Journal of Obstetrics and Gynaecology
ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20
A case of vulvar superficial angiomyxoma: A rare clinical entity Mucize Ozdemir, Isil Uzun, Ayse Karahasanoglu & Canan Ceylan To cite this article: Mucize Ozdemir, Isil Uzun, Ayse Karahasanoglu & Canan Ceylan (2016): A case of vulvar superficial angiomyxoma: A rare clinical entity, Journal of Obstetrics and Gynaecology, DOI: 10.3109/01443615.2016.1148674 To link to this article: http://dx.doi.org/10.3109/01443615.2016.1148674
Published online: 29 Feb 2016.
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Date: 17 March 2016, At: 19:54
Journal of Obstetrics and Gynaecology, 2016; Early Online: 1–2 ß 2016 Taylor & Francis ISSN 0144-3615 print/ISSN 1364-6893 online DOI: 10.3109/01443615.2016.1148674
CASE REPORT
A case of vulvar superficial angiomyxoma: A rare clinical entity Mucize Ozdemir1, Isil Uzun2, Ayse Karahasanoglu1 & Canan Ceylan3 Department of Obstetrics and Gynecology, Su¨leymaniye Education and Research Hospital, Istanbul, Turkey, 2Department of Obstetrics and Gynecology, Acibadem Bakirkoy Hospital, Istanbul, Turkey, and 3Department of Pathology, Istanbul University, Istanbul Medical School, Istanbul, Turkey
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1
Introduction Superficial angiomyxomas are rare cutaneous tumours frequently involving the subcutis. It occurs most commonly in the fourth decade, presenting as a slowly growing painless mass that usually measures less than 50 mm. Superficial angiomyxomas are benign; however, it has a 30–40% rate of local non-destructive recurrence and it should be completely excised with a clear margin whenever possible (Calonje et al. 1999; Nucci and Fletcher 2000). Here we report a case of a 26 year-old female with a superficial angiomyxoma of the vulva.
Case report A 26 year-old woman, G2P2, was admitted to our clinic with a complaint of painless mass in the subcutaneous layer of the vulva between the right labium majus and the clitoris. The patient presented a history of vulvar mass excision two years ago but had no histopathologic report or medical documents. Local examination showed a well-circumscribed soft mass, measuring about 6 43 cm. The patient underwent local excision of the mass under general anaesthesia. The overlying skin had no ulcerative or erosive lesion (Figure 1). Microscopically, the tumour was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. These cells showed low cellularity with no atypia and mitosis. Variable-sized thin-walled capillaries and the scattered inflammatory cells, especially stromal neutrophils were seen in the stroma. Based on the pathologic and clinical features, superficial angiomyxoma was diagnosed.
Discussion Superficial angiomyxomas are more commonly located on extragenital sites, especially the head, neck and the trunk (Calonje et al. 1999; Nucci and Fletcher 2000). Genital Superficial Angiomyxomas usually involve the vulvovaginal region, almost always the vulva (Fetsch et al. 1997). Multiple cutaneous myxomas and angiomyxomas are occasionally features of Carney’s complex which is an autosomal dominant disorder, associated with cutaneous myxomas. But the association with Carney’s complex has not been shown in genital Superficial Angiomyxomas (Fetsch et al. 1997). Only 19 cases of
vulvar superficial angiomyxomas have been reported in the literature. Due to its rare occurrence, vulvar superficial angiomyxomas may be misdiagnosed as a polyp, Bartholin cyst, Gartner duct cyst or a perineal herniation in the physical examination. The diagnosis of vulvar superficial angiomyxoma is usually made by the pathologist (Kim et al. 2010). Superficial angiomyxomas are well-circumscribed, nodular or multinodular lesions, which frequently extend to subcutaneous fat. They are usually bigger than 5 cm in diameter. The cut surface is myxoid or gelatinous. There are multinucleated cells with few or no mitotic figures. Thin walled capillary-like blood vessels are present. Presence of stromal neutrophils is almost always invariable, which is a useful diagnostic aid. Superficial angiomyxomas are benign lesions with no metastatic potential; however, local non-destructive recurrence is not uncommon and the lesions should be excised with a clear resection margin (Bedlow et al. 1997; Calonje et al. 1999; Nucci and Fletcher 2000; Mc Cluggage 