Tröbs R-B, Krauss M, Geyer C, Tannapfel A, Körholz D, Hirsch W
Kinderchirurgische Klinik, Marienhospital Herne, Ruhr-Universität Bochum, Widumer Strasse 8, 44627 Herne.
Klin Padiatr. 2007 May-Jun;219(3):146-51. doi: 10.1055/s-2007-973847.
Testicular and even more paratesticular tumours in children are rare. The aim of the study is to characterise the spectrum of these lesions with focus on the feasibility and effectiveness of testis sparing surgery. Twenty-four boys treated between 1980 and 2004 at the University Leipzig Medical Centre were evaluated. At presentation patients were between 5 months and 18 years old (median 23 months). Generally a high rate of malignant or potentially malignant tumours was observed. The majority of these tumours occurred in the first three years of age. The spectrum of testicular tumours comprised 13 germ cell tumours (6 yolk sac tumours, 6 teratomas, 1 embryonal carcinoma) and 4 sex cord stromal tumours (2 Leydig's cell, Sertoli's cell, granulosa cell). Both Leydig's cell tumours were endocrine active. Further on, we observed 3 boys with paratesticular rhabdomyosarcoma (RMS), and three with testicular and paratesticular metastases (Wilms' tumour, neuroblastoma, leukaemia). Serum alpha1-fetoprotein (AFP) was clearly elevated in 5 of 6 yolk sac tumours but remained within normal limits concerning the other entities. Human chorionic gonadotrophin was normal in all cases tested. During the observation period high inguinal orchidectomy was the surgical standard method. Dependent on tumour histology, stage and the recommended treatment schedule postoperative chemotherapy was added. Testis sparing surgery was performed in 3 boys with primary testicular tumours (2 Leydig's cell, mature cystic teratoma). Local relapses were not observed. Systemic relapses occurred in 3 cases (2 RMS, leukaemia). During a median follow up of 5 years all patients with primary testicular tumours survived event free. Meta-analysis of the recent literature revealed that testis sparing surgery is feasible and save in prepubertal boys after exclusion of a malignant tumour. If a testis sparing approach is planned, the following criteria are essential: 1. The presence of a well defined circumscribed nodule confirmed by imaging. 2. Normal levels of serum AFP and hCG. 3. The presence of sufficient healthy testicular parenchyma. However, the high rate of malignant or potentially malignant tumours suggests that high inguinal orchidectomy should remain the surgical standard of therapy.
儿童睾丸及睾丸旁肿瘤较为罕见。本研究旨在描述这些病变的范围，重点关注保留睾丸手术的可行性和有效性。对1980年至2004年间在莱比锡大学医学中心接受治疗的24名男孩进行了评估。就诊时患者年龄在5个月至18岁之间（中位数为23个月）。总体而言，观察到恶性或潜在恶性肿瘤的发生率较高。这些肿瘤大多发生在3岁之前。睾丸肿瘤谱包括13例生殖细胞肿瘤（6例卵黄囊瘤、6例畸胎瘤、1例胚胎癌）和4例性索间质肿瘤（2例Leydig细胞瘤、支持细胞瘤、颗粒细胞瘤）。2例Leydig细胞瘤均具有内分泌活性。此外，我们观察到3例睾丸旁横纹肌肉瘤（RMS）患儿，以及3例睾丸和睾丸旁转移瘤（肾母细胞瘤、神经母细胞瘤、白血病）患儿。6例卵黄囊瘤中有5例血清甲胎蛋白（AFP）明显升高，但其他病例均在正常范围内。所有检测病例的人绒毛膜促性腺激素均正常。在观察期内，高位腹股沟睾丸切除术是手术的标准方法。根据肿瘤组织学、分期和推荐的治疗方案，术后加用化疗。对3例原发性睾丸肿瘤（2例Leydig细胞瘤、成熟囊性畸胎瘤）患儿实施了保留睾丸手术。未观察到局部复发。3例出现全身复发（2例RMS、白血病）。在中位随访5年期间，所有原发性睾丸肿瘤患者均无事件生存。对近期文献的荟萃分析表明，在排除恶性肿瘤后，保留睾丸手术在青春期前男孩中是可行且安全的。如果计划采用保留睾丸的方法，以下标准至关重要：1. 影像学证实存在边界清晰的局限性结节。2. 血清AFP和hCG水平正常。3. 存在足够的健康睾丸实质。然而，恶性或潜在恶性肿瘤的高发生率表明，高位腹股沟睾丸切除术仍应作为手术治疗的标准。
