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Vulvar cellular angiofibroma: A case report
American Journal of Obstetrics and Gynecology (2005) 193, 1750–2
www.ajog.org
Vulvar cellular angiofibroma: A case report Ryan Kerkuta, MD*, Colleen M. Kennedy, MD, MS, Jo A. Benda, MD, Rudolph P. Galask, MD, MS Departments of Obstetrics and Gynecology, and Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA Received for publication April 29, 2005; revised June 28, 2005; accepted August 8, 2005
KEY WORDS Vulva Cellular angiofibroma
Cellular angiofibroma is a benign growth initially described in 1997, with few reports to date. A 31-year-old woman presented with a 3-year history of a small left labial mass, which had recently increased in size to 5 cm, and was clinically thought to be a lipoma. A simple excision was performed. Histologically, the mass was consistent with a cellular angiofibroma. Ten months later, the growth has not recurred. Cellular angiofibroma is a rare, benign mesenchymal lesion typically occurring on the vulva, and should be considered in the differential diagnosis of a painless, soft, vulvar mass. Ó 2005 Mosby, Inc. All rights reserved.
Cellular angiofibroma of the vulva is a rare benign growth of mesenchymal origin that was initially described by Nucci in 1997.1 There is little information about follow-up after excision. Previously described as a lesion of middle-aged women, we describe a case of a vulvar cellular angiofibroma that occurred in a 31-year-old woman.
Case report A 31-year-old parous, white woman was referred for further management of an enlarging, nontender vulvar
The study was funded in part by the NICHD 1K23 HD045769-01 as part of a K23 Career Development Award (C.M.K.). The sponsor of the study had no role in study design, data collection, data analysis, data interpretation, or in the writing of the report. Reprints not available from the authors. * Anticipated graduation from the University of Iowa, Roy J. and Lucielle A. Carver College of Medicine in May 2006. 0002-9378/$ - see front matter Ó 2005 Mosby, Inc. All rights reserved. doi:10.1016/j.ajog.2005.08.021
mass. The patient had first noticed a ‘‘walnut-sized’’ left labial growth about 3 years earlier and ultimately sought medical attention from her local physician when it enlarged to ‘‘lemon sized.’’ The physician referred the patient to a tertiary care center after attempted drainage of the mass was unsuccessful. The patient had genital warts excised 15 years earlier and had no other history of sexually transmitted diseases. She used low-dose oral contraceptive pills and denied tobacco, alcohol, or illicit drug use. She had no personal or family history of breast or gynecologic cancer. Regular Papanicolaou test smears were all normal. Examination revealed a 5-cm, soft, mobile, nontender mass involving the left labia majora (Figure 1). Clinically, the mass resembled a lipoma. Small bowel was not palpable in the mass. Groin lymph nodes were not enlarged on palpation. The remainder of the pelvic examination was otherwise unremarkable. She underwent an uncomplicated simple resection of the mass in the operating room. Similar to lipoma
Kerkuta et al
1751
Figure 2 Bland ovoid to spindled nuclei and absence of mitotic figures is present in this high-power view (original magnification: !60).
Figure 1
Vulvar mass.
excisions, the pseudocapsule was easily enucleated after the initial skin incision. The patient’s postoperative course was uncomplicated. At the time of follow-up 10 months later, there was no sign of recurrence.
Pathology On gross examination, the mass was 5 ! 4 ! 3 cm, uniform, and fibrous with no fluid accumulation or cystic spaces. Microscopically, spindle- and oval-shaped stromal cells with bland nuclei and minimal mitotic activity were present. These were interspersed with thin bands of collagen fibers and rare adipocytes. The mass contained numerous small vessels and a focal perivascular lymphoid infiltrate. Mast cells were present (Figure 2). Immunohistochemical staining was positive for vimentin, estrogen receptor, and progesterone receptor, and negative for CD34, smooth muscle specific actin, desmin, muscle specific actin, and S-100. These findings are consistent with the diagnosis of cellular angiofibroma.