2004). The differential diagnosis of superficial angiomyxomas with aggressive angiomyxoma is very important. Aggressive angiomyxoma is a non-metastasizing, locally infiltrative tumour which occurs most commonly in the soft tissue of female pelvis and perineum. Aggressive angiomyxoma are large lesions usually greater than 10 cm in diameter (Dierickx et al. 2008). Typically aggressive angiomyxoma, like many of the other vulvovaginal mesenchymal lesions, is clinically thought to represent a cystic lesion, such as a Bartholin’s gland cyst. On gross examination, they are typically poorly circumscribed lesions with agelatinous, myxoid or fibrous consistency. Aggressive angiomyxomas are non-encapsulated tumours with an infiltrative edge and tendency to local recurrence. Histologically, the tumour consists of a hypocellular population of small neoplastic cells. Mitotic activity and nuclear atypia are absent. In general, this is a larger deeper-seated lesion and has an infiltrative margin that contrasts with the multi-nodular growth pattern of the superficial angiomyxoma. The blood vessels are more variable in aggressive angiomyxoma usually with a component thick walled vessel, whereas the presence of stromal neutrophils favours the superficial angiomyxoma. In contrast to aggressive angiomyxoma, the recurrence of superficial angiomyxoma is non-destructive and metastasis of superficial angiomyxoma has not been reported before (Steeper and Rosai 1983; Nucci and Fletcher 2000).
Correspondence: Isil Uzun, Yedikule Konaklari B4/D4 Fatih, Istanbul, Turkey. Tel: +90 5325141526. E-mail: [email protected]
2
M. Ozdemir et al. In summary, the authors described a very rare mesenchymal tumour arising in vulvar area. Vulvar superficial angiomyxomas are rare benign neoplasms that should be distinguished from other vulvar soft tissue tumours, especially aggressive angiomyxomas which have infiltrative nature and high risk of recurrence. Declaration of interest: All of the authors state that there are no conflicts of interest.
References
Downloaded by [Purdue University Libraries] at 19:54 17 March 2016
Figure 1. The operation.
Angiomyofibroblastoma is another mesenchymal tumour which occurs commonly on the vulva. It is a well-circumscribed lesion that clinically is often thought to represent a Bartholin’s gland cyst. Angiomyofibroblastomas usually measure less than 5 cm in diameter. Histologically, an angiomyofibroblastoma shows alternating hypocellular and hypercellular areas. Superficial angiomyxomas can be distinguished from angiomyofibroblastoma by characteristics of multinodular growth, sparse cellularity with abundant myxoid material (Mc Cluggage 2004; Nucci and Fletcher 2000). Overall angiomyofibroblastoma is a benign non-recurring lesion and local excision with clear margins is an adequate treatment. There is one report of Angiofibroblastoma with a sarcomatous transformation that recurred (Nielsen et al. 1997).
Bedlow AJ, Sampson SA, Holden CA. 1997. Congenital superficial angiomyxoma. Clinical and Experimental Dermatology 22:237. Calonje E, Guerin D, McCormick D, Fletcher CD. 1999. Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. The American Journal of Surgical Pathology 23:910–917. Dierickx I, Deraedt K, Poppe W, Verguts J. 2008. Aggressive angiomyxoma of the vulva: a case report and review of literature. Archives of Gynecology and Obstetrics 277:483. Fetsch JF, Laskin WB, Tavasolli FA. 1997. Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. International Journal of Gynecological Pathology 16:325–334. Kim HY, Kim GY, Lim SJ, Ki KY, Kim HC. 2010. Giant superficial angiomyxoma of the vulva: a case report and review of the literature. Journal of Cutaneous Pathology 37:672–677. McCluggage GW. 2004. A review and update of morphologically bland vulvovaginal mesenchymal lesions. International Journal of Gynecological Pathology 24:26–38. Nielsen GP, Young RH, Dickerson GR, Rosenberg AE. 1997. Angiomyofibroblastoma of the vulva with sarcomatous transformation. The American Journal of Surgical Pathology 21:1104–1108. Nucci MR, Fletcher CD. 2000. Vulvovaginal soft tissue tumours: update and review. Histopathology 36:97–108. Steeper TA, Rosai J. 1983. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic softtissue neoplasm. Perineum report of cases a type gynecologic tissue. The American Journal of Surgical Pathology 7:463–475.
ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20
A case of vulvar superficial angiomyxoma: A rare clinical entity Mucize Ozdemir, Isil Uzun, Ayse Karahasanoglu & Canan Ceylan To cite this article: Mucize Ozdemir, Isil Uzun, Ayse Karahasanoglu & Canan Ceylan (2016): A case of vulvar superficial angiomyxoma: A rare clinical entity, Journal of Obstetrics and Gynaecology, DOI: 10.3109/01443615.2016.1148674 To link to this article: http://dx.doi.org/10.3109/01443615.2016.1148674
Published online: 29 Feb 2016.
Submit your article to this journal
Article views: 4
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ijog20 Download by: [Purdue University Libraries]
Date: 17 March 2016, At: 19:54
Journal of Obstetrics and Gynaecology, 2016; Early Online: 1–2 ß 2016 Taylor & Francis ISSN 0144-3615 print/ISSN 1364-6893 online DOI: 10.3109/01443615.2016.1148674
CASE REPORT
A case of vulvar superficial angiomyxoma: A rare clinical entity Mucize Ozdemir1, Isil Uzun2, Ayse Karahasanoglu1 & Canan Ceylan3 Department of Obstetrics and Gynecology, Su¨leymaniye Education and Research Hospital, Istanbul, Turkey, 2Department of Obstetrics and Gynecology, Acibadem Bakirkoy Hospital, Istanbul, Turkey, and 3Department of Pathology, Istanbul University, Istanbul Medical School, Istanbul, Turkey
Downloaded by [Purdue University Libraries] at 19:54 17 March 2016
1
Introduction Superficial angiomyxomas are rare cutaneous tumours frequently involving the subcutis. It occurs most commonly in the fourth decade, presenting as a slowly growing painless mass that usually measures less than 50 mm. Superficial angiomyxomas are benign; however, it has a 30–40% rate of local non-destructive recurrence and it should be completely excised with a clear margin whenever possible (Calonje et al. 1999; Nucci and Fletcher 2000). Here we report a case of a 26 year-old female with a superficial angiomyxoma of the vulva.
Case report A 26 year-old woman, G2P2, was admitted to our clinic with a complaint of painless mass in the subcutaneous layer of the vulva between the right labium majus and the clitoris. The patient presented a history of vulvar mass excision two years ago but had no histopathologic report or medical documents. Local examination showed a well-circumscribed soft mass, measuring about 6 43 cm. The patient underwent local excision of the mass under general anaesthesia. The overlying skin had no ulcerative or erosive lesion (Figure 1). Microscopically, the tumour was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. These cells showed low cellularity with no atypia and mitosis. Variable-sized thin-walled capillaries and the scattered inflammatory cells, especially stromal neutrophils were seen in the stroma. Based on the pathologic and clinical features, superficial angiomyxoma was diagnosed.
Discussion Superficial angiomyxomas are more commonly located on extragenital sites, especially the head, neck and the trunk (Calonje et al. 1999; Nucci and Fletcher 2000). Genital Superficial Angiomyxomas usually involve the vulvovaginal region, almost always the vulva (Fetsch et al. 1997). Multiple cutaneous myxomas and angiomyxomas are occasionally features of Carney’s complex which is an autosomal dominant disorder, associated with cutaneous myxomas. But the association with Carney’s complex has not been shown in genital Superficial Angiomyxomas (Fetsch et al. 1997). Only 19 cases of
vulvar superficial angiomyxomas have been reported in the literature. Due to its rare occurrence, vulvar superficial angiomyxomas may be misdiagnosed as a polyp, Bartholin cyst, Gartner duct cyst or a perineal herniation in the physical examination. The diagnosis of vulvar superficial angiomyxoma is usually made by the pathologist (Kim et al. 2010). Superficial angiomyxomas are well-circumscribed, nodular or multinodular lesions, which frequently extend to subcutaneous fat. They are usually bigger than 5 cm in diameter. The cut surface is myxoid or gelatinous. There are multinucleated cells with few or no mitotic figures. Thin walled capillary-like blood vessels are present. Presence of stromal neutrophils is almost always invariable, which is a useful diagnostic aid. Superficial angiomyxomas are benign lesions with no metastatic potential; however, local non-destructive recurrence is not uncommon and the lesions should be excised with a clear resection margin (Bedlow et al. 1997; Calonje et al. 