Damato Angela, Pusceddu Sara, Milione Massimo, Mazzaferro Vincenzo, Magli Michelle, Seregni Ettore, De Braud Filippo, Buzzoni Roberto
Tumori. 2013 Jul-Aug;99(4):e148-51. doi: 10.1177/030089161309900422.
The incidence of neuroendocrine tumors is rising, and this rise is explained by more than just better diagnostic procedures. About 85% of these neoplasms arise in gastrointestinal or pulmonary sites, but cases where the location is more unusual also occur in clinical practice. The tailgut cyst is a rare entity well described in the medical literature, but a neuroendocrine tumor within such a cyst is a very rare event, with about 30 cases described in the literature to date. In this report we present the case of a young woman with this unusual diagnosis. The characteristics of the case differ from most previous case reports in a few respects: the patient was a young rather than middle-aged female; she had a presacral mass with a significant solid component; at diagnosis, there was evidence of a lytic lesion in the coccyx. Despite this particular medical presentation, radical surgery was accomplished. In this disease the greatest risk is local relapse, but adjuvant radiotherapy may compromise the patient's fertility. We therefore opted for strict control only, but this decision might be debatable.
神经内分泌肿瘤的发病率正在上升，这种上升不仅仅是因为诊断程序的改进。这些肿瘤约85%起源于胃肠道或肺部，但在临床实践中也会出现位置更为罕见的病例。尾肠囊肿是医学文献中描述得很清楚的一种罕见实体，但囊肿内的神经内分泌肿瘤却是极为罕见的事件，迄今为止文献中仅描述了约30例。在本报告中，我们介绍了一位患有这种罕见诊断的年轻女性病例。该病例的特征在几个方面与大多数先前的病例报告不同：患者是年轻女性而非中年女性；她有一个骶前肿块，伴有明显的实性成分；诊断时，尾骨有溶骨性病变的证据。尽管有这种特殊的临床表现，但还是成功进行了根治性手术。在这种疾病中，最大的风险是局部复发，但辅助放疗可能会损害患者的生育能力。因此，我们仅选择了严格观察，但这一决定可能存在争议。
