皮肌炎合并贲门癌的临床及病理特点分析

Clinical and Pathological Analysis of Dermatomyositis Combined with Cardiac Orifice Cancer

目的分析皮肌炎(DM)合并贲门癌患者的临床及病理特点。方法收集1例DM合并贲门癌患者的临床表现及开放式骨骼肌活体组织检查(活检)、组织化学染色病理与手术切除贲门病变组织的病理结果资料,进行回顾性分析。结果本例为老年男性患者,临床表现为四肢近端肌肉伴吞咽肌无力2个月。查体:双上眼睑轻度水肿性紫红斑,咀嚼肌、吞咽肌肌力弱,髂腰肌、四肢近端肌肌力Ⅲ+级、远端肌肌力Ⅴ-级。实验室检查:红细胞沉降率46 mm/h、血肌酸激酶145 U/L、补体C40.44 g/L,均增高,C反应蛋白水平正常。肌电图:部分被检肌肉可见震颤、正相电位与波幅低、时限窄多相电位。活检骨骼肌组织化学染色光镜病理:可见典型束周肌纤维萎缩,肌内膜、肌束膜、血管周围炎性细胞浸润,未见坏死肌纤维。病理诊断为DM。电子纤维胃镜示:贲门灶状黏膜腺体重度不典型增生,可疑癌变。手术切除的贲门病变组织病理诊断为腺癌。结论骨骼肌活检病理诊断是确诊DM的必备条件。中老年DM患者易并发恶性肿瘤,应积极行相关肿瘤筛查。

Objective To analyze clinical and pathological features of patients with dermatomyositis （DM） combined with cardiac orifice cancer. Methods Clinical data, skeletal muscle biopsy, pathological results of histochemistry staining and surgical resection of cardiac tissues of a patient with DM combined with cardiac orifice cancer were retrospectively analyzed. Resuits Clinical manifestations of the elderly male patient were limbs proximal muscle progressive weakness and mild dysphagia for more than 2 months. Clinical examination showed a heliotrope rash on the upper eyelids with mild oedema, the strength of masse- ter and swallowing muscle were weak, muscle strength score of iliopsoas and limbs proximal muscles were III＋ , of distal muscles were V -. Laboratory examination： erythrocyte sedimentation rate （ESR） 46 mm/h, creatine kinase （CK） 145 U/L, complement C4 0.44 g/L were increased and cross - reacting protein （CRP） was normal. Electromyography showed fibrillation poten- tials, positive sharp waves and MUPs with shorten duration and lower amplitude. Skeletal muscle histochemistry pathology： there were no necrotic fibers, typical perifascicular atrophies were observed, and inflammatory cells infiltrated in perimysium, endomysium and perivascular. Pathological diagnosis： DM. Electronic gastroscope showed cardiac focal mucosa glands had severe atypical hyperplasia and suspicious canceration. Pathological analysis of cardiac tissue： glandular cancer. Conclusion The skeletal muscle biopsy and pathology analysis is a necessary method to diagnose DM. Elderly patients with DM tend to be complicated by malignant tumor, and should be given relative cancer screening.