表现为胸腔积液的胸膜孤立性髓系肉瘤一例并文献复习

Pleural effusion as the first manifestation of pleural isolated myeloid sarcoma:a case report and literature review

目的探讨胸膜孤立性髓系肉瘤(isolated myeloid sarcoma,IMS)的临床特征。方法报道1例表现为单侧胸腔积液的胸膜孤立性髓系肉瘤(pleural isolated myeloid sarcoma,PIMS)。以"髓系肉瘤"、"胸腔积液"和"myeloid sarcoma""pleural effusions"为检索词在知网、万方数据库及PubMed数据库检索相关文献并进行分析。结果患者男,59岁,以"右侧胸痛2个月,加重伴胸闷、气短2周"为主诉入院。胸部CT示右侧胸腔积液。积液流式细胞学示CD34+异常细胞占有核细胞的37.6%。内科胸腔镜胸膜活检病理为淋巴组织增生性病变。胸膜及细胞块病理切片免疫组织化学染色CD34、CD117阳性,诊断为PIMS。共检索到资料相对完整的英文文献2篇,各报道1例患者,加上本例共3例,男2例,女1例,年龄分别为59、51和56岁。1例表现为右上腹痛、恶心,体重下降,胸腔积液中见大量低分化的恶性肿瘤细胞,十二指肠、胆囊、肠系膜淋巴结活检证实为IMS。另1例表现为气短、上颚区进行性肿胀6周,胸腔积液中髓系原始细胞占0.82,齿龈活检证实为IMS。结论表现为胸腔积液的PIMS罕见,胸腔积液细胞学与胸膜组织病理学检查有助于该病的诊断。

Objective To analyze the clinic features of isolated myeloid sarcoma(IMS)involving the pleural cavity.Methods A case of pleural isolated myeloid sarcoma(PIMS)with pleural effusion as the first manifestation was described.The related cases in literatures were reviewed with“myeloid sarcoma”and“pleural effusions”as the keywords to search China HowNet,Wanfang database and PubMed database.Results A 59-year-old man complained of right chest pain for 2 months and worsening pain with distress and shortness of breath for 2 weeks.The chest CT scan showed pleural effusion on the right side.Flow cytometric analysis of pleural fluid showed that a population of blasts with CD34 expressing was 37.6%of the total nucleated cells.The pleural biopsy through medical thoracoscopy indicated lymphoproliferative lesions by pathological examination.Immunohistochemistry was performed on pleural histological sections and cell blocks of pleural effusions,which showed CD34 and CD117 positive expression.The diagnosis of PIMS was finally made.Two literature papers with 2 complete cases were found and reviewed.The 3 cases were analyzed.There were 2 males and 1 female.The age was 59,51,56 years respectively.One case was a patient with 3 weeks of right upper quadrant and epigastric pain,nausea,and weight loss.Cytological examination of the pleural fluid showed numerous poorly differentiated malignant cells.Histology from an open laparotomy in duodenal biopsies,gallbladder,and mesenteric lymph nodes supported the diagnosis of IMS.The other case was a patient with 6 weeks of dyspnea and a large swelling in the upper vestibular region.Thoracentesis showed 82%myeloid blasts in the pleural fluid.A gingival biopsy showed a diffuse infiltration by cells with a blastic appearance and supported IMS.Conclusion PIMS was a very rare cause of pleural effusions.The cytological and histopathological evidences were useful to diagnose IMS involving the pleural cavity.