Primary pure angiosarcoma of the testis: a vanishingly rare malignancy. Case report and literature review

BACKGROUND: Primary pure angiosarcoma of the testis is an exceptionally rare testicular malignancy, which is poorly understood. We present the fifth and youngest case in the current medical literature. Additionally, all cases of angiosarcoma of the testicle, both occurring with associated germ cell tumour and without, were compared in an extended tabular format. CASE PRESENTATION: A 56-year old man presented with unilateral scrotal pain, swelling and erythema. Ultrasonography revealed two testicular lesions with a high suspicion of malignancy but serum tumour markers were negative. A radical orchidectomy was performed with clear surgical margins. Diagnosis of primary pure angiosarcoma of the testis was confirmed on subsequent histopathology. CONCLUSIONS: Primary pure angiosarcoma is a rare testicular neoplasm. We present the fifth case in the literature. Clinical and radiological features are non-specific. The diagnosis is purely histological, with the pathologist choosing immunohistochemistry based on abnormal morphology. Local invasiveness is variable but metastatic sites are typical for extra-gonadal angiosarcomas. Primary pure testicular angiosarcoma diagnosis confers a relatively better prognosis compared to angiosarcoma arising in the context of a testicular germ cell tumour. While extra-gonadal angiosarcomas are associated with high rates of local recurrence following resection, in all cases of testicular angiosarcoma there were no local recurrences following radical orchidectomy. Surgical resection remains the most effective treatment for both subtypes of testicular angiosarcoma.