Vörös András, Kaiser László, Somfay Attila, Pálinkás Attila
Szegedi Tudományegyetem Szent-Györgyi Albert Orvos- és Gyógyszerésztudományi Centrum, Altalános Orvostudományi Kar, Patológia Intézet, Allomás u. 2, 6720 Szeged.
Orv Hetil. 2007 Jul 8;148(27):1281-5. doi: 10.1556/OH.2007