|Year : 2015 | Volume
| Issue : 4 | Page : 937-939
Aggressive angiomyxoma of vulva in a 13-year-old female
Pooja Srivastava, Charanjeet Ahluwalia, Sufian Zaheer, Ashish Kumar Mandal
Department of Pathology, VMMC and Safdarjung Hospital, New Delhi, India
|Date of Web Publication||15-Feb-2016|
Department of Pathology, 270 A, Hauz Rani, Malviya Nagar, New Delhi - 110 017
Source of Support: None, Conflict of Interest: None
Aggressive angiomyxoma is a rare mesenchymal neoplasm that typically affects females of reproductive age. It involves preferentially pelvic and perineal regions and was first described by Steeper and Rosai in 1983. Peak age of incidence for this tumor is in the fourth decade with very few cases reported in young girls. We present a case of this rare tumor in a 13-year-old female.
Keywords: Aggressive, angiomyxoma, vulva
|How to cite this article:|
Srivastava P, Ahluwalia C, Zaheer S, Mandal AK. Aggressive angiomyxoma of vulva in a 13-year-old female
. J Can Res Ther 2015;11:937-9
| > Introduction|| |
Aggressive angiomyxoma is a rare mesenchymal neoplasm arising preferentially in pelvic and perineal regions of adults. It most commonly involves females of reproductive age group; however, cases in males have also been reported with female to male ratio being 6.6:1.  It was first described by Steeper and Rosai in 1983  and since then fewer than 350 cases have been reported in the literature.  It is a slow growing indolent neoplasm with a high propensity for local recurrence and a low tendency to metastasize.  The peak age of incidence is fourth decade of life with an age range of 11-77 years,  but very few cases have been reported in patients <15 years of age. We present a case of this rare tumor in a 13-year-old female.
| > Case report|| |
A 13-year-old female presented with complaints of slow growing mass involving left labia majora. The painless mass was gradually increasing in size for 3 months. She did not have any other constitutional symptoms. Her general and systemic examination was within normal limits. Local examination revealed a polypoidal mass arising from left labia majora. The mass was nontender, soft in consistency and the overlying skin appeared normal. An ultrasound was performed and suggested the possibility of a vascular lesion. The tumor was excised and sent for histopathological examination. On gross examination, skin covered the soft-tissue mass which measured 7.5 cm × 5 cm × 3 cm [Figure 1]. The overlying skin showed an ulcer measuring 4 cm × 1.5 cm in the dependent part. The cut surface was gray-white with a glistening, gelatinous appearance and areas of hemorrhage and congestion. Microscopy showed a sparsely cellular tumor having spindle and stellate shaped cells with eosinophilic cytoplasm lacking nuclear pleomorphism and mitosis [Figure 2]. The stroma was loose myxoid with wispy collagen fibrils [Figure 3]. Numerous blood vessels were seen ranging in size from thin-walled capillaries to thick walled vascular channels, some of which showed perivascular hyalinization [Figure 4]. Two of the resected margins were involved by the tumor. Tumor cells showed weak positivity for alcian blue. On immunohistochemistry, tumor cells were positive for vimentin, smooth muscle actin (SMA), desmin, estrogen receptor (ER) and were negative for cytokeratin (CK), S-100 and progesterone receptor (PR). Based on these findings, the tumor was determined to be an aggressive angiomyxoma.
|Figure 2: Sparsely cellular tumor having spindle and stellate shaped cells with eosinophilic cytoplasm and lacking nuclear pleomorphism (H and E, ×200)|
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|Figure 3: Stroma is loose and myxoid with wispy collagen fibrils at places (H and E, ×100)|
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|Figure 4: Numerous blood vessels were seen ranging in size from thin-walled capillaries to thick walled vascular channels (H and E, ×100)|
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| > Discussion|| |
Aggressive angiomyxoma is rare locally invasive mesenchymal neoplasms first described 30 years ago.  In 90% of cases, these tumors present in women of reproductive age with a peak incidence in the fourth decade of life.  These neoplasms are often clinically misdiagnosed as gartner duct cyst, vaginal polyps, myxoma, lipoma, Bartholin's cyst, pelvic floor hernia,  especially in young patients where these lesions are the most common differential diagnoses. The tumors are frequently misdiagnosed prior to surgery because they are rare, radiological findings are nonspecific, and they are subepithelial making office biopsy less advisable.  The differential diagnosis includes angiomyofibroblastoma, superficial angiomyxoma, fibroepithelial stromal polyps, myxoid lipomatous tumor and myxoid leiomyomas.  Aggressive angiomyxoma is the only myxoid tumor with a prominent thick and thin walled vascular component. Immunohistochemically, tumor cells stain for actin, desmin, vimentin, ER, PR but are negative for S-100, carcinoembryonic antigen and CK.  Our case also showed spindle to stellate shaped cells in the myxoid background with prominent vasculature and tumor cells were positive for vimentin, SMA, desmin, and ER. Pathogenesis of this tumor is not clear;  however, chromosomal translocation involving 12q13-15 leading to HMGA2 gene rearrangement has been found in at least some of these tumors. 
The surgical treatment is wide local excision with tumor-free margins,  but negative margins are difficult to obtain because the tumor is locally invasive and has the same consistency as the surrounding connective tissue. Interestingly, in a review of over 100 cases of angiomyxoma, the chance of remaining disease-free with complete resection was not statistically different from those with incomplete resection (50% vs. 40% in 10 years).  Radical resection may be considered, but fertility concerns or the anticipation of significant operative morbidity may influence patient decisions.  Alternative treatment options include hormone manipulation as tumor cells are usually estrogen and PR-positive.  Hormonal therapy with tamoxifen, raloxifene or gonadotropin-releasing hormone analogues has been shown to reduce tumor size and help to achieve near complete resection in large tumors. Hormone therapy can also be used to treat tumor recurrence.  Radiotherapy and chemotherapy are considered less suitable due to the low mitotic activity of this tumor. Han-Guerts et al. propose the following guideline in treating aggressive angiomyxoma: (1) Complete excision of the lesion when possible and avoiding mutilating surgery, (2) adjunct therapy when partial resection is performed is acceptable using arterial embolization and/or hormonal treatment, and (3) radiotherapy is reserved to cases that are resistant to embolization and/or hormonal therapy and still symptomatic.  Recurrence rates from the largest case series range from 25% to 47%, with 85% of these occurring within 5 years of initial surgery.  There are no definite guidelines for postoperative management of vulvar aggressive angiomyxoma but due to high propensity for local recurrence even many years after primary disease; several authors have recommended periodic checkup with physical examination and magnetic resonance imaging up to 15 years after treatment. 
| > Conclusion|| |
The diagnosis of aggressive angiomyxoma should be considered in all reproductive age females presenting with slow growing vulval swellings. Tumor recurrence is very common and usually late after primary surgery, so long-term surveillance is a necessity.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]