Aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis
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- Salman, M.C., Kuzey, G.M., Dogan, N.U. et al. Arch Gynecol Obstet (2009) 280: 485. doi:10.1007/s00404-009-0934-2
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Aggressive angiomyxoma is an uncommon soft tissue tumor which preferentially involves pelvic and vulvoperineal regions of young adult females. The typical characteristics include gelatinous appearance and locally infiltrative nature without evidence of nuclear atypia or mitosis. Treatment involves surgery, but local recurrence is high in spite of apparently complete surgical resection.
A 28-year-old woman who had had a history of surgically excised aggressive angiomyxoma of vulva presented with a complaint of vulvar mass. The mass was removed and histopathologic examination revealed an aggressive angiomyxoma without necrosis, atypia, and mitosis.
Aggressive angiomyxoma should be kept in mind when an asymptomatic and slow-growing vulvar mass is detected in young females. The surgery should aim wide local excision and long-term follow-up is necessary due to high rate of local recurrence.
KeywordsMesenchymal tumorStromaFibroblastBlood vesselsWide local excisionVulva
Aggressive angiomyxoma was first described in 1983 as a distinctive soft tissue tumor which preferentially involves pelvic and vulvoperineal regions of young adult females . It is an uncommon tumor with only about 150 cases reported in the English literature up to date. The typical characteristics include slow growth, gelatinous appearance and locally infiltrative, but non-metastasizing nature without evidence of nuclear atypia or mitosis [4, 7]. Treatment involves surgery consisting of local excision with tumor-free margins, but local recurrence is high in spite of apparently complete surgical resection [7, 9].
Here, we report a case of aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis despite a wide local re-excision.
Aggressive angiomyxoma is a soft tissue tumor which may involve pelvis, retroperitoneum, vagina, vulva, perineum, and buttocks and usually presents as a polipoid or cystic mass . Although the age distribution is wide ranging from 6 to 77, the peak incidence is at fourth decade of life [2, 3]. The size is also variable, but most are larger than 10 cm .
The tumor lacks a capsule and grows slowly with local infiltration [2, 3]. Due to this slow and insidious growth pattern, the patients are often asymptomatic and masses are detected incidentally during examinations or imaging studies . Both the primary and recurrent disease of the current case was asymptomatic.
Histologically, the tumor consists of monotonous population of spindle-shaped cells that infiltrate the surrounding tissues and lack significant nuclear atypia or mitotic figure lying on a loose collagenous myxoid background with numerous medium-sized vessels that are typically clustered . Aggressive angiomyxomas were reported to derive from myofibroblasts as a phenotypic variant of the basic fibroblast with cytoskeletal adoption appropriate to situations as wound healing which may be the reason for the locally invasive character and induction of neovascularity . The neoplastic cells may also show immunoreactivity for desmin, smooth muscle actin, muscle specific actin, vimentin, and estrogen and progesterone receptors [3, 7]. The tumor cells appear to be hormonally influenced . The tumor of our case was negative for S-100, desmin, smooth muscle actin, estrogen, and progesterone receptors.
Preoperative diagnosis demands high index of suspicion due to its rarity . In addition, imaging studies such as computed tomography, ultrasonography, and bone scan may be performed before surgery since the real extent of tumor is often underestimated by examination and invasion into the bladder, gastrointestinal tract, and bone has been described . The true extent of the tumor may be documented by magnetic resonance imaging and thus preoperative magnetic resonance imaging may aid surgical approach . Also, the disease may not represent a purely localized disease since pulmonary metastasis was reported in one case . The probable diagnosis of the first lesion was a lipoma in our patient, but the second lesion caused a high suspicion of recurrence and a pelvic imaging was performed to document possible extension of tumor.
The best treatment of choice consists of surgical excision with wide tumor-free margins . However, local recurrences up to 70% of cases may be seen after adequate surgical resection . The term “aggressive angiomyxoma” was preferred due to these high rates of recurrences which may be detected as late as 15 years after initial diagnosis . Therefore, long term follow-up is inevitable after surgery. Although, a second wider excision has been advocated in patients with positive surgical margins after primary resection in order to decrease the risk of recurrence, patients with clear resection margins were reported to have a recurrence risk similar to that of patients with involved surgical margins . Also, the tumor size is not correlated with recurrence. For this reason, incomplete or partial resection is acceptable when high operative morbidity is a concern and fertility preservation is needed . In general, adjuvant radiotherapy or chemotherapy is not preferred due to the low mitotic activity . The recurrence was observed in our case in spite of a wide re-excision with negative surgical margins. Therefore, she refused a second wider excision in recurrent disease.
In conclusion, aggressive angiomyxoma should be kept in mind when an asymptomatic and slow-growing vulvar mass is detected in young females. The surgery should aim wide local excision and long-term follow-up is necessary due to high rate of local recurrence. Due to possible involvement of wide areas, at least pelvic imaging should be performed before surgery and the patient should be informed about the possibility of relatively radical surgical interventions.
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