Lipomatous angiomyofibroblastoma: A 20-year literature review

Abstract

Lipomatous angiomyofibroblastoma (LAMF) is a rare superficial muco-cutaneous tumor of the female genital tract in women of reproductive age and early menopause. We report the case of a 36-year-old woman who presented with an asymptomatic 1 cm round vulvar mass. The tumor showed both hypercellular and hypocellular areas composed of spindle and epithelioid cells set in a loose fibromyxoid background. Anastomosing networks of small to medium sized dilated vessels, surrounded by condensed spindle cells, were distributed equally throughout the tumor. Mature adipocytes comprised more than half of the tumor's volume. The neoplastic cells were reactive for both desmin and vimentin and did not express CD34, smooth muscle actin, S100 protein and muscle-specific actin. Making the right diagnosis may be challenging to medical professionals as LAMF may mimic both clinically and histologically several other benign and malignant gynecological lesions. LAMF has a benign course without evidence of local recurrence or metastatic potential.

Paratesticular Angiomyofibroblastoma-Like Tumor: Unusual Case of a Solidocystic Form

“…Atypia, mitosis, and necrosis are usually absent [2–4, 7, 9, 11]. Rare mitoses (between 1 and 4 mitoses/50 high power fields) were observed in other reported cases [4, 5, 7, 8]. Scattered cytonuclear atypia were reported by João et al [3].…”

“…Intralesional fat tissue can sometimes be present [ 1 – 3 ]. A lipomatous variant has been described [ 8 , 10 ]. In this case, the mature adipocytes occupy more than 50% of tumor volume [ 8 ].…”

“…A lipomatous variant has been described [ 8 , 10 ]. In this case, the mature adipocytes occupy more than 50% of tumor volume [ 8 ]. Atypia, mitosis, and necrosis are usually absent [ 2 – 4 , 7 , 9 , 11 ].…”

“…Tumor regression has been seen in angiomyofibroblastoma [ 8 ]. Only one case of tumor recurrence after 13 years has been reported, and no cases of sarcoma or pejorative evolution transformation have yet been described [ 1 , 4 , 5 , 8 ]. The recommended treatment is the surgical excision with clear wide margins.…”

Vulvar Cutaneous Myxoma in a Patient With Carney Complex: Avoiding Pitfalls of Myxoid Lesions of the Vulva

“…In our case, mature lipocytes intervening the 2 myxoid nodules may have represented lipomatous AMF. 4 These lipocytes were however outside of the fibrous septae, and thus constituted normal subcutaneous fat. Furthermore, desmin, ER, and PR are usually expressed in AMF.…”