Department of Breast Surgery, The First Affiliated Hospital, College of Medicine, Jiaxing College, Jiaxing, P. R. China.
Breast Care (Basel). 2012 Dec;7(6):490-2. doi: 10.1159/000345466.
Poland's Syndrome (PS) is a rare congenital syndrome characterized by the unilateral partial or complete absence of the sternocostal head of the pectoralis major muscle and ipsilateral brachysyndactyly or syndactyly of the fingers. It has been reported to be associated with other diseases, but PS accompanied by neurofibroma has rarely been reported.
We report a rare case of a 16-year-old man with neurofibroma of the left breast accompanied by PS. Physical examination showed a mass in the left breast with skin hyperpigmentation and increased body hair. Intraoperative exploration showed a mass measuring 3-4 cm between the dysplastic pectoralis major and minor muscles. Pathologic examination of the resected mass showed cutaneous diffuse neurofibroma. A simple left mastectomy was performed. The postoperative course was uneventful with no evidence of recurrence at 4 months.
Neurofibroma may also be accompanied by PS and should be differentiated from gynecomastia when a tumor grows in the breast.
波兰氏综合征（PS）是一种罕见的先天性综合征，其特征为单侧部分或完全缺失胸大肌的胸骨肋头部以及同侧短指（趾）或并指（趾）。它已被报道与其他疾病有关，但伴有神经纤维瘤的 PS 则很少见。
我们报告了一例罕见的 16 岁男性左侧乳腺神经纤维瘤伴 PS 病例。体格检查显示左侧乳腺有一肿块，伴有皮肤色素沉着和多毛。术中探查显示在发育不良的胸大肌和胸小肌之间有一个 3-4 厘米的肿块。切除肿块的病理检查显示为皮肤弥漫性神经纤维瘤。行单纯左侧乳房切除术。术后 4 个月无复发，无并发症。
神经纤维瘤也可能伴有 PS，当肿瘤在乳房中生长时，应与男性乳房发育相鉴别。
