Onyszczuk Magdalena
Department of Pathomorphology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland.
Medicine (Baltimore). 2025 Apr 25;104(17):e42106. doi: 10.1097/MD.0000000000042106.
Angiosarcoma is a malignant endothelial cell tumor of vascular or lymphatic origin and accounts for approximately 2% of all soft tissue neoplasms. Primary pulmonary angiosarcoma (PPA) is extremely rare, with <30 cases reported in the literature. Moreover, because of non clinical manifestation and low positive diagnostic rate of traditional, noninvasive or minimally invasive diagnostic methods, the early diagnosis of this neoplasm is really challenging.
A 73-year-old woman initially presented with anemia and hemoptysis, which has occurred over a period of 4 weeks before admission. Subsequently, the patient experienced progressive weakness, anxiety attacks, and loss of appetite.
The bronchoscopy and chest X-ray failed to detect the cause of the symptoms. The patient was initially treated for acute interstitial pneumonia. Subsequent video-assisted thoracic surgery with parietal pleura sampling identified the presence of neoplastic tissue of unknown primary origin.
Transfusion of red blood cell concentrate and high-flow oxygen therapy were administered. In view of poor performance status and unfavorable prognosis, a decision was made not to escalate the therapy. The patient died 21 days after admission. An autopsy was ordered to determine the exact cause of death and revealed multiple nodular lesions scattered in both lung parenchyma and on the pleural surface. The final diagnosis of poorly differentiated epithelioid PPA was made on the basis of histopathological and immunohistochemical postmortem examination.
Presented case cautions that PPA should be considered in patient with hemoptysis, negative bronchoscopy and non-characteristic radiological findings. When the disease is suspected, biopsy and microscopic examination should be implemented to confirm the diagnosis as soon as possible and apply timely treatment. Moreover, due to the small number of PPA cases there is no standard diagnostic and treatment protocol available. Thus, further investigations and sharing experiences, may aid better understanding of this malignancy and lead to improvement in patients' survival.
血管肉瘤是一种起源于血管或淋巴管的恶性内皮细胞肿瘤，约占所有软组织肿瘤的2%。原发性肺血管肉瘤（PPA）极为罕见，文献报道的病例不足30例。此外，由于其无临床表现，且传统的非侵入性或微创诊断方法的阳性诊断率较低，该肿瘤的早期诊断极具挑战性。
一名73岁女性最初出现贫血和咯血症状，入院前4周内持续出现。随后，患者出现进行性虚弱、焦虑发作和食欲不振。
支气管镜检查和胸部X线检查未能发现症状原因。患者最初被诊断为急性间质性肺炎并接受治疗。随后的电视辅助胸腔镜手术及壁层胸膜采样发现了原发部位不明的肿瘤组织。
给予浓缩红细胞输血和高流量氧疗。鉴于患者身体状况较差且预后不良，决定不再加强治疗。患者入院21天后死亡。进行尸检以确定确切死因，结果显示双肺实质和胸膜表面散在多个结节性病变。根据组织病理学和免疫组织化学尸检结果，最终诊断为低分化上皮样PPA。
本病例提示，对于咯血、支气管镜检查阴性且影像学表现不典型的患者，应考虑PPA的可能。怀疑该病时，应尽快进行活检和显微镜检查以确诊并及时治疗。此外，由于PPA病例数量较少，尚无标准的诊断和治疗方案。因此，进一步的研究和经验分享可能有助于更好地了解这种恶性肿瘤，提高患者生存率。
