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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 36  |  Issue : 2  |  Page : 308-311

Angiomyofibroblastoma: Imaging and histopathology of a rare benign mesenchymal tumor


Department of Radiodiagnosis and Imaging, Tapadia Diagnostic Centre, Aurangabad, Maharashtra, India

Date of Web Publication16-Sep-2019

Correspondence Address:
Dr. Mohammed Ashfaque Tinmaswala
Department of Radiodiagnosis and Imaging, Tapadia Diagnostic Centre, Aurangabad, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TJOG.TJOG_79_17

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  Abstract 


Angiomyofibroblastomas, aggressive angiomyxomas and cellular angiofibromas are rare mesenchymal tumours with many overlapping radiological, histopathological and immunohistochemical features. Amongst these tumours angiomyofibroblastoma is relatively benign mesenchymal tumour with very low chances of recurrence. It is clinically confused with bartholin gland cyst due to its well demarcated and smooth appearance. Due to overlapping histopathological features its very difficult even for experienced pathologists to differentiate between these mesenchymal tumours. Earlier desmin reactivity was thought to be specific for angiomyofibroblastoma but recently many aggressive angiomyxomas have also been found to be positive for desmin. Ultrasound, computed tomography and magnetic resonance imaging may be useful in diagnosis and ruling out more sinister malignancies. A well demarcated lesion with characteristic histopathological appearance of alternating hypo and hypercellular edematous regions with abundant blood vessels and stromal cells with dispersed chromatin is usually seen in angiomyofibroblastoma. Immunohistochemistry may further help in diagnosis. We here report a case of vaginal angiomyofibroblastoma. The diagnosis was made on the basis of imaging and was confirmed by histopathology and immunohistochemistry.

Keywords: Histopathology and immunohistochemistry; imaging; mesenchymal tumours; vaginal angiomyofibroblastoma.


How to cite this article:
Kulkarni A R, Tinmaswala MA, Shetkar S V, Kondekar S. Angiomyofibroblastoma: Imaging and histopathology of a rare benign mesenchymal tumor. Trop J Obstet Gynaecol 2019;36:308-11

How to cite this URL:
Kulkarni A R, Tinmaswala MA, Shetkar S V, Kondekar S. Angiomyofibroblastoma: Imaging and histopathology of a rare benign mesenchymal tumor. Trop J Obstet Gynaecol [serial online] 2019 [cited 2019 Dec 15];36:308-11. Available from: http://www.tjogonline.com/text.asp?2019/36/2/308/266874




  Introduction Top


Angiomyofibroblastomas, cellular angiofibromas, and angiomyxomas are some of the rare mesenchymal tumors usually arising from genital tract of middle-aged females.[1] There is considerable histopathological and immunohistochemical overlap between these tumors and diagnosis of these lesion; apart from histopathology and immunohistochemistry, it depends on close morphological examination.[2] Angiomyofibroblastoma is a benign mesenchymal tumor first described by Fletcher et al. in 1992.[3] Histologically, it consists of vascular and stromal components and usually arises from vulva, vagina, perineum, and uterine cervix. In men, these tumors may involve inguinoscrotal region.[4] Unlike aggressive angiomyxoma and cellular angiofibroma, it is a less likely to recur after complete excision.[5] We report here a case of a 35-year-old female presenting with swelling involving vagina. Imaging by ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) was done which showed features suggestive of mesenchymal tumor. Tumor was excised laparoscopically with a rim of healthy tissue. Gross appearance, imaging, histopathology, immunohistochemistry (reactive to desmin), and estrogen and progesterone receptor positivity proved it to be angiomyofibroblastoma.


  Case Report Top


A 35-year-old female patient presented with complaints of sensation of heaviness and mass coming out of vagina since 6 months. There was history of this swelling being painless and gradually increasing in size. On per speculum examination, there was evidence of a globular mass. The tumor was smooth, pinkish white, and compressible. There was no visible ulceration or bleeding over its surface [Figure 1].
Figure 1: Per speculum examination showing paravaginal swelling which is grossly smooth without any evidence of ulceration or bleeding

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Ultrasound showed an isoechoic mass lesion arising from right vaginal wall. On color Doppler imaging, there was evidence of vascular channels traversing the mass lesion [Figure 2] and [Figure 3]. There was no abdominal or pelvic lymphadenopathy on ultrasound examination.
Figure 2: Transperineal ultrasound showing isoechoic mass lesion arising from right vaginal wall near anal sphincter (arrow)

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Figure 3: Transvaginal ultrasound showing well-defined homogeneous lesion with traversing vascular channels

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Contrast-enhanced CT showed inhomogeneous mass in pouch of Douglas. This mass was seen arising out of right vaginal wall and was causing displacement of rectum. At the level of perineum, this mass was at right paravaginal region and was seen extending into gluteal fat [Figure 4], [Figure 5], [Figure 6].
Figure 4: Contrast-enhanced CT: Axial image showing inhomogeneous mass in POD causing rectal displacement to right

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Figure 5: At the level of perineum, an enhancing mass is seen at right paravaginal region with extension into gluteal fat

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Figure 6: Contrast-enhanced CT right parasagittal image showing polypoidal soft tissue density mass with few enhancing components

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An MRI was also done which showed hyperintense polypoidal lesion arising out of right vaginal wall. This lesion was seen protruding into presacral space [Figure 7].
Figure 7: Coronal MRI T1 (left) and T2 (right)-weighted images showing polypoidal mass arising from right vaginal wall and showing signal intensity similar to muscles with few areas of flow void and necrosis

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Based on imaging features, the differential diagnoses considered were cellular angiofibroma, angimyxoma, and angiomyofibroblastoma. The patient was operated by laparoscopic excision of the mass with a rim of healthy tissue.

