原发性睾丸及睾丸旁组织骨肉瘤:文献回顾与更新

A. Venyo
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引用次数: 0

摘要

睾丸和睾丸旁组织骨肉瘤是一个术语,指的是含有影响睾丸或睾丸旁组织的纯类骨恶性细胞的恶性肿瘤。这些肿瘤往往表现为阴囊内或睾丸内的非特异性肿块。它也可能与鞘膜积液有关。临床检查可能发现睾丸或受影响的睾丸旁组织中有一个硬到硬的肿块。腹股沟腺可能正常,但在极少数情况下,晚期病例可能有淋巴结肿大。如果有相关的鞘膜积液,可能有鞘膜积液的临床检查证据。全血细胞计数、血清尿素和电解质以及肝功能测试将趋于正常,除非存在需要相应调查的非相关病理。然而,如果存在多个肝转移,肝功能测试结果可能显示肝功能测试的一个或多个元素出现紊乱。血清β-人绒毛膜促性腺激素、甲胎蛋白和乳酸脱氢酶水平将趋于正常,除非骨肉瘤与偶尔发生的同步生殖细胞肿瘤有关。在睾丸肿瘤的情况下,放射学成像可以显示睾丸内或病变部位阴囊内有不均匀肿块的证据,并且肿块往往与钙化有关。治疗倾向于仅涉及睾丸的局限性肿瘤的睾丸根治术,但睾丸旁骨肉瘤倾向于通过睾丸根治术加上睾丸旁组织的整体切除来治疗。如果有转移的证据,那么可以给予辅助治疗。对切除的睾丸骨肉瘤标本进行大体检查,往往会发现一个边界清晰、坚固、坚实、白褐色、膨胀性肿块,压迫了睾丸周围的薄壁组织。睾丸标本原发性骨肉瘤的显微镜检查往往显示多形性圆形至纺锤形细胞片,其中含有突出的核仁、类骨区和分散的矿化区、高有丝分裂活性和非典型有丝分裂、类骨区域中有时有破骨细胞。睾丸或任何睾丸旁结构的骨肉瘤的免疫组织化学研究倾向于显示肿瘤细胞对波形蛋白呈阳性染色,但肿瘤细胞对平滑肌肌动蛋白、CD34、细胞角蛋白、结蛋白、抑制素、myo-D1和S-100蛋白呈阴性染色。
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Primary Osteosarcoma of Testis and Para-Testicular Tissues: A Review and Update of the Literature
Osteosarcoma of the testis and para-testicular tissue is a terminology that refers to a malignant tumor that contains pure osteoid forming malignant cells with no skeletal origin that affect the testis or para-testicular tissues. These tumors tends to present as an intra-scrotal or testicular mass which is non-specific. It may also present in association with a hydrocele. Clinical examination may reveal a firm to hard mass in the testis or in the affected para-testicular tissue. The inguinal glands may be normal but on rare occasions there may be lymph node enlargement in advanced cases. If there is an associated hydrocele there may be clinical examination evidence of a hydrocele. The full blood count, serum urea and electrolytes and liver function tests would tend to normal unless there are un-related pathologies which would need to be investigated accordingly. Nevertheless, if there are multiple liver metastases the liver function test results may show derangement in one or more elements of the liver function tests. The serum Beta Human Chorionic gonadotrophin, alpha fetoprotein, and lactate dehydrogenase levels would tend to normal unless the osteosarcoma is associated with a synchronous Germ cell tumor which does happen occasionally. Radiology imaging could show evidence of a heterogeneous mass within the testis in the case of a testicular tumor or in an intra-scrotal area at the site of the lesion and the mass tends to be associated with calcification. Treatment tends to be radical orchidectomy for localized tumors involving the testis only, but para-testicular osteosarcomas tend to be treated by radical orchidectomy plus en bloc excision of the para-testicular tissue. If there is evidence of metastasis then adjuvant therapy can be given. Gross examination of specimens of an excised osteosarcoma of the testis would tend to show a well-circumscribed, firm, solid, whitish tan, expansile mass which had compressed the surrounding parenchyma of the testis. Microscopic examination of primary osteosarcoma of the testis specimen would tend to show sheets of pleomorphic round to spindle cells that contain prominent nucleoli, areas of osteoid and scattered areas of mineralization, high mitotic activity and atypical mitosis, osteoid and at times osteoclasts among the area of osteoid. Immunohistochemistry studies of osteosarcoma of the testis or any para-testicular structures would tend to show the tumor cells stain positively for vimentin, but the tumor cells would stain negatively for: smooth muscle actin, CD34, cytokeratin, desmin, inhibin, myo-D1, and S-100 proteins.
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