Aggressive Angiomyxoma in the Scrotum: A Case Series and Literature Review

Aggressive angiomyxoma is a rare mesenchymal tumor, characterized by frequent local recurrences. Our aim is to assess the role of radical resection. Retrospective case review. The records of five patients with aggressive angiomyxoma during the period from 1984 to 1998 were reviewed and analyzed. A MEDLINE search from 1983 to May 1999 was performed. The clinical presentation, surgical treatment, resection margin involvement and clinical outcomes were analyzed. Together with our five cases, 106 cases have been reported in the world literature. The female-to-male ratio was 6.6:1. The age distribution was wide, with the peak incidence at 31 to 35. The local recurrence rate was high. Seventy-one percent of recurrence occurred within the first 3 years. Patients with clear resection margins have similar chances of remaining disease-free compared with those having tumor-involved resection margins. There was no correlation between the size of the tumors and the chance of recurrence. Though we aim for complete resection, incomplete or partial resection is acceptable, especially when high operative morbidity is anticipated and preservation of fertility is an issue. Long-term follow-up and careful monitoring with imaging techniques are essential for timely identification of recurrence and prompt resection.

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient. We document this case and report a similar one, found in the literature, of a postmenopausal woman with pulmonary and mediastinic metastases. These cases may expand the current concepts of potential behavior of aggressive angiomyxoma.

Aggressive angiomyxoma is an uncommon mesenchymal tumor that preferentially involves the pelvic and perineal regions of females. Since its initial description in 1983, approximately 65 cases have been reported in the English literature. The clinical and pathologic features of 29 cases of aggressive angiomyxoma were evaluated in a review of archival material from the Armed Forces Institute of Pathology (1960-1992). Histochemical stains for mucosubstances and immunohistochemistry (avidin-biotin complex method) were utilized to characterize the neoplasms further. All patients were females, between 16 and 70 years (median; 34 years). The soft tissues of the pelvis, perineum, vulva, buttock, retroperitoneum, and inguinal regions were involved. The majority of the tumors were > or = 10 centimeters in greatest dimension. Follow-up ranging from 8 to 198 months (mean, 93 months; median, 95 months) was available for 22 patients. Eight patients developed recurrent tumor, from 10 months to 7 years after the initial resection. No patient developed metastases and there were no tumor related deaths. Histologically, the neoplasms were sparsely to moderately cellular and predominantly composed of bland, relatively nondescript. stellate and spindled cells embedded in a loosely collagenized matrix with scattered vessels of varied caliber. A few cases contained some tumor cells with more abundant eosinophilic cytoplasm that raised the possibility of focal smooth muscle differentiation. The tumor matrix was no more than weakly reactive for mucosubstances; thus, while glycosaminoglycans are present to a limited extent, edema fluid appears to be a major component of the noncollagenous stroma. The neoplastic cells were at least focally immunoreactive for desmin (22/22), smooth muscle actin (19/20), muscle specific actin (16/19), vimentin (17/17), CD34/QBEND-10 (8/16), and estrogen (13/14) and progesterone (9/10) receptor. All of the examined tumors were negative for S100 protein (20/20). Ki67 (MIB1) immunoreactivity was present in <1% of the tumor nuclei in all 16 cases tested. Aggressive angiomyxoma is a distinctive, locally aggressive, mesenchymal tumor that appears to be relatively site specific and has a peak incidence in females in the fourth decade of life. There is a strong propensity for local recurrence but metastatic disease has not been reported. Since the first evidence of recurrence may be many years after the initial resection, long term follow-up is required. The neoplastic cells of aggressive angiomyxoma exhibit fibroblastic and myofibroblastic features and appear to be hormonally influenced. The possibility that the progenitor cell has a capacity for smooth muscle differentiation is raised.