Böthig R, Ahyai S, Kühn K, Pramono S
Abt. Neuro-Urologie, Berufgenossenschaftliches Unfallkrankenhaus Hamburg, Hamburg.
Aktuelle Urol. 2008 Jan;39(1):64-7. doi: 10.1055/s-2007-959216.
Aggressive angiomyxoma (AA) is a rare mesenchymal tumour of the connective tissue of the pelvis, which was described mainly in women in their reproductive period of life. Until now 45 cases of AA in men are documented with predominantly inguinal, parafunicular or scrotal localisation. These tumours slowly infiltrate the adjacent tissue and since symptoms are noticed only later these tumours have reached a considerable size at the time of diagnosis. In contrast to their benign histological appearance and almost entire absence of metastasis AA tends to (multiple) relapse.
We report on a 46-year-old male with a large tumour in the perineum. After complete resection, histological analysis revealed an AA. 26 months after surgery there is no evidence of relapse.
AA has to be considered as a possible diagnosis for obscure tumours of the pelvis. Since these tumours tend to relapse, margin-negative resection is most important. Also in cases of relapse, secondary excision should be achieved within healthy tissue. Adjuvant hormonal chemotherapy or radiation can be considered in cases of multiple relapse. Because of the risk of local recurrence rather than metastasis, these patients need a long-term follow up.
侵袭性血管黏液瘤（AA）是一种罕见的盆腔结缔组织间叶肿瘤，主要见于育龄期女性。目前有45例男性AA的病例记录，主要位于腹股沟、精索旁或阴囊部位。这些肿瘤会缓慢浸润相邻组织，由于症状出现较晚，因此在诊断时肿瘤已发展到相当大的尺寸。尽管其组织学表现为良性且几乎无转移，但AA仍倾向于（多次）复发。
我们报告一例46岁男性，会阴部有一个大肿瘤。完整切除术后，组织学分析显示为AA。术后26个月无复发迹象。
对于盆腔不明原因的肿瘤，AA应被视为一种可能的诊断。由于这些肿瘤容易复发，切缘阴性的切除最为重要。对于复发的病例，二次切除也应在健康组织内进行。对于多次复发的病例，可考虑辅助激素化疗或放疗。由于存在局部复发而非转移的风险，这些患者需要长期随访。
