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| Year : 2016 | Volume
: 59
| Issue : 2 | Page : 257-259 |
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| Angiomyofibroblastoma of the right labia major |
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Ibrahim Gelincik1, Ayhan Yildirim2, Ilyas Sayar3, Cevat Aktas4
1 Department of Pathology, Namik Kemal University, Faculty of Medicine, Tekirdağ, Turkey
2 Department of Pathology, Diyarbakır Pediatric and Obstetrics Gynecology Hospital, Diyarbakır, Turkey
3 Department of Pathology, Erzincan University, Faculty of Medicine, Erzincan, Turkey
4 Department of Histology and Embryology, Namik Kemal University, Faculty of Medicine, Tekirdağ, Turkey
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| Date of Web Publication | 9-May-2016 |
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How to cite this article:
Gelincik I, Yildirim A, Sayar I, Aktas C. Angiomyofibroblastoma of the right labia major. Indian J Pathol Microbiol 2016;59:257-9 |
Editor,
Angiomyofibroblastoma (AMFB) of the vulva was first advocated by Fletcher et al. in 1992.[1] AMFB is a rare, benign mesenchymal tumor that occurs in the subcutaneous tissue of the vulva and vagina in women and the scrotum in men, and must be differentiated, especially from aggressive angiomyxoma because of its different biological behavior. Female patients are usually premenopausal and present with a vulval mass that is often diagnosed initially as a Bartholin's cyst. Histologically, the tumors are well-circumscribed and characterized by alternating hypo- and hypercellular areas with abundant thin-walled blood vessels. The tumor cells are bland and spindle-shaped or epitheloid and tend to concentrate around the vessels or cluster in small nests.[2] Immunoreactivity for both desmin and vimentin is detected in almost all tumor cells, which also reveal estrogen and progesterone receptors, but staining for cytokeratin is negative.[3] In this report, we describe a case of AMFB in a 41-year-old female patient. A 41-year-old female patient, single, presented with a painless mass of the right labia major, which has been noticed first by the patient 1 year previously. Examination revealed a well-circumscribed, mobile swelling located in the subcutaneous tissue of the right labia major. Ultrasonography demonstrated a soft tissue tumor with homogeneous echo and normal vascularity. The resected tumor had a well-circumscribed border brownish soft appearance measuring 3 2, 2 1, 8 cm. Its cut surface was solid, homogenous, myxoid-like with focal areas, and grayish in color [Figure 1]a. Microscopically, the mass consisted of spindled and plump oval stromal cells of fibroconnective tissue with edematous stroma and abundant vessels of various wall thickness [Figure 1]b. The tumor cells were focally clustered with an epithelioid appearance [Figure 1]c. No mitotic or atypical cells were seen. Immunohistochemically, the stromal cells showed strong positivity for vimentin and desin [Figure 2]a and [Figure 2]b, but were negative for CD34, CD31, factor Vlll, smooth muscle actin, S-100, and pan cytokeratin. İn addition to, the nucleus of the stromal cells showed positivity for estrogen and progesterone receptors. The final diagnosis was AMFB of the right labia major. This distinctive tumor was first delineated by Fletcher et al. in 1992, who described 10 cases involving the vulva [1] with only one case reporting a large pedunculated mass. There is a marked predilection for the female genital tract, predominantly the vulva, although rare cases have also been reported to arise in the scrotum and the inguinal area in males.[1] The patients ranged in age from 17 to 86 years (mean, 45 years). Our patient at 41-year-old is representative of this typical age range. They usually complain of a painless mass that has been present from a few weeks to up to 13 years.[4] Our patient presented with a painless mass of the right labia major, which has been noticed first by the patient 1 year previously. On gross examination, the tumors were well-circumscribed and ranged in size from 0.5 to 30 cm in maximal diameter (mean, 5.9 cm). They have a soft to rubbery consistency and a bulging, pink, and somewhat lobulated sectioned surface.