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 Table of Contents 
CASE REPORT
Year : 2012  |  Volume : 3  |  Issue : 1  |  Page : 47-49  

Aggressive angiomyxoma of the vulva


1 Department of Gynaecologic Oncology, Debabrata Barmon, Department of Gynaecologic Oncology, Dr. B. Borooah Cancer Institute (Regional Institute for Treatment and Research) Gopinath Nagar, Guwahati, Assam, India
2 Department of Pathology, Debabrata Barmon, Department of Gynaecologic Oncology, Dr. B. Borooah Cancer Institute (Regional Institute for Treatment and Research) Gopinath Nagar, Guwahati, Assam, India

Date of Web Publication20-Jul-2012

Correspondence Address:
Debabrata Barmon
Department of Gynaecologic Oncology, Dr. B. Borooah Cancer Institute (Regional Institute for Treatment & Research) Gopinath Nagar, Guwahati- 781 016, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-7800.98820

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   Abstract 

Aggressive angiomyxoma is a rare, benign neoplasm occurring in 3 rd to 5 th decade of life that can be mistaken both clinically and on microscopy for several other conditions, it should be included as a differential diagnosis for any vaginal mass. These lesions have a predilection for female pelvic soft tissues, slow in growth, and are characterized histologically by a predominantly myxoid stroma and an abundance of thin and thick walled vascular channels. This is a deep soft tissue tumor, which as the name suggests, may have a locally aggressive course. Most tumors occur in women and are large, usually greater than 10 cm, slowly growing, and painless. Standard of care treatment for angiomyxoma has been surgery. Some authors believe that it is the only possible treatment, but surgery is often radical and can be mutilating, with massive blood loss.

Keywords: Aggressive angiomyxoma, myxoma, myxoid neoplasm, female perineum


How to cite this article:
Barmon D, Kataki AC, Sharma J D, Bordoloi J. Aggressive angiomyxoma of the vulva. J Mid-life Health 2012;3:47-9

How to cite this URL:
Barmon D, Kataki AC, Sharma J D, Bordoloi J. Aggressive angiomyxoma of the vulva. J Mid-life Health [serial online] 2012 [cited 2019 Aug 21];3:47-9. Available from: http://www.jmidlifehealth.org/text.asp?2012/3/1/47/98820


   Introduction Top


Aggressive angiomyxoma was first reported as a distinct variant of myxoid neoplasms in the female vagina and pelvis by Steeper and Rosai. [1] These tumors mostly occurs in 3 rd to 5 th decade of life and 95% in females. These lesions have a predilection for female pelvic soft parts, slow growth, frequent recurrences, and are characterized histologically by a predominantly myxoid stroma and an abundance of thin and thick walled vascular channels. Many authors have subsequently reported this lesion in female and male patients. This is a deep soft tissue tumor, which as the name suggests, may have a locally aggressive course. Most tumors occur in women and are large, usually greater than 10 cm, slowly growing, and painless. The most common location is in the perineal region and may exert pressure on adjacent organs.


   Case Report Top


A 35 year old woman, Hosp. No. - A 8332 came to our OPD on 05.08.05 with chief complaints of growth in the vulva - 2 month, bleeding from the growth - 1 month, irregular period - 6 month. She is P4+1. She chews betel nut and leaf. There is no family history of malignancy. No history of HTN, DM, TB. Tubectomy was done a year ago.

On examination, GC is fair, pallor +, no palpable LN. P/A- no organomegaly, others NAD, groin- NAD. Local examination of the vulva revealed a 8 × 5 cms pedunculated growth [Figure 1] having its thick pedicle of about 1.5 cm thickness hanging from the clitoreal region slightly on the left side [Figure 2]. P/S/V- cervix- NAD, vaginal walls- NAD, Uterus - N/S, mobile, adnexae free. P/R- NAD.
Figure 1: Clinical presentation

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Figure 2: Growth coming out from the clitoreal region

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On investigation, Hb %- 9.8 gm%, ABO gr.- O +ve, RBS- 101 mg/dl, RFT-WNL, LFT-WNL.  Pap smear More Details-NAD, CXR-NAD, USG (W/A) - NAD. Pre operative biopsy revealed Aggressive Angiomyxoma.

She was planned for wide excision of the vulval growth; accordingly it was done under SA on 18.08.05 [Figure 3]. On sectioning, the mass had focally infiltrating margins and a rubbery glistening grey/white surface [Figure 4]. Post operative period was uneventful, and patient was discharged on 27.08.05.

Post op HPE revealed Aggressive Angiomyxoma [Figure 5] and [Figure 6], cut margins negative. IHC studies revealed Desmin and Vimentin +ve, ER, PR +ve and S100 protein negative. She is under regular follow-up, and doing well for the last 5 years.
Figure 3: After the repair

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Figure 4: Specimen cut open

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Figure 5: HPE shows thin epidermis and underlying hypo cellular myxoid tissue containing blood vessels of various calibers

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Figure 6: High power view showing hyaline thickening of blood vessels

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   Discussion Top


Aggressive angiomyxoma is a rare, benign neoplasm that can be mistaken both clinically and on microscopy for several other conditions, it should be included as a differential diagnosis for any vaginal mass. [2] It is important to diagnose this condition because the tumour is locally infiltrative and requires wide excision and follow up. It occurs mainly in the female pelvis, vulva, or perineum, though some cases are described in men. Standard of care treatment for angiomyxoma has been surgery. Some authors believe that it is the only possible treatment, but surgery is often radical and can be mutilating, with massive blood loss. It also requires postoperative intensive care monitoring, and the recurrence rate after surgery remains high even if negative margins are obtained at the initial resection. A retrospective review has shown that patients having positive margins were as likely to have recurrence as those with negative margins. [3]

Immunohistochemistry of the tumor cells revealed diffuse immunoreactivity for estrogen receptors, progesterone receptors, vimentin, and CD34. No expression of S-100 protein, Bcl-2 protein, CD117 (c-kit gene product), epithelial membrane antigen, desmin, or h-caldesmon could be demonstrated.

 
   References Top

1.Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm Am J Surg Pathol 1983;7:463-75.  Back to cited text no. 1
[PUBMED]    
2.Rai L, Nandyala V, Shetty J, Kumar V, Rao L. Aggressive angiomyxoma: An important differential diagnosis for a vaginal mass. Aust N Z J Obstet Gynaecol 2004;44:367-8.  Back to cited text no. 2
[PUBMED]    
3.Chan YM, Hon E, Ngai SW, Ng TY, Wong LC. Aggressive angiomyxoma in females: Is radical resection the only option? Acta Obstet Gynecol Scand 2000;79:216-20.  Back to cited text no. 3
[PUBMED]    


    Figures

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



 

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