Its underlying mechanisms are still under study 2. Unusual Abdominal and Pelvic Tumors. Case Reports in Surgery. According to the histopathological and immunohistochemical findings, the case was interpreted as intra-abdominal aggressive angiomyxoma. The radix of the tumor was reaching until the posterior of the vagina. This is a relatively rare tumor that most surgeons will not find in their careers 8 , and the fact that the case presented here is that of a male patient reinforces its unusual nature It has been reported in male and female children as young as 2 years old 2, 3, 5.
MRI is more helpful than the other imaging studies. Cytogenetic analysis of the 7 female cases reported at that time showed karyotype abnormalities associated with a translocation in the 12q region and even the loss of an X chromosome. In differential diagnosis among angiomyxoma, myxoid liposarcoma and myxoma for these patients, patients with angiomyxoma should test positive for vimentin and negative for S protein To date few cases have been described in sites other than these areas 2, 4, 7, An immobile, painless mass which filled lower quadrants of the abdomen was palpated. Although its features are benign, two cases with distant metastasis in women for whom aggressive angiomyxoma was fatal have been described.
Rev Med Hosp Gen Mex ; 70 2: It is locally aggressive and metastasizes quickly and easily. A small incision in the right perianal margin is made to remove the last adhesions and the resected piece 7. This is probably because complete resection is easier in the paratesticular region of men than in the pelvis and perineum of women 1.
This incident followed 2 years of slow growth of a mass at right gluteus level adjacent to the posterior midline. Intravenous pyelograms, marrow scintigraphy, barium enemas and pelvic angiography have also been used to assess the extent of the lesion before surgery 6. Journal of Medical Case Reports ; 4: 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, Extension from the perineum into the pelvis is common and often unnoticed clinically 9.
In the absence of sufficient studies hormone therapy cannot replace surgery. The current treatment of AA is complete surgical excision with tumor-free margins [ 168 ]. Translation from Spanish to English by T.
Archive ouverte HAL – Aggressive angiomyxoma: A case series and literature review
Unusual Abdominal and Pelvic Tumors. Angiomyxoma diagnosed in a man cae for abdominal lipectomy. Aggressive angiomyxoma AA is an uncommon mesenchymal tumor which is predominantly encountered among adult females in reproductive age [ 1 ]. Among women sites which are frequently involved include the vulva, the vagina, the groin, the buttocks and the peritoneum, but it has also been described in the oral cavity, the uterus, the cervix, the bladder and the pararenal space 4, 7, They are liferature with pink or red outer surfaces and gray colored cross sections resulting from myxoid stroma that is rich in collagen fibers.
On T1-weighted MR imaging, the tumor shows isosignal compared to the muscles while on T2 high signal intensity is detected. It is likely due to the loose myxoid matrix and high-water content of AA [ 8 ].
Furthermore, because the revieq of the tumor is often underestimated by clinical examination, these imaging studies also help us in deciding the surgical strategy.
Aggressive angiomyxoma: A case series and literature review
According to the immunohistochemical findings of the present tumor, our case was diagnosed as AA. Aggressive angiomyxoma was first described in by Steeper and Rosai as a locally infiltrative benign mesenchymal neoplasm. The tumor was completely excised by sharp and blunt dissection Figure 2. Although it is almost exclusively encountered among females in reproductive age, rare cases have been diagnosed in the perimenopausal female, children, and male patients ajgiomyxoma 7 ].
Juan Fernando Medina R. About 2 years later Begin published his experience with 9 patients which included the first report of this entity among men 5. Management requires complete revieww of the lesion and the clinical and imaging follow-up given their high rate of recurrence.
Case Reports in Surgery
Nevertheless, longer-term follow-up using the same studies should be considered 11, Currently this translocation is considered to be the most common chromosomal abnormality associated with the emergence of human mesenchymal neoplasms, and thus HMGA2 cannot be used as a specific marker for aggressive angiomyxoma.
Int J Gynecol Pathol ; 25 4: Because of its rarity, the clinical presentation and the treatment method of the tumor have been described mostly based on individual case presentations. Abstract Aggressive angiomyxoma AA is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions.
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. Operative findings of the tumor. Clinical case report and literature review Juan Fernando Medina R.
Case reports suggest that medical treatment with GnRH agonists are a viable option for both primary and recurrent tumors, especially given the radical and often debilitating nature of the surgery required to remove the tumor.