Aggressive angiomyxoma of vulva and vagina: a series of three cases and review of literature

Abstract

Angiomyxoma of vulva and vagina refers to a rare disease; diagnosis is not at all clinical, thus, cases presenting as bartholin cyst, benign vulval lesions and vaginal wall cysts should have complete radiological work up before excision, as pre-diagnosis can change the treatment modality and prognosis of patient.

Vulval angiomyxoma: a case report

“…5 Although slow growing, they may attain large sizes (up to 60 cm) but are usually 10-20 cm in their greatest diameter and have a marked tendency to recur locally (up to 72%), even decades after resection. [3][4][5] In our case it was also a huge mass of size 30 cm × 14cm × 10 cm ( Figure 2, Figure 3). Usually, this tumor is non-metastasizing, but there are reports of multiple metastases in women treated initially by excision and who later succumbed to metastatic disease.…”

“…3,4 The case in discussion belongs to this group. The term "aggressive" refers to the locally infiltrative nature of this lesion and its tendency to recur.…”

“…4 On gross examinations, these tumors are characteristically soft, bulky masses with a smooth external surface, and exhibit a gelatinous appearance on cut section. 3 Histologic examination shows a sparsely cellular tumor composed of pale to eosinophilic stroma studded with numerous haphazardly arranged blood vessels that stand out against the myxoid background and range in size from thin-walled capillaries and venules to larger muscular arteries ( Figure 5). The stroma is distinctly myxoid with intermixed, wispy collagen fibrils; scattered, smooth muscle bundles; and extravasated erythrocytes.…”

“…7,8 Immunohistochemically, most AA express different combinations of estrogen and progesterone receptors, vimentin, desmin, smooth-muscle actin, muscle-specific actin, CD34, and CD44, but all are invariably negative for S-100, CEA, and keratin. 3,4 Cytogenetic and molecular analysis revealed clonal karyotypic abnormalities including chromosomal translocation involving chromosome 12 associated with rearrangement of the HMGIC gene and it was proposed that AA molecularly belonged to the benign group of mesenchymal tumors showing multiple aberration region involvement. HMGIC expression in aggressive angiomyxoma is of value in the distinction of difficult cases from its histological mimics.…”

“…8 Angiography typically shows a hypervascular mass. 3,4,8 Our patient was not subjected to radiological investigation as its clinical appearance at presentation was that of a benign mass. Wide surgical excision is the traditional treatment of choice.…”