[4] Our patient's tumor was a well-circumscribed border brownish soft appearance measuring 3 2, 2 1, 8 cm. The histogenesis of AMFB is unknown. It is believed that it originates from primitive mesenchymal cells. Microscopic examination confirms the well-demarcated nature of the lesion and shows alternating hypercellular and hypocellular edematous regions with abundant blood vessels. The cells tend to cluster around blood vessels, sometimes forming compact foci.[4] It is a slow growing tumor associated with the very low mitotic activity. There is minimal nuclear atypicality, and it can rarely undergo sarcomatous change.[5] Our patient was not seen mitotic or atypical cells. Although occasional cases recur locally, AMFBs generally behave in a benign fashion. The lesions can be treated easily by simple excision as our patient. The main differential diagnosis of AMFB is aggressive angiomyxoma, which is distinctive locally aggressive fibromyxoid tumor of the pelvic and genital soft tissues. In contrast to AMFB aggressive angiomyxoma is a deeply seated tumor with infiltrative growth pattern, which frequently results in entrapment of mucosal glands and nerves. Aggressive angiomyxoma is poorly circumscribed, and the stroma cells are a short spindle or stellate shaped within a loose myxoid matrix. The hyaluronic acid-rich stroma also contrasts with the absence of stromal mucin in AMFB. The key microscopic features are of aggressive angiomyxoma are medium- to large-sized vessels with a muscularized, thick, and hyalinized wall. Whereas, the blood vessels of AMFB are thin-walled, venular or capillary-sized. Our case was not observed any prominent hyalinization and hypertrophy in the vessel walls, or mucin in the stroma. In contrast, the blood vessels of our case are thin-walled, venular, or capillary-sized. The immunohistochemical profile of the AMFBs reported is almost uniformly positive for vimentin and frequently positive for desmin. The tumor cells demonstrate variable expression for smooth muscle actin and are frequently immunoreactive for progesterone and estrogen receptors, suggestive of the sex steroid dependency of this tumor. AMFBs are typically negative for CD34 but may demonstrate some reactivity about vessels, as the vessels are typically reactive.[6] İn our case, the stromal cells showed strong positivity for vimentin and desrnin, but the stromal cells were negative for CD34, CD31, factor Vlll, smooth muscle actin, S-100, and pan cytokeratin. İn addition to, nucleus of the stromal cells showed positivity for estrogen and progesterone receptors. AMFB do not infiltrate deep structures, and wide excision is recommended in all cases. | Figure 1: (a) Macroscopically, on cut section, the tumor is well circumscribed, solid, homogeneous, myxoid-like with focal areas (arrow), greyish in color. (b) Microscopically, stromal cells of fibroconnective tissue with edematous stroma and abundant vessels (H and E, ×200) and, (c) clustered of the tumor cells with epithelioid appearances (H and E, ×200)
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 | Figure 2: Immunohistochemical staining (a) diffuse positive reaction for vimentin (IHC, ×200) and, (b) diffuse positive reaction for desmin (IHC, ×200)
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Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Fletcher CD, Tsang WY, Fisher C, Lee KC, Chan JK. Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. Am J Surg Pathol 1992;16:373-82.  |
| 2. | Micheletti AM, Silva AC, Nascimento AG, Da Silva CS, Murta EF, Adad SJ. Cellular angiofibroma of the vulva: Case report with clinicopathological and immunohistochemistry study. Sao Paulo Med J 2005;123:250-2. |
| 3. | Horiguchi H, Matsui-Horiguchi M, Fujiwara M, Kaketa M, Kawano M, Ohtsubo-Shimoyamada R, et al. Angiomyofibroblastoma of the vulva: Report of a case with immunohistochemical and molecular analysis. Int J Gynecol Pathol 2003;22:277-84. |
| 4. | 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. |
| 5. | McCluggage WG. A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol 2005;24:26-38. |
| 6. | Kurman RJ, Ronnett BM, Sherman ME, Wilkinson EJ. Tumors of the Cervix, Vagina, and Vulva. AFIP Atlas of Tumor Pathology. Vol. 13. Washington, DC: American Registry of Pathology; 2010. p. 349-52. |
Correspondence Address:
Ibrahim Gelincik
Department of Pathology, Namik Kemal University, Faculty of Medicine, Tekirdağ
Turkey
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.182033
[Figure 1], [Figure 2] |
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