Hirai T, Yamanaka A, Fujimoto T, Takahashi A, Takayama Y, Yamanaka K
Department of Chest Surgery, Fukui Red Cross Hospital, 2-4-1 Tsukimi, Fukui 918-8501, Japan.
Jpn J Thorac Cardiovasc Surg. 2001 Jul;49(7):457-60. doi: 10.1007/BF02913913.
A case of multiple thymoma associated with myotonic dystrophy is reported. The patient was a 42-year-old man who had two separate encapsulated thymoma in the anterior mediastinum, at 3 cm in diameter on the right side, and at 4 cm in diameter on the left. Extended thymo-thymomectomy was performed. Microscopically, the tumor in the right thymic lobe was predominantly mixed type, and that in the left predominantly epithelial type. Neuromuscular disease appeared to develop, with severe sputum retention in the larynx and he was referred to neurology at 6 months after surgery. On examination, he presented a characteristic hatchet face, muscle atrophy, muscle weakness, percussion myotonia and grip myotonia, and diagnosis was revised to be multiple thymoma associated with myotonic dystrophy. This association is extremely rare. It is difficult to clarify whether this association was a syndrome or coincidence.
报告了一例与强直性肌营养不良相关的多发性胸腺瘤病例。患者为一名42岁男性，前纵隔有两个独立的包膜性胸腺瘤，右侧直径3厘米，左侧直径4厘米。实施了扩大胸腺-胸腺瘤切除术。显微镜下，右胸腺叶的肿瘤主要为混合型，左胸腺叶的肿瘤主要为上皮型。术后6个月出现神经肌肉疾病，伴有严重的喉部痰液潴留，遂转诊至神经科。检查时，他呈现出典型的斧状面容、肌肉萎缩、肌无力、叩击性肌强直和握力性肌强直，诊断修订为与强直性肌营养不良相关的多发性胸腺瘤。这种关联极为罕见。很难明确这种关联是一种综合征还是巧合。
