Aggressive angiomyxoma of scrotum in a young male: A rare entity
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/MJDRDYPU.MJDRDYPU_297_16
Keywords: Aggressive angiomyxoma, rare entity, scrotal mass
TAG:2]Introduction[/TAG:2] Aggressive angiomyxoma (AAM) was first described by Steeper and Rosai in 1983. AAM must be differentiated from other benign myxoid neoplasms given its propensity for local recurrence [1] AAM is a rare benign mesenchymal myxoid tumor of the pelvis and perineum which occurs almost exclusively in adult females. Overall, its incidence is about 6-fold higher in females.[2] It usually arises from the soft tissues of the pelvic region, perineum, vulva, and buttock. The adjective “aggressive” emphasizes the neoplastic character of the blood vessels, its locally infiltrative nature, and the high risk of local recurrence after surgical excision.[3] It is called angiomyxoma because of its myxoid nature and prominent vascularity.[4] Rarely, this tumor appears in males simulating inguinal hernia, testicular neoplasm, spermatic cord neoplasm, hydrocele, or spermatocele.[2]
A 15-year-old male child presented with a slow-growing scrotal swelling for 6 months. Clinical examination revealed diffuse scrotal swelling measuring about 6 cm × 5 cm × 3 cm. Ultrasound scrotum showed a diffuse subcutaneous scrotal wall thickening with hydrocele. Testis and epididymis on both sides showed no abnormalities. A subtotal excision of the scrotal mass was done and sent for histopathological examination. Gross appearance
Microscopic appearance Sections studied showed ill-defined lobules, composed of predominantly hypocellular, myxoid areas with spindle to stellate cells. There are numerous blood vessels of varying sizes. The focal area showed perivascular mononuclear inflammatory infiltrate [Figure 1], [Figure 2], [Figure 3], [Figure 4].
AAM was first described by Steeper and Rosai in 1983. Around 150 cases have been described in literature since then.[4] It is a morphologically distinctive, slowly growing vulvovaginal mesenchymal neoplasm that occurs chiefly in adult women with a marked tendency for local recurrence. It usually does not metastasize, but few cases have been reported in literature where metastasis has occurred.[5],[6] Due to its locally infiltrative course and frequent recurrences, it has been termed aggressive. The occurrence of AAM in men is extremely rare, and in men, AAM is usually derived from the pelviperineal interstitial tissue involving the scrotum (38%), spermatic cord (33%), perineal region (13%), and intrapelvic organs (8%).[3] AAM in the scrotal region may present as a scrotal mass, often mistaken for a hernia or hydrocele.[4] Detailed radiological workup may be helpful in detection, but histology is the gold standard for establishing the diagnosis.[4] In both males and females, the gross and microscopic appearance and clinical course of disease are similar. On gross examination, the tumors are lobulated, soft to rubbery, solid masses. The cut surface reveals a glistening, soft homogeneous appearance. Recurrent tumors show more prominent areas of hemorrhage and fibrosis.[7] Microscopically, the tumor is composed of widely scattered spindled to stellate-shaped cells with ill-defined cytoplasm and variably sized, thin- or thick-walled vascular channels in a myxoid stroma that is rich in collagen fibers. Cellularity is usually low, but focal areas show increased cellularity, particularly around large vessels. Mitotic figures are rare or absent. The stroma is characterized by prominent myxoid change with fine collagen fibrils, often with areas of erythrocyte extravasation. A characteristic feature is the presence of variably sized vessels that range from small thin-walled capillaries to large vessels with secondary changes including perivascular hyalinization and medial hypertrophy.[8] Differential diagnosis of this tumor includes angiomyofibroblastoma, cellular angiofibroma, cutaneous myxoma, myxoid neurofibroma, intramuscular myxoma, and myxoid lipoma.[9] Immunohistochemically, there is positivity for desmin (more so in the female counterpart of this lesion), smooth muscle actin (about half of the cases), CD34 (about half), and estrogen and progesterone receptors (100%, irrespective of gender).[1]
Being a rare lesion, AAM must be considered in the differential diagnosis of scrotal swelling. Careful preoperative workup should be carried out in suspicious cases. Rejection with clear margins is necessary to prevent recurrence. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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