a clear margin when possible. The rate of local recurrence is approximately 30e40%, and is associated with inadequate resection [2,3].
http://dx.doi.org/10.1016/j.tjog.2013.08.001 1028-4559/Copyright Ó 2014, Taiwan Association of Obstetrics & Gynecology. Published by Elsevier Taiwan LLC. All rights reserved.
C.-C. Lee et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 104e106
105
Fig. 1. (A) A pedunculated tumor arising from the left vulva with a brownish, elastic, and multilobular appearance. Some scale crust (black arrow) and erosive lesions (white arrow) were observed on this tumor. (B) The tumor, measuring 7 cm 3 cm 2 cm, was excised.
The diagnosis of superficial angiomyxoma is not usually made by clinicians, but rather by pathologists [7]. In microscopic findings, superficial angiomyxomas are well-circumscribed but unencapsulated multilobulated lesions, which frequently extend to the subcutaneous fat. The tumors consist of lobules of spindle-shaped or satellite fibroblast-like tumor cells with an abundance of myxoid stroma containing numerous thin-walled, small-to-medium-sized blood vessels and inflammatory cells [8]. The spindle cells of superficial angiomyxomas have been reported to be negative for actin, S100, and desmin [7]. In addition, nuclear pleomorphism and mitoses are uncommon in superficial angiomyxomas. Superficial angiomyxoma was first described with Carney’s complex syndrome by Carney et al [1] in 1985. In 1988, 28 cases of superficial angiomyxomas without other evidence of Carney’s complex were reported by Allen et al [2]. In 1999, Calonje et al [3] also reported the clinicopathological and immunohistochemical features of superficial angiomyxomas as an independent disease entity in 39 patients. Carney’s syndrome, a rare autosomal dominant disorder, is characterized by neoplasia involving the heart,
Fig. 2. Coronal view computed tomography. A subcutaneous vulvar mass with a welldefined margin was observed (star). The tumor did not invade through the deep perineal fascia (arrow) into the pelvic cavity.
central nervous system, and endocrine organs [1]. Cardiac myxomas can lead to sudden death, and the genetic defect responsible for this complex may be caused by mutations of the PRKAR1A gene on chromosome 17q. The presence of pigmented skin and mucosal lesions is an important hallmark of this syndrome. The association between Carney’s syndrome and superficial angiomyxomas arising in the genital region is unclear. However, when cutaneous myxomas are noted in multiple sites, Carney’s syndrome should be highly suspected [7]. Superficial angiomyxoma arises more commonly in males, with an age distribution from birth to 82 years and a peak incidence in the 4th decade of life [4e6]. These lesions can arise anywhere in the superficial tissue, but are most commonly found on the trunk, lower extremities, head and neck, and arms. Only a few cases of superficial angiomyxomas in the genital region have been reported [3,6,8]. In male patients, the tumors usually involve the scrotum. As with our patient, the superficial angiomyxomas previously reported in females occurred over the vulva, groin, or mons pubis with sizes ranging from 0.6 cm to 12.5 cm. However, the age of our patient was younger than the mean age (23 years) noted in other studies [3,6,8]. Our patient did not have associated manifestations of Carney’s syndrome including skin pigmentation, mucosal disorder, or other abnormal tumors. She also had no family history of myxomas. Therefore, we highly suspected that the superficial angiomyxoma was an independent lesion. However, a superficial angiomyxoma may be the earliest presentation of Carney’s syndrome in some cases [1]. Therefore, regular follow-up was still arranged for our patient. Superficial angiomyxoma should also be distinguished from aggressive angiomyxoma. Aggressive angiomyxoma occurs commonly in the soft tissue of the pelvic and peritoneal region. It occurs mainly in females and rarely in males; however, it is not easy to distinguish superficial angiomyxoma from aggressive angiomyxoma by clinical characteristics alone [6]. Histologically, a poorly circumscribed and infiltrative margin is seen with aggressive angiomyxomas. In addition, compared with the variable thin-wall vessels in superficial angiomyxomas, diffused median-sized thickwall blood vessels are noted in aggressive angiomyxomas [7]. Angiomyofibroblastomas should also be distinguished from superficial angiomyxomas. Clinically, angiomyofibroblastomas almost always occur in the vulvovaginal region of females, but also occasionally occur in the inguinoscrotal region of males. It is usually a cystic lesion, such as Bartholin’s duck cyst. Histologically, angiomyofibroblastomas show a well-circumscribed margin and are composed of alternating hypercellular and hypocellular areas [7]. The tumor contains numerous thin-walled, capillary-like vessels
106
C.-C. Lee et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 104e106
Fig. 3. (A) The lobulated tumor contained prominent myxoid stroma and thin-walled vessels. Cystic and basaloid epithelial components are observed. Original magnification: 40. (B) Thin-walled vessels and spindle to stellate tumor cells are observed. The presence of neutrophils is also characteristic of this tumor. Original magnification: 200. (C) Overlying normal vulvar skin histology: 100.
surrounded with epithelioid or plasmacytoid tumor cells. These characteristics can be used to distinguish angiomyofibroblastomas from superficial angiomyxomas. In conclusion, superficial angiomyxoma is a rare benign soft tissue tumor, rarely arising in the genital region. These tumors can be independent lesions or the earliest presentation of Carney’s syndrome. Local recurrence is noted even after excision, and regular follow-up is recommended for these patients. References [1] Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985;64:270e83.
[2] Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with or without epithelial components. Report of 30 tumors in 28 patients. Am J Surg Pathol 1988;12:519e30. [3] Calonje E, Guerin D, Mc Cormick D, Fletcher CD. Superficial angiomyxoma. Clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23: 910e7. [4] Basak S, Rogers S, Solomonsz AF. Superficial angiomyxoma of the vulva: a case report of a rare cutaneous tumour. J Obstet Gynaecol 2011;31:360e1. [5] Nakamura M, Tokura Y. Superficial angiomyxoma on the scrotum of a child. Pediatr Dermatol 2011;28:200e1. [6] Kim HS, Kim GY, Lim SJ, Ki KD, Kim HC. Giant superficial angiomyxoma of the vulva: a case report and review of the literature. J Cutan Pathol 2010;37:672e7. [7] Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumours: update and review. Histopathology 2000;36:97e108. [8] Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol 1997;16:325e34.