Dörr-Jerat Niels Michael, May Claus, Knolle Jürgen, Schmidt Sylke, Krüger Marcus
Department of Thoracic Surgery, Martha-Maria Hospital Halle, Dölau, Germany.
Department of Pathology, Martha-Maria Hospital Halle, Dölau, Germany.
Front Surg. 2024 Jun 27;11:1393159. doi: 10.3389/fsurg.2024.1393159. eCollection 2024.
We present the case of a patient with recurrent bilateral hemothorax. After misdiagnosis despite several histological samples, a pleural manifestation of epithelioid angiosarcoma was diagnosed by further immunohistological staining. Based on this situation, we aim to sensitize the reader to this rare disease.
A 73-year-old fully conscious woman presented with dyspnea for 3 days. She was in stable general condition, pain was denied, she had a history of cigarette smoking, she had no cardiopulmonary events, and she was not receiving any anticoagulation medication. Physical examination revealed decreased breath sounds on the left side, and her hemoglobin level was 7.0 mmol/L.
The initial chest x-ray showed a left-sided effusion. Hemothorax was then diagnosed. Further investigation revealed no evidence of malignancy (CT, EBUS, cytology, etc.). VATS was performed, and biopsies of pleural lesions did not reveal congruent findings for the hemothorax. Due to recurrent bilateral hemothorax with the need for erythrocyte transfusion, the patient underwent several operations, including histological sampling, without evidence of malignancy. After further processing, an additional pathological report revealed an epithelioid angiosarcoma defined by massively proliferating epithelioid cells strongly positive for ERG and CD31 and negative for CD34. The neoplastic cells coexpressed D2-40 (podoplanin). Finally, due to multiple cerebral metastases, palliative therapy was indicated.
Physicians and pathologists treating spontaneous hemothorax need to have broad knowledge of the possible, sometimes rare, etiologies. If the clinical course and intraoperative findings do not agree with the histopathological results, this finding must be questioned, and further immunohistochemical staining is mandatory. Thus, in the case of recurrent hemothorax, angiosarcoma of the pleura should also be considered for differential diagnosis.
我们报告一例复发性双侧血胸患者的病例。尽管进行了多次组织学检查仍被误诊，经进一步免疫组织化学染色后诊断为上皮样血管肉瘤的胸膜表现。基于这种情况，我们旨在提高读者对这种罕见疾病的认识。
一名73岁意识清醒的女性因呼吸困难3天就诊。她的一般状况稳定，否认疼痛，有吸烟史，无心肺疾病史，未接受任何抗凝药物治疗。体格检查发现左侧呼吸音减弱，血红蛋白水平为7.0 mmol/L。
初步诊断、干预措施及结果：最初的胸部X线显示左侧胸腔积液，随后诊断为血胸。进一步检查未发现恶性肿瘤证据（CT、EBUS、细胞学检查等）。进行了电视辅助胸腔镜手术（VATS），胸膜病变活检未发现与血胸相符的结果。由于复发性双侧血胸且需要输注红细胞，患者接受了多次手术，包括组织学取样，但均未发现恶性肿瘤证据。经过进一步处理，一份额外的病理报告显示为上皮样血管肉瘤，其特征为大量增殖的上皮样细胞，ERG和CD31呈强阳性，CD34呈阴性。肿瘤细胞共表达D2-40（足板蛋白）。最后，由于出现多处脑转移，指示进行姑息治疗。
治疗自发性血胸的内科医生和病理学家需要对可能的病因（有时是罕见病因）有广泛的了解。如果临床过程和术中发现与组织病理学结果不一致，必须对这一发现提出质疑，并进行进一步的免疫组织化学染色。因此，对于复发性血胸病例，也应考虑胸膜血管肉瘤进行鉴别诊断。
