|Year : 2001 | Volume
| Issue : 1 | Page : 96-97
Aggressive angiomyxoma of the epididymis - a case report
Shubha N Rao, Erel Dais, Shivananda Prabhu, TK Susheel Kumar, Paul Jose
Department of General Surgery, Kasturba Medical College, Attavar, Mangalore, India
Shubha N Rao
B-21, RNE Flats, KMC Quarters, Mangalore - 575 003
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Aggressive angiomvxoma is a distinctive, locally aggressive but non-metastasizing soft tissue tumour of the pelvic region and perineum. This is seen more commonly in premenopausal women. We report a case that occurred in a man who presented with swelling in the inguino-scrotal region for which the patient underwent herniorraphv for hernia with excision of the mass at the upper pole of the testis, in the region of the epididymis.
Keywords: Aggressive Angiomyxoma; Myxoid Tumour; Soft Tissue Tumour.
|How to cite this article:|
Rao SN, Dais E, Prabhu S, Susheel Kumar T K, Jose P. Aggressive angiomyxoma of the epididymis - a case report. Indian J Urol 2001;18:96-7
| Introduction|| |
Aggressive angiomyxoma was first recognised as a distinct soft-tissue tumour of adult women less than 40 yrs of age by Steeper and Rosai in 1983. Reports of AAM are rare. In both men and women, most cases of AAM involve the perineum and perianal region grossly. AAM is a large, soft, myxoid, apparently well-circumscribed lesion.
Microscopically, it shows hypocellular, myxoid tissue with numerous medium sized vessels. Tumour cells are spindle cells, fibroblasts and myofibroblasts, that lack significant nuclear atypia and mitotic activity. Cells are immuno-reactive to muscle actin but do not show S-100 protein. We hereby present a case of aggressive angiomyxoma and discuss the histologic features most helpful in differential diagnosis from other tumours.
| Patient and Methods|| |
A 56-year-old male patient presented with a swelling in the left inguino-scrotal region since one year. This patient had a left inguinal hernia which was reducible. In addition after reduction, a scrotal swelling was found which was non-tender and soft in consistency and was transilluminant. The patient underwent left inguinal herniorraphy. On scrotal exploration, a cystic swelling containing yellow-coloured fluid was found lying at the upper pole of the testis.
The tumour was easily resected without jeopardizing the testis or spermatic cord. The patient recovered without complications.
| Results|| |
Pathology: The lesion was cystic in nature and contained serous, yellowish fluid. Histologically, the tumour was composed of spindly & stellate cells in a myxoid background. Multiple thin- and thick-walled blood filled vessels were seen.
| Discussion|| |
Mesenchymal tumours present a diagnostic dilemma because a wide variety of them exhibit a myxoid stroma which can be so prominent that it is difficult to differentiate intramuscular myxoma and myxoid liposarcoma.
Aggressive angiomyxoma is a rare, distinctive tumour involving the pelvic soft tissues (particularly vulva) clinically characterised by infiltrative growth, which has frequent local recurrence and lacks documented metastases.,,, It often attains a large size and can involve the deep paravaginal and pararectal tissues particularly in recurrences.
Only a small number of cases have been documented in men and they occur over a wide age range in variety of sites in genital tract such as perineum, perianal region, spermatic cord, inguinal region and pelvic soft tissues.
Aggressive angiomyxoma should be distinguished from benign tumours with a low risk of recurrence such as intramuscular myxoma, neurofibroma, myxoid lipoma, spindle cell lipoma, angiomyofibroblastoma on one hand and from tumours with metastatic potential such as myxoid liposarcoma, myxoid malignant fibrous histiocytoma, embryonal rhabdomyosarcoma on the other.
The genital tract is not a site for occurrence of myxoma which occurs almost exclusively within skeletal muscle. Furthermore, vascular proliferation is not a feature of intramuscular myxoma. Neurofibroma does not display the prominent vascularity of AAM. The genitalia is not a common site for spindle cell lipoma. Furthermore, lack of characteristic spindle cells and sclerotic background of spindle cell lipoma do not support the diagnosis of lipomatous tumour.
Angiomyofibroblastoma is characterised by circumscribed borders. It is actin-negative and desmin-positive.
Myxoid liposarcoma shows straight arborizing vessels and lipoblasts and absence of thick hyalinised blood vessels.
Myxoid MFH is characterised by prominent curvilinear branching small vessels among which pleomorphic tumour cells are scattered. Embryonal rhabdomyosarcoma is seen almsot exclusively in the first two decades of life. It can be recognised by the much higher cellularity, nuclear pleomorphism, presence of occasional rhabdomyoblasts and lack of significant vascular component.
In conclusion, aggressive angiomyxoma merits wider recognition as a distinctive tumour type which can occur around the genital region of men.
| References|| |
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|2.||Begin LR, Clement PB, Kirk ME et al. Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol 1985; 16: 621-628. |
|3.||Allen PW. Myxoid tumours of soft tissue. Pathol Annu 1980: 15: 133-191. |
|4.||Destian S. Ritchie WGM. Aggressive angiomyxoma: CT appearance. Am J Gastroenterol 1986: 81: 711-713. |
|5.||Hilgers RD, Pai R, Bartow SA et al. Aggressive angiomyxoma of vulva. Obstet Gynaecol 1986; 68 (suppl): 60S-62S. |
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