Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 11 cases with long-term follow-up

Abstract

The sex distribution of cases reported in this study was roughly equal, in contrast to previous reports emphasising the predominance of this tumour in females. Our study confirms the local aggressive nature of aggressive angiomyxoma, although our local recurrence rate is lower than previous reports (9% versus 36-72%); no metastases and/or disease-related patient deaths were documented. All cases arising in females were positive for desmin, while three of the six cases arising in males were negative for desmin, supporting previous findings and indicating that the lesion may be somewhat different in males. The strong diffuse positivity for CDK4 in all six cases tested goes some way in implicating CDK4, either directly or indirectly, in tumourigenesis. The negative immunostaining for MDM2 would argue against functional amplification of this gene.

Aggressive angiomyxoma presenting as a vulval polyp

“…[9] This hypothesis is supported by the fact, that the tumor cells express desmin and in some cases α-smooth muscle actin along with desmin. [1,2,4,5] Ultrastructural evidence for smooth muscle differentiation has also been described. [8] Recent cytogenetic and molecular studies have identified a variety of genetic alterations, involving the chromosome 12, in the region 12q13-15.…”

“…[2] This tumor is common in the pelvic perineal region of the adults. [1,2,5,6] Its occurrence in retroperitoneum, urinary bladder, vagina and vulva in women and in scrotum, epididymis, testis, inguinal region and bladder in males, has also been documented in the literature. [6] The reported female to male ratio is approximately 6:1.…”

“…[6] Clinically the tumor presents as a locally infiltrative mass with potential for recurrence, many years after the primary excision. [1,2,5,6] In all the seven cases including the present one, where the presentation was that of a pedunculated mass, the diagnosis was unsuspected preoperatively [5] and in these cases, local excision around the pedicle base can effect a cure, provided the pedicle base is free of tumor. Except for positive surgical margins there are no clinical or histological predictors for tumor recurrence and hence the effort should be towards obtaining adequate clearance with tumor-free resected margins.…”

“…[1,2] The reported local recurrences varied from 9% to 72% during long-term follow-up. [1] So far two cases have been documented in the literature where the recurrent tumor metastasized to the lungs, resulting in death of the patient in one case. [3] They often present as an infiltrative mass in the pelvic-perineal region of adults, more common in women.…”

“…[3] They often present as an infiltrative mass in the pelvic-perineal region of adults, more common in women. [1,2,4] Clinical presentation as a polyp is rare, with only six such cases being documented in the world literature so far. [5] The pathologist should closely scrutinize the resected margins for any evidence of tumor extension, which is the most significant predictor for tumor recurrence.…”