Linear spiradenoma with focal malignant transformation

Linear spiradenoma with focal malignant transformation
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DOI:
10.1046/j.1468-3083.2003.00779.x
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发表时间:
2003-05-01
影响因子:
9.2
通讯作者:
Hein, R
Hein, R
中科院分区:
医学2区
文献类型:
--
作者:
Braun-Falco, M;Bonel, H;Hein, R

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恶性螺旋腺瘤是一种极其罕见的附件肿瘤,其临床特征是先前存在的、长期存在的良性螺旋腺瘤迅速增大。显微镜检查通常揭示良性螺旋腺瘤和恶变区域之间的连续体。生物学行为是不可预测的,应被视为潜在致命的肿瘤。治疗建议需要根治性手术切除。报道的一名 23 岁女性病例说明了几乎覆盖小腿正面的大线状螺旋腺瘤内 1.5 厘米结节的恶变。回顾文献,多发性螺旋腺瘤的恶变发生率似乎略高于单发性螺旋腺瘤。与完全切除所有螺旋腺瘤结节相比,尚不清楚仅限于恶性区域的切除是否是明智的治疗选择。在本例中,转化区域被切除。 2年后,患者未再复发。
Malignant spiradenoma is an exceedingly rare adnexal tumour clinically characterized by rapid enlargement of a pre-existing, long-standing benign spiradenoma. Microscopic examination typically reveals a continuum between benign spiradenoma and areas of malignant transformation. Biological behaviour is unpredictable and it should be regarded as a potentially lethal neoplasm. Treatment recommendations require radical surgical excision. The reported case of a 23-year-old female illustrates the malignant transformation of a 1.5 cm nodule within a large linear spiradenoma almost covering the frontal aspect of the lower leg. In reviewing the literature, malignant transformation seems to occur slightly more often in multiple than in solitary spiradenomas. It is unclear whether excision restricted to the malignant area is an advisable treatment option in comparison to total excision of all spiradenoma nodules. In the presented case, the transformed area was excised. The patient is free of recurrence after 2 years.