Hyer Steve L, McAleese Jonathan, Harmer Clive L
Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK.
World J Surg Oncol. 2007 Jul 9;5:77. doi: 10.1186/1477-7819-5-77.
Neuroendocrine tumours (NET) are tumours arising from neuroendocrine cells of neural crest origin. They are characterised by the presence of neurosecretory granules which react positively to silver stains and to specific markers including neuron specific enolase, synaptophysin and chromogranin. Metastasis to the skin occurs infrequently but primary soft tissue NET is excessively rare.
We report our experience with 3 such cases. In the first case, the NET originated in muscle and was treated with wide surgical excision and adjuvant radiotherapy. The second case presented as a subcutaneous mass in the foot and the tumour was positive on 123I mIBG scan. She has had prolonged recurrence-free survival following primary hypo-fractionated radiotherapy. In the third case, a cutaneous nodule proved to be a NET and at surgery, lymph node disease was present. He has remained disease-free after surgical excision without the need for external beam radiotherapy.
These tumours appear to have a good prognosis. Complete excision offers potentially curative treatment. Adjuvant radiotherapy may be helpful when the tumour margin is narrow. For patients with unresectable disease or where surgery would not be appropriate, radiotherapy appears to be an effective therapeutic option.
神经内分泌肿瘤（NET）起源于神经嵴来源的神经内分泌细胞。其特征是存在对银染色以及包括神经元特异性烯醇化酶、突触素和嗜铬粒蛋白在内的特定标志物呈阳性反应的神经分泌颗粒。皮肤转移很少见，但原发性软组织NET极其罕见。
我们报告了3例此类病例的经验。第一例中，NET起源于肌肉，采用广泛手术切除和辅助放疗进行治疗。第二例表现为足部皮下肿块，肿瘤在123I间碘苄胍扫描中呈阳性。她在接受原发性低分割放疗后长期无复发生存。第三例中，一个皮肤结节被证实为NET，手术时存在淋巴结病变。他在手术切除后一直无病生存，无需外照射放疗。
这些肿瘤似乎预后良好。完整切除提供了潜在的治愈性治疗。当肿瘤切缘狭窄时，辅助放疗可能会有帮助。对于无法切除的疾病或手术不合适的患者，放疗似乎是一种有效的治疗选择。
