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Rare case of metastatic aggressive angiomyxoma-first case of renal metastasis

Sarah Fatima Siddiqui

Introduction: Aggressive Angiomyxoma (AA) is a rare, locally invasive, mesenchymal tumor known to be benign. It most commonly arises in the vulvovaginal region, perineum and pelvis of women. Most reports of this disease come from case reports, with approximately 350 known cases. With only 3 case reports of metastases and one of death, ours being the fourth case of metastasis and second that of death from Aggressive Angiomyxoma. Ours is the first case of AA to be associated with renal metastasis.

Objective: The objective of this case report is summaries the knowledge of this tumor we have till now and to highlight the need to collect further extensive data on this tumor to better understand its unpredictable nature and reassess its image as a nonmutating tumor.

Methods: A 45-year-old lady referred from a private hospital with provisional diagnosis of cervical fibroid based on her MRI findings. On examination, a large mass was felt in the vagina which was firm, smooth, nontender and immobile. CT scan revealed a large (13 x 12x 10 cms) mass displacing bowel, uterus, ureters, causing, hydroureteronephrosis and bilateral renal metastasis. Her chest x-ray too revealed probable metastasis. Biopsy of the mass was performed which confirmed diagnosis of Angiomyxoma and Immunohistochemistry further confirmed it. She opted for neoadjuvant GnRH therapy. Unfortunately, the patient expired soon after initiating the therapy.

Result: Aggressive angiomyxoma is a relatively rare tumor that presents commonly as asymptomatic mass in the genital area in reproductive age group.   It is more common in women of reproductive age; women to men ration 6:1. Only 7 cases reporting AAM originating outside the pelvic region. Aggressive angiomyxoma has a very high risk for local relapse. Hence, we need to differentiate it from other mesenchymal tumors occurring in this region. AA tends to displace adjacent organs without invading them but its locally infiltrative nature appears to have led the bulky tumors invade adjacent structures as seen in our case and surprisingly also cause distant metastasis. There have been 3 cases of metastasis reported to date. Only one out of these noted the outcome- death from the disease. Ours being the second case of death from aggressive angiomyxoma and the first one to report renal metastasis associated with aggressive angiomyxoma.

Conclusions: Our case, together with unusual previously reported cases, may lead to retrospection of nature of AA, suggesting in a small percentage of cases, possibility of malignancy having an unpredictable outcome as pointed by some authors who have proposed classifying AAM as a tumor of intermediate malignancy with unpredictable behavior.