|Year : 2015 | Volume
| Issue : 1 | Page : 88-90
Aggressive angiomyxoma of vulva: A case report and review of literature
Bhakti Dattatraya Deshmukh, Medha P Kulkarni, Yasmin A Momin, Kalpana R Sulhyan
Department of Pathology, Government Medical College, Miraj, Maharashtra, India
|Date of Web Publication||13-Apr-2015|
Dr. Bhakti Dattatraya Deshmukh
Department of Pathology, Government Medical College, Pandharpur road, Miraj - 416 410, Maharashtra
Source of Support: None, Conflict of Interest: None
Aggressive angiomyxoma is a distinctive, locally aggressive, mesenchymal tumor that preferentially involves the pelvic and perineal regions of females with a peak incidence in the 4 th decade of life. The neoplastic cells of aggressive angiomyxoma exhibit fibroblastic and myofibroblastic features and appear to be hormonally influenced. There is a strong propensity for local recurrence. The recurrence may be seen years after the initial resection; hence, long-term follow-up is required. We report a case of a 22-year-old woman with a huge pedunculated aggressive angiomyxoma arising from right labia majora.
Keywords: Aggressive, angiomyxoma, vulva
|How to cite this article:|
Deshmukh BD, Kulkarni MP, Momin YA, Sulhyan KR. Aggressive angiomyxoma of vulva: A case report and review of literature. Arch Med Health Sci 2015;3:88-90
|How to cite this URL:|
Deshmukh BD, Kulkarni MP, Momin YA, Sulhyan KR. Aggressive angiomyxoma of vulva: A case report and review of literature. Arch Med Health Sci [serial online] 2015 [cited 2019 Jun 25];3:88-90. Available from: http://www.amhsjournal.org/text.asp?2015/3/1/88/154952
| Introduction|| |
Aggressive angiomyxoma (AA) is a rare myxoid mesenchymal tumor of the genital, perineal, and pelvic region, occurring in adult women. Steeper and Rosai first described AA in 1983 as a rare, slow-growing locally infiltrative neoplasm.  Histology is characterized by a hypocellular tumor with myxoid stroma and an abundance of thin- and thick-walled vascular channels. The tumor cells are positive for desmin, smooth muscle actin, vimentin, and oestrogen and progesterone receptor. Some tumors are positive for CD34, whereas S100 is invariably negative. Few tumors show a non-random involvement of chromosomal band 12q15. Primary surgical resection is the treatment of choice, although treatment with gonadotropin releasing hormone agonists is an emerging therapy.
| Case Report|| |
A 22-year-old woman presented with a gradually increasing huge pedunculated mass arising from right labia majora [Figure 1] since 2 years. The clinical diagnosis was lipoma. Surgical excision was done. We received skin covered pedunculated mass measuring 8 × 8 × 5 cm. The overlying skin showed multiple ulcers. The cut surface was gelatinous, myxoid [Figure 2]. Microscopy showed a hypocellular tumor comprised scattered spindle to stellate-shaped cells having bland nuclei with ill-defined cytoplasm and numerous haphazardly arranged variably sized blood vessels set in abundant myxoid stroma [Figure 3] and [Figure 4]. No nuclear atypia or mitotic figures were noted. The diagnosis of aggressive angiomyxoma was rendered.
|Figure 3: Photomicrograph of the tumor showing variably sized blood vessels set in an abundant myxoid stroma (H and E, ×100)|
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|Figure 4: Photomicrograph of the tumor showing spindle to stellate cells (H and E, ×100)|
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| Discussion|| |
AA is most often found in the lower pelvis, more specifically the genital, perineal, and pelvic region.  In men, it occurs over a wide variety of sites such as perineum, scrotum, spermatic cord, and inguinal region simulating inguinal hernia, testicular/ spermatic cord neoplasm, hydrocele, or spermatocele.  The female: Male ratio is 6:1.  The peak incidence is during the 4 th decade. Tumors occurring during pregnancy have a rapid growth owing to increased estrogen and progesterone production during this period. 
The patients present with a slow-growing mass, accompanied by regional pain, a feeling of local pressure, or dyspareunia. AA is often clinically misdiagnosed as Bartholin's cyst, labial cyst, vaginal polyps, myxoma, lipoma, or vaginal prolapse. , Early recognition requires high index of suspicion.
On computed tomography (CT) scan, these tumors have a well-defined margin with attenuation less than that of muscle. On T1-weighted magneticresonance imaging (MRI), these tumors are isointense or have low signal intensity, and have whorled pattern of high signal intensity on T2-weighted MRI. This may be related to the loose myxoid matrix and high water content of angiomyxoma. 
The tumors frequently are large, lobulated, and are non-encapsulated with finger-like projections infiltrating the surrounding soft tissues. , These tumors are soft with homogenous glistening cut surface.  Areas of hemorrhage and cystic change may be present. 
Microscopically, these tumors show low to moderate cellularity and are composed of relatively uniform, small, stellate, and spindled cells set in a loosely collagenous, myxoedematous matrix with scattered vessels of varying caliber. Mitotic figures are infrequent. Although the tumor name implies abundant myxoid matrix, these neoplasms are usually only weakly positive for mucosubstances, a finding that suggests edema fluid as a major component of the non-collagenous stroma. 
Immunohistochemically, these tumors express estrogen receptors and progesterone receptors (ER and PR), suggesting that they may be hormone dependent. The tumor cells also express vimentin, desmin, and smooth muscle actin (SMA) and are negative for S-100.  Ki-67 labelling demonstrates a low proliferative index. 
The term aggressive was introduced to emphasize the locally aggressive behavior and the high potential for local recurrence. The name angiomyxoma was chosen because of the similarity to myxoma and the notable vascular component. 
Although tumor cells show myofibroblastic phenotype, the histogenesis of tumor is poorly understood.  The differential diagnoses range from myxolipoma, and myxoid leiomyoma to myxofibrosarcoma, myxoid liposarcoma, and malignant fibrous histiocytoma. The distinctively striking vascular component, reminiscent of the myxoid liposarcomas, in AA helps in ruling out most of these neoplasms. The absence of lipoblasts in AA helps to differentiate it from a liposarcoma. 
The incidence of local recurrence is 36-72%. Correlation between tumor size and subsequent risk of recurrence has not been demonstrated.  Accurate preoperative diagnosis should alert the surgeon to the need for wide excision for prevention of local recurrence.  The excision is difficult because of the infiltrative nature of the tumor, absence of well defined capsule, and the tumor having the same consistency as that of normal connective tissue.  In our case, the lesion being pedunculated, the excision was complete and the postoperative period was uneventful. There has been no evidence of local recurrence till date.
The criteria that reliably predict the behavior of AA are not known, and recurrences have been observed in small lesions.  Hence, long-term follow-up is necessary.
| References|| |
Steeper TA, Rosai J. Aggressive angiomyxoma of the pelvis and perineum: Report of nine cases of a distinctive type of gynaecologic soft tissue neoplasm. Am J Surg Pathol 1983;7:463-75.
Konndo T. Aggressive angiomyxoma in the inguinal region: A case report. J Med Case Rep 2010;4:396.
Mandal S, Dhingra K, Roy S, Khurana N. Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling. Indian J Pathol Microbiol 2008;51:259-60.
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Martín-Cartes JA, Bustos-Jiménez M, Tamayo-López MJ, Palacios-González MC, Gómez-Cabeza de Vaca V, Ortega AM. Aggressive angiomyxoma: An unusual female pelvic tumour. Report of three cases and review of the literature. Surg Sci 2010;1:40-5.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]