The histopathological examination revealed the lesion to be consisting of blood vessels and stromal cells with dispersed chromatin [Figure 8].
Figure 8: Histopathological examination showed spindle cells in fascicles interspersed with number of thin walled blood vessels. Individual cells were elongated with vesicular nucleus and dispersed chromatin. No significant mitosis was seen

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Immunohistochemistry revealed it to be strongly positive for estrogen and progesterone receptors. The lesion was found to be immune-reactive for desmin. Well-demarcated lesion, histopathological examination, and desmin positivity of the lesion without any evidence of mitosis favored diagnosis of angiomyofibroblastoma. Post surgery, the patient is disease-free since 8 months.


  Discussion Top


Fletcher et al. described 10 cases of benign tumors of vulva which mimicked aggressive angiomyxoma.[3] On gross examination, these tumors are typically well circumscribed, with rubbery consistency and pink in color. On ultrasound, these tumors usually present as mixed echoic soft tissue tumors sometimes containing cystic spaces within the mass. The correlation of ultrasound findings with pathological findings suggests that echogenic areas correspond to hypocellular myxoid stroma, hypercellularity presents as hypoechogenicity on ultrasound, and cystic spaces correspond to dilated glands in vulva. On, CT angiomyofibroblastoma may present as hypoattenuating mass with inhomogeneous central enhancement. Hypoenhancement most likely corresponds to hypocellular myxoid stroma and inhomogeneous enhancement may be related to hypercellular zones.[6] On MRI, these tumors appear isointense to skeletal muscles on T1- and hyperintense on T2-weighted images. Postcontrast images may show strong enhancement.[7] These imaging features are not exclusive to angiomyofibroblastoma, and it is important to distinguish this rare tumor from other equally rare mesenchymal tumors such as aggressive angiomyxomas and cellular angiofibromas.[8]

The histopathological examination of angiomyofibroblastoma may show well-demarkated lesion with alternating hypo- and hypercellular edematous regions with abundant blood vessels. Tumor cells tend to cluster around blood vessels and there is presence of epitheloid elements in angiomyofibroblastoma.[9] Immunohistochemical examination of these tumors show them to be positive for desmin. In fact, some authors have previously concluded that desmin can be used to differentiate between cellular angiofibromas and angiomyofibroblastomas.[10] But many cases of aggressive angiomyxomas have also been reported to be desmin positive, hence histopathological and immunohistochemical diagnosis of angiomyofibroblastoma even today remains fairly complicated. Complicated as it may be, it is of utmost clinical importance to differentiate between angiomyofibroblastoma and other mesenchymal tumors such as aggressive angiomyxomas and cellular angifibromas because angiomyfibroblastoma runs a benign course and is less likely to recur than more aggressive lesions such as aggressive angiomyxoma.


  Conclusion Top


Angiomyofibroblastoma is a rare mesenchymal tumor which usually arises from female genital tract. It needs to be differentiated from other equally rare but more aggressive mesenchymal tumors such as cellular angiofibroma and aggressive angiomyxoma. A well-demarcated lesion with characteristic radiological appearance, having immunoreactivity to desmin and estrogen and progesterone receptor positivity is more likely to be angiomyofibroblastoma than cellular angiofibroma or aggressive angiomyxoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

The authors thank the pathologists who kindly contributed to histopathological and immunohistochemical examination: Dr. Pratap Patil, deputy laboratory director, PathCare Labs Pvt Ltd, Hyderabad.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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.  Back to cited text no. 1
    
2.
McCluggage WG, Perenyei M, Irwin ST. Recurrent cellular angiofibroma of the vulva. J Clin Pathol 2002;55:477-9.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
    
4.
Madrigal B, Veiga M, Vara A, Pérez del Río MJ, González Carril F, Fresno MF, et al. An aggressive inguinal (parafunicular) angiomyxoma in a male patient. Arch Esp Urol 1999;52:785-8.  Back to cited text no. 4
    
5.
Saleh MM, Yassin AH, Zaklama MS. Recurrent angiomyofibroblastoma of the vagina: A case report. Eur J Gynaecol Oncol 2007;28:324.  Back to cited text no. 5
    
6.
Kim SW, Lee JH, Han JK, Jeon S. Angiomyofibroblastoma of the vulva: Sonographic and computed tomographic findings with pathologic correlation. J Ultrasound Med 2009;28:1417-20.  Back to cited text no. 6
    
7.
Geng J, Hu S, Wang F. Large paravaginal angiomyofibroblastoma: Magnetic resonance imaging findings. Jpn J Radiol 2011;29:152-5.  Back to cited text no. 7
    
8.
Koo PJ, Goykhman I, Lembert L, Nunes LW. MRI features of cellularangiomyofibroma with pathologic correlation. J Magn Reson Imaging 2009;29:1195-8.  Back to cited text no. 8
    
9.
Hisaoka M, Kouho H, Aoki T, Daimaru Y, Hashimoto H. Angiomyofibroblastoma of the vulva: A clinicopathologic study of seven cases. Pathol Int 1995;45:487-92.  Back to cited text no. 9
    
10.
Colombat M, Liard-Meillon ME, De Saint-Maur P, Sevestre H, Gontier MF. Cellular angiofibroma. A rare vulvar tumor. Report of a case. Ann Pathol 2001;21:145-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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