Leiomyosarcoma of the testis is a rare disease and only case reports (Level V Evidences) are available due to lack of clinical trial. Low grade tumour is manageable by early diagnosis and treatment by high inguinal orchidectomy while in high grade tumours, metastasis or recurrence, chemotherapy is advised.
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Introduction:
Testicular cancers have good outcome due to potential of cure even in the presence of metastatic disease. In case of cancer progression or recurrence after initial chemotherapy, these patients are candidates for salvage therapy. Among all tumours, non-seminoma is more aggressive than seminoma. If both seminoma and non-seminoma are present or the alpha-fetoprotein (AFP) concentration is elevated, the tumour should be treated as a non-seminoma.
Initial therapy of testicular tumour is selected according to the American Joint Committee on Cancer (AJCC 2010) stage group; risk stratification (good, intermediate, or poor risk), as per the guidelines of the International Germ Cell Cancer Collaborative Group;[1] and histology (seminoma …show more content…
Rare diseases range from cystic fibrosis and haemophilia to Angelman Syndrome, with an incidence of about 1 in 15,000, to Opitz trigonocephaly syndrome, which is extremely rare with about one case per million people.[5] In this review, we will focus on Primary testicular Leiomyosarcoma, which is very rare with only a few cases being reported in the literature till date. They should be differentiated from epididymo-orchitis, sarcomas of the spermatic cord and germ cell tumours. In this tumour, due to lack of data on the natural history, histological criteria for diagnosis and treatment recommendations, there is lack of proper guidelines regarding treatment. Most of the reported cases of previous literatures indicate that this may be an indolent tumour with a potential for cure if treated