Comment Mesenchymal tumors of the vulva include leiomyoma, hemangioma, and lipoma. Spindle cell tumors include neurofibroma, schwannoma, and smooth muscle sarcoma. More unusually encountered masses include angiomyofibroblastoma, angiomyxoma, and cellular angiofibroma.1-3 The distinction of cellular angiofibroma from these lesions is morphologic, although immunohistochemical
studies are typically performed to confirm the diagnosis.4 Although cellular angiofibromas are benign mesenchymal tumors, exclusion of other vulvovaginal soft tissue tumors, including aggressive angiomyxoma and sarcoma, is essential to avoid unnecessary aggressive treatment.3,5,6 Clinically, an inguinal hernia should also be considered in the differential diagnosis of a vulvar mass, as the approach to a hernia repair differs from excising a simple mass. Cellular angiofibroma has been identified in middleaged women with a mean age of 48 (range 37-77) years.1,3,5 Clinically, cellular angiofibroma is often mistaken for a Bartholin gland, labial, or submucosal cyst.1,3 Although local excision with clear margins is the treatment of choice, there is little information about the long-term follow-up of this tumor.6 No cases with metastasis are reported in the literature.4 However, there is 1 report of tumor recurrence, in which a 49-year-old woman underwent a simple excision of a well-circumscribed 4-cm lesion and had recurrent swelling develop at the site of the previous excision 6 months later.4 A 6.5-cm well-circumscribed mass was excised without complication, with no further evidence of recurrence noted at 10 months. Both lesions were consistent with cellular angiofibroma. Although similar lesions have been reported outside the vulva,4,7 cellular angiofibroma should not be mistaken for a nasopharyngeal angiofibroma, a polypoid intranasal growth found typically in adolescent boys.7 This lesion is life threatening and is characterized by a parallel arrangement of the collagen fibers. Characteristic features of cellular angiofibroma are small lesions (typically less than 3.0 cm) with wellcircumscribed margins, although extension into surrounding tissue was described in 1 case in which a
1752 46-year-old woman underwent initial enucleation, followed by 2 re-excisions and had no sign of recurrence at 19 months.1 The cells are spindle shaped, lying between bands of collagen. Hyalinized vessels, abundant mast cells, and scant adipocytes may be present. Few mitotic figures are identified. Cellular pleomorphism is rare, and tumor necrosis has not been described. Immunohistochemically, cellular angiofibromas are positive for vimentin and may express reactivity for CD34, whereas desmin, actin, S-100 protein, keratin, and epithelial membrane antigen are negative.4,6,8,9 In addition, the tumor has been found to be estrogen and progesterone receptor positive.3-5 However, the significance of the positive estrogen and progesterone receptors in angiofibroma is unknown. These receptors are normal in subepithelial mesenchymal cells of the lower female genital tract, so this may be a reflection of the cell of origin rather than an alteration in a neoplasm.4 Alternatively, it has been postulated that the expression of estrogen and progesterone receptors suggests a role in the pathogenesis of this tumor.3 The patient presented in this report was younger and her mass larger than most others reported in the literature. Thus, cellular angiofibroma should be considered in the differential diagnosis of a soft, painless, vulvar mass even in younger women.
Kerkuta et al
Acknowledgment We thank Ingrid E. Nygaard, MD, MS, for her editorial assistance with the manuscript.
References 1. 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. 2. Curry JL, Olejnik JL, Wojcik EM. Cellular angiofibroma of the vulva with DNA ploidy analysis. Int J Gynecol Pathol 2001;20:200-3. 3. Dargent J-L, de Saint Aubain N, Galdon MG, Valaeys V, Cornut P, Noel J-C. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature. J Cutan Pathol 2003;30:405-11. 4. McCluggage WG, Perenyei M, Irwin ST. Recurrent cellular angiofibroma of the vulva. J Clin Pathol 2002;55:477-80. 5. Lane JE, Walker AN, Mullis EN, Etheridge JG. Cellular angiofibroma of the vulva. Gynecol Oncol 2001;81:326-9. 6. Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumours: update and review. Histopathology 2000;36:97-108. 7. Garijo MF, Val-Bernal JF. Extravulvar subcutaneous cellular angiofibroma. J Cutan Pathol 1998;25:327-32. 8. Dikmen Y, Yucebilgin MS, Kazandi M, Zekioglu O, Akalin T, Ozdemir N. Cellular angiofibroma of the vulva: report of a case. Eur J Gynaecol Oncol 2004;25:242-4. 9. 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.