1999; Nucci and Fletcher 2000; Mc Cluggage 2004). The differential diagnosis of superficial angiomyxomas with aggressive angiomyxoma is very important. Aggressive angiomyxoma is a non-metastasizing, locally infiltrative tumour which occurs most commonly in the soft tissue of female pelvis and perineum. Aggressive angiomyxoma are large lesions usually greater than 10 cm in diameter (Dierickx et al. 2008). Typically aggressive angiomyxoma, like many of the other vulvovaginal mesenchymal lesions, is clinically thought to represent a cystic lesion, such as a Bartholin’s gland cyst. On gross examination, they are typically poorly circumscribed lesions with agelatinous, myxoid or fibrous consistency. Aggressive angiomyxomas are non-encapsulated tumours with an infiltrative edge and tendency to local recurrence. Histologically, the tumour consists of a hypocellular population of small neoplastic cells. Mitotic activity and nuclear atypia are absent. In general, this is a larger deeper-seated lesion and has an infiltrative margin that contrasts with the multi-nodular growth pattern of the superficial angiomyxoma. The blood vessels are more variable in aggressive angiomyxoma usually with a component thick walled vessel, whereas the presence of stromal neutrophils favours the superficial angiomyxoma. In contrast to aggressive angiomyxoma, the recurrence of superficial angiomyxoma is non-destructive and metastasis of superficial angiomyxoma has not been reported before (Steeper and Rosai 1983; Nucci and Fletcher 2000).
Correspondence: Isil Uzun, Yedikule Konaklari B4/D4 Fatih, Istanbul, Turkey. Tel: +90 5325141526. E-mail: [email protected]
2
M. Ozdemir et al. In summary, the authors described a very rare mesenchymal tumour arising in vulvar area. Vulvar superficial angiomyxomas are rare benign neoplasms that should be distinguished from other vulvar soft tissue tumours, especially aggressive angiomyxomas which have infiltrative nature and high risk of recurrence. Declaration of interest: All of the authors state that there are no conflicts of interest.
References
Downloaded by [Purdue University Libraries] at 19:54 17 March 2016
Figure 1. The operation.
Angiomyofibroblastoma is another mesenchymal tumour which occurs commonly on the vulva. It is a well-circumscribed lesion that clinically is often thought to represent a Bartholin’s gland cyst. Angiomyofibroblastomas usually measure less than 5 cm in diameter. Histologically, an angiomyofibroblastoma shows alternating hypocellular and hypercellular areas. Superficial angiomyxomas can be distinguished from angiomyofibroblastoma by characteristics of multinodular growth, sparse cellularity with abundant myxoid material (Mc Cluggage 2004; Nucci and Fletcher 2000). Overall angiomyofibroblastoma is a benign non-recurring lesion and local excision with clear margins is an adequate treatment. There is one report of Angiofibroblastoma with a sarcomatous transformation that recurred (Nielsen et al. 1997).
Bedlow AJ, Sampson SA, Holden CA. 1997. Congenital superficial angiomyxoma. Clinical and Experimental Dermatology 22:237. Calonje E, Guerin D, McCormick D, Fletcher CD. 1999. Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. The American Journal of Surgical Pathology 23:910–917. Dierickx I, Deraedt K, Poppe W, Verguts J. 2008. Aggressive angiomyxoma of the vulva: a case report and review of literature. Archives of Gynecology and Obstetrics 277:483. Fetsch JF, Laskin WB, Tavasolli FA. 1997. Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. International Journal of Gynecological Pathology 16:325–334. Kim HY, Kim GY, Lim SJ, Ki KY, Kim HC. 2010. Giant superficial angiomyxoma of the vulva: a case report and review of the literature. Journal of Cutaneous Pathology 37:672–677. McCluggage GW. 2004. A review and update of morphologically bland vulvovaginal mesenchymal lesions. International Journal of Gynecological Pathology 24:26–38. Nielsen GP, Young RH, Dickerson GR, Rosenberg AE. 1997. Angiomyofibroblastoma of the vulva with sarcomatous transformation. The American Journal of Surgical Pathology 21:1104–1108. Nucci MR, Fletcher CD. 2000. Vulvovaginal soft tissue tumours: update and review. Histopathology 36:97–108. Steeper TA, Rosai J. 1983. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic softtissue neoplasm. Perineum report of cases a type gynecologic tissue. The American Journal of Surgical Pathology 7:463–475.
