aggressive angiomyxoma ( aam ) is a rare , acquired , benign , neoplasm arising from mesenchymal tissue . the term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with recurrence . under 250 cases of aam were reported in the literature since the first description of aam in 1983 . a case of aam in a post - menopausal woman a 55-year - old female patient was referred by a gynaecologist with the complaint of a gradually progressive , painless swelling in the vagina for the past 2 years . a mother of four children , she attained menopause 10 years ago and was asymptomatic otherwise . , there was a glistening , white , gelatinous , non - tender , polypoid swelling , with multiple , superficial ulcerations , protruding out of the vaginal orifice . on close inspection , the tumor was found to be pedunculated , measuring 8 cm long , soft to rubbery in consistency and originating from the anterior vaginal wall [ figure 1a  c ] . there was another sessile lesion , 0.5 cm  0.5 cm , over the posterior vaginal wall . ( a and b ) a glistening , white , gelatinous , polypoid swelling , with multiple , superficial ulcerations , originating from the anterior vaginal wall , protruding out of the vaginal orifice . ( c ) an excised , pedunculated mass , about 8 cm long with multiple , superficial ulcerations hpe of the excised mass showed a tumor , composed of fibromyxoid stroma , around proliferating vascular channels of varying size , covered by ulcerated stratified squamous epithelium . the features were suggestive of an aam [ figure 2a  d ] . ( a ) tumor composed of fibromyxoid stroma around proliferating vascular channels of varying size covered by ulcerated stratified squamous epithelium 10 , h and e. ( b ) proliferating vascular channels in fibromyxoid stroma 10 , h and . ( c ) surface epithelium covered by neutrophils 10 , h and e. ( d ) congested vascular channel 20 , h and e aam is an uncommon mesenchymal neoplasm , occurring predominantly in the pelvi - perineal region of adults , first described in 1983 , by steeper and rosai . often reported from the vulvovaginal region , simultaneous abdominal swelling and gluteal region involvement has also been reported . aams are usually solitary , but multi focal involvement has been noticed by various authors , similar to our patient . solitary tumors are reported to be without any systemic abnormalities while multiple lesions could be a manifestation of the carney complex . the present patient , though having multiple lesions , did not have any clinical evidence of the carney complex in the form of myxomas at other sites or other endocrine pathology . the carney complex , a familial multitumoral syndrome , comprises spotty skin pigmentation , ( lentigenes and blue nevi ) , myxomas ( heart , skin , and breast ) , endocrine over activity , usually manifested by endocrine tumors , ( adrenal cortex , pituitary , testis , and thyroid ) schwannomas , and two unusual pigmented tumors , epithelioid blue nevus ( skin ) , and psammomatous melanotic schwannoma ( involving skin , viscera or nerve tissue ) . carney complex has been linked to chromosome 2p16 and the prkar1a gene at 17 q22 - 24 . cutaneous myxomas range in appearance from small , sessile , opalescent or dark pink papules to large finger - like pedunculated lesions . in the present case , angiomyofibroblastoma , cellular angiofibroma , superficial myofibroblastoma and fibroepithelial polyp are other conditions , which occur predominantly over the perineum and may be confused with aam . the size of the lesion usually > 5 cm , hypocellularity , presence of thick walled blood vessels , infiltrative nature , absence of involvement of the superficial epithelium favor the diagnosis of aam . the estrogen and progesterone receptor positivity suggests that aam might be hormone dependent as rapid growth has been observed during pregnancy . the tumor occurs in women in 3 and 4 decade affecting vulva , perineum , and pelvis . in the present case , patient attained menopause 10 years earlier and the duration of the present illness was only 2 years . it could be presumed that , probably she was unaware of the intravaginal growth until the time it protruded out and became palpable externally . aams must be differentiated from cutaneous focal mucinosis , since , myxomas are true neoplasms , and although benign , may recur after incomplete excision . histologically angiomyxoma is a mesenchymal tumor , composed of fibroblasts within a strong myxoid background . vascular proliferation is also prominent and virtually no mitoses are reported , similar to the findings in the present case . in addition to the characteristic features of aam , other changes such as ulceration of the squamous epithelium with areas of necrosis observed in our case could be attributed to the infection associated with friction due to chronic exposure . the vast majority of cases demonstrate positivity for desmin in the myxoid bundles and/or stromal cells while actions and cd34 may be variably positive . chromosomal translocation of the 12 q13 - 15 band involving the high mobility group a ( hmga2 ) gene has been described . chromosomal analysis and other special histochemical marker studies could not be carried out since the patient could not afford these investigations . imaging of these tumors is important to determine the extent and thus the optimal approach to surgery . after the biopsy report was available , the patient was advised ultra sonography ( usg ) and magnetic resonance imaging of the abdomen and pelvis , but the results were not contributory . surgical excision as undertaken in our case is the treatment of choice . gonadotropin releasing hormone agonist therapy seems to have a significant beneficial role , especially as an adjuvant to surgery or where surgery is not possible . other reported alternative methods of treatment to decrease the chances of local recurrences are pre - operative angiographic embolization , pre - operative external beam irradiation , and intra - operative electron beam radiotherapy . this could probably be because patients with aam often register with the gynaecologist or a surgeon and rarely , if ever , report to a dermatologist .
myxoid tumors are a heterogeneous group of lesions characterized by a marked abundance of extra cellular mucoid ( myxoid ) matrix.[1 ] the term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with recurrence.[2 ] a case of aggressive angiomyxoma arising from the vagina in a 55-year - old woman is reported for its rarity .