Several beneficial results with tamoxifen or gonadotropin-releasing hormone GnRH agonist have been described [ 1 , 9 , 17 ]. Unfortunately, the highest recurrence rates after resection still remain a major surgical problem that should be solved. In view of these findings, we think that there is still a lack of knowledge about the diagnosis of AA among the clinicians. The female-to-male ratio has been reported as 6. First, the tumor may not have been diagnosed prior to initial surgery, and the extent of the tumor may not be perceived. In women it is most commonly found in the perineum and pelvic organs including the bladder and uterus 3. It is most frequently found in the scrotum, perineum, groin, pelvis and spermatic cord 1.
Introduction Aggressive angiomyxoma AA is an uncommon mesenchymal tumor which is predominantly encountered among adult females in reproductive age [ 1 ]. Respecting this view, we believe that it is not compatible with the oncological principles. View at Google Scholar I. These tumors usually occur at later ages among men, between the third and fifth decade of life with an average age of 46 years 2, 11, The tumor is distinguished from other lesions by these histopathologic features. Abstract Aggressive angiomyxoma AA is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions.
Case Reports in Surgery
In view of these data, the demographic characteristic and the tumor localization of our patient were similar to the majority of previously reported cases. Ann R Coll Surg Engl ; Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Ultrasound may show a well-defined hypoechoic mass with thin multiple internal echogenic septa with frankly cystic appearance 2, 9, This has created some controversy over whether surgical margins are associated with recurrence, but the results of these reports have not been possible to reproduce 2, On exploration, a giant, soft, rubbery, and gelatinous appearing mass like lung tissue that approximately filled serues whole abdomen was noted.
Presentation of the presacral tumor after performing digital dissection of retrorectal space. Among female patients it usually presents as a long lasting mass accompanied by perineal, labial or pelvic pains similar to pulsating pressures. Clinical case report and literature review Juan Fernando Medina R. A year-old nulliparous female patient with a previous history of left femoral hernia operation was admitted to our hospital. They described it as having a predilection for the soft tissues of the pelvis and perineum of young women and of being aggressive in the sense of having high frequencies of local recurrence even after adequate initial treatment9.
Complete resection is considered to be the treatment of choice for primary tumors and for recurrences6, 7, There have been no reports of metastasis among men to date Lesions disappear or their size, as measured clinically and with imaging, decreases within three months to a year.
However, there are series of cases which have reported no differences in rates of recurrence among patients with negative surgical margins and those with positive margins. The diagnosis of AA is very difficult because it is often asymptomatic until the tumor reaches large sizes. MRIs are preferable to CAT scans because of their better tissue characterization and lack of ionizing radiation.
Case Report and Literature Review. Incidence and reports of the disease have been increasing each year The tumor was invading adjacent soft tissue including adipose tissue, muscles, and nerves. Findings on CT and MR imaging.
Consequently MRIs are the method of choice for monitoring and controlling possible recurrences 2, 3, 6, 7, 9, 10, Several beneficial results with tamoxifen or gonadotropin-releasing hormone GnRH agonist have been described [ 1917 ].
Aggressive angiomyxoma AA is an uncommon mesenchymal tumor which is predominantly encountered among adult females in reproductive age [ 1 ].
Archive ouverte HAL – Aggressive angiomyxoma: A case series and literature review
The earliest reported recurrence happened just two months after initial surgery 6, 10, Journal of Medical Case Reports ; 4: The tumor has exerted pressure on the bladder, the left ureter, uterus, and left iliac vein.
Most are diagnosed in the first two to three years after initial surgery with average recurrence time between 9 and 14 months following surgery 2,9, 11, 12, Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor.
The current treatment of AA is complete surgical excision with tumor-free margins [ 168 ]. However, there is still a debate about the treatment because of high recurrence rates in spite of wide surgical excision.
Aggressive angiomyxoma in men: Clinical case report and literature review
However GnRH agonists are used literatuure mainly as adjuvant therapy for residual or recurrent tumors among patients who are poor candidates for surgery or for those who refuse surgery but who test positively for estrogen and progestin receptors, independently of the strength of that positivity 2, 6, 7.
This gene is an architectural transcription factor that is primarily expressed during embryogenesis and is a member of the High Mobility High Mobility Group-HMG of proteins. Case Presentation A year-old nulliparous female patient with a previous history of left femoral hernia operation was admitted to our hospital. It has been reported in male and female children as young as 2 years old 2, 3, 5.