Leiomyosarcoma of paratesticular tissues is very uncommon. It arises from smooth muscles which form the muscles of the scrotal sac, the seminiferous tubules, and the vascular tissues of the spermatic cord(). Its principal location is in the testicular walls and the spermatic cord [1
Epididymal leiomyosarcoma occurs in men with ages between 50 and 80 years. It may also occur in children as there has been a reported case in a 6-year-old child [2
Certain predisposing factors have been described in the literature leading to testicular leiomyosarcoma such as exposure to large doses of anabolising steroids [3
], chronic inflammation [4
], or past radiotherapy.
There are no reported predisposing factors leading to epididymal leiomyosarcoma with respect to its prognosis in the literature.
It is difficult to define aggressive forms of this tumor due to its relatively rare occurrence; however, its aggressivity seems less important with respect to its location, as there has only been a single case reported of recurrence and of metastasis. The lack of a universal histological classification contributes to the difficulty in determining histological characters that influence the prognosis of this tumor.
The only character described frequently is the mitotic index which is often elevated. Nevertheless, it seems not to help predict the agressivity of the tumor as well as its prognosis as it is not strictly related to the recurrence of the tumor nor the occurrence of metastasis [5
Histological studies of the mass biopsied showed elevated mitotic index.
Immunohistochemical studies showed differentiated smooth muscle cells that tested positive for actin, desmin, and caldesmon. The diagnosis of leiomyosarcoma was made upon these findings.
Most authors prefer treatment by orchidectomy only [6
]. An adjuvant therapy was realized in two cases: chemotherapy adjuvant in the first case (due to elevated mitotic index) [7
] and radiochemotherapy in the second case [8
Some authors reported that an adjuvant therapy may be performed in cases where there exist elevated mitotic index. However, it is difficult to determine the benefits of this complementary treatment as few cases have been reported in the literature [9