www.ajog.org
Vulvar cellular angiofibroma: A case report Ryan Kerkuta, MD*, Colleen M. Kennedy, MD, MS, Jo A. Benda, MD, Rudolph P. Galask, MD, MS Departments of Obstetrics and Gynecology, and Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA Received for publication April 29, 2005; revised June 28, 2005; accepted August 8, 2005
KEY WORDS Vulva Cellular angiofibroma
Cellular angiofibroma is a benign growth initially described in 1997, with few reports to date. A 31-year-old woman presented with a 3-year history of a small left labial mass, which had recently increased in size to 5 cm, and was clinically thought to be a lipoma. A simple excision was performed. Histologically, the mass was consistent with a cellular angiofibroma. Ten months later, the growth has not recurred. Cellular angiofibroma is a rare, benign mesenchymal lesion typically occurring on the vulva, and should be considered in the differential diagnosis of a painless, soft, vulvar mass. Ó 2005 Mosby, Inc. All rights reserved.
Cellular angiofibroma of the vulva is a rare benign growth of mesenchymal origin that was initially described by Nucci in 1997.1 There is little information about follow-up after excision. Previously described as a lesion of middle-aged women, we describe a case of a vulvar cellular angiofibroma that occurred in a 31-year-old woman.
Case report A 31-year-old parous, white woman was referred for further management of an enlarging, nontender vulvar
The study was funded in part by the NICHD 1K23 HD045769-01 as part of a K23 Career Development Award (C.M.K.). The sponsor of the study had no role in study design, data collection, data analysis, data interpretation, or in the writing of the report. Reprints not available from the authors. * Anticipated graduation from the University of Iowa, Roy J. and Lucielle A. Carver College of Medicine in May 2006. 0002-9378/$ - see front matter Ó 2005 Mosby, Inc. All rights reserved. doi:10.1016/j.ajog.2005.08.021
mass. The patient had first noticed a ‘‘walnut-sized’’ left labial growth about 3 years earlier and ultimately sought medical attention from her local physician when it enlarged to ‘‘lemon sized.’’ The physician referred the patient to a tertiary care center after attempted drainage of the mass was unsuccessful. The patient had genital warts excised 15 years earlier and had no other history of sexually transmitted diseases. She used low-dose oral contraceptive pills and denied tobacco, alcohol, or illicit drug use. She had no personal or family history of breast or gynecologic cancer. Regular Papanicolaou test smears were all normal. Examination revealed a 5-cm, soft, mobile, nontender mass involving the left labia majora (Figure 1). Clinically, the mass resembled a lipoma. Small bowel was not palpable in the mass. Groin lymph nodes were not enlarged on palpation. The remainder of the pelvic examination was otherwise unremarkable. She underwent an uncomplicated simple resection of the mass in the operating room. Similar to lipoma
Kerkuta et al
1751
Figure 2 Bland ovoid to spindled nuclei and absence of mitotic figures is present in this high-power view (original magnification: !60).
Figure 1
Vulvar mass.
excisions, the pseudocapsule was easily enucleated after the initial skin incision. The patient’s postoperative course was uncomplicated. At the time of follow-up 10 months later, there was no sign of recurrence.
Pathology On gross examination, the mass was 5 ! 4 ! 3 cm, uniform, and fibrous with no fluid accumulation or cystic spaces. Microscopically, spindle- and oval-shaped stromal cells with bland nuclei and minimal mitotic activity were present. These were interspersed with thin bands of collagen fibers and rare adipocytes. The mass contained numerous small vessels and a focal perivascular lymphoid infiltrate. Mast cells were present (Figure 2). Immunohistochemical staining was positive for vimentin, estrogen receptor, and progesterone receptor, and negative for CD34, smooth muscle specific actin, desmin, muscle specific actin, and S-100. These findings are consistent with the diagnosis of cellular angiofibroma.
Comment Mesenchymal tumors of the vulva include leiomyoma, hemangioma, and lipoma. Spindle cell tumors include neurofibroma, schwannoma, and smooth muscle sarcoma. More unusually encountered masses include angiomyofibroblastoma, angiomyxoma, and cellular angiofibroma.1-3 The distinction of cellular angiofibroma from these lesions is morphologic, although immunohistochemical
studies are typically performed to confirm the diagnosis.4 Although cellular angiofibromas are benign mesenchymal tumors, exclusion of other vulvovaginal soft tissue tumors, including aggressive angiomyxoma and sarcoma, is essential to avoid unnecessary aggressive treatment.3,5,6 Clinically, an inguinal hernia should also be considered in the differential diagnosis of a vulvar mass, as the approach to a hernia repair differs from excising a simple mass. Cellular angiofibroma has been identified in middleaged women with a mean age of 48 (range 37-77) years.1,3,5 Clinically, cellular angiofibroma is often mistaken for a Bartholin gland, labial, or submucosal cyst.1,3 Although local excision with clear margins is the treatment of choice, there is little information about the long-term follow-up of this tumor.6 No cases with metastasis are reported in the literature.4 However, there is 1 report of tumor recurrence, in which a 49-year-old woman underwent a simple excision of a well-circumscribed 4-cm lesion and had recurrent swelling develop at the site of the previous excision 6 months later.4 A 6.5-cm well-circumscribed mass was excised without complication, with no further evidence of recurrence noted at 10 months. Both lesions were consistent with cellular angiofibroma. Although similar lesions have been reported outside the vulva,4,7 cellular angiofibroma should not be mistaken for a nasopharyngeal angiofibroma, a polypoid intranasal growth found typically in adolescent boys.7 This lesion is life threatening and is characterized by a parallel arrangement of the collagen fibers. Characteristic features of cellular angiofibroma are small lesions (typically less than 3.0 cm) with wellcircumscribed margins, although extension into surrounding tissue was described in 1 case in which a
1752 46-year-old woman underwent initial enucleation, followed by 2 re-excisions and had no sign of recurrence at 19 months.1 The cells are spindle shaped, lying between bands of collagen. Hyalinized vessels, abundant mast cells, and scant adipocytes may be present. Few mitotic figures are identified. Cellular pleomorphism is rare, and tumor necrosis has not been described. Immunohistochemically, cellular angiofibromas are positive for vimentin and may express reactivity for CD34, whereas desmin, actin, S-100 protein, keratin, and epithelial membrane antigen are negative.4,6,8,9 In addition, the tumor has been found to be estrogen and progesterone receptor positive.3-5 However, the significance of the positive estrogen and progesterone receptors in angiofibroma is unknown. These receptors are normal in subepithelial mesenchymal cells of the lower female genital tract, so this may be a reflection of the cell of origin rather than an alteration in a neoplasm.4 Alternatively, it has been postulated that the expression of estrogen and progesterone receptors suggests a role in the pathogenesis of this tumor.3 The patient presented in this report was younger and her mass larger than most others reported in the literature. Thus, cellular angiofibroma should be considered in the differential diagnosis of a soft, painless, vulvar mass even in younger women.
Kerkuta et al
Acknowledgment We thank Ingrid E. Nygaard, MD, MS, for her editorial assistance with the manuscript.
References 1. 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. 2. Curry JL, Olejnik JL, Wojcik EM. Cellular angiofibroma of the vulva with DNA ploidy analysis. Int J Gynecol Pathol 2001;20:200-3. 3. Dargent J-L, de Saint Aubain N, Galdon MG, Valaeys V, Cornut P, Noel J-C. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature. J Cutan Pathol 2003;30:405-11. 4. McCluggage WG, Perenyei M, Irwin ST. Recurrent cellular angiofibroma of the vulva. J Clin Pathol 2002;55:477-80. 5. Lane JE, Walker AN, Mullis EN, Etheridge JG. Cellular angiofibroma of the vulva. Gynecol Oncol 2001;81:326-9. 6. Nucci MR, Fletcher CDM. Vulvovaginal soft tissue tumours: update and review. Histopathology 2000;36:97-108. 7. Garijo MF, Val-Bernal JF. Extravulvar subcutaneous cellular angiofibroma. J Cutan Pathol 1998;25:327-32. 8. Dikmen Y, Yucebilgin MS, Kazandi M, Zekioglu O, Akalin T, Ozdemir N. Cellular angiofibroma of the vulva: report of a case. Eur J Gynaecol Oncol 2004;25:242-4. 9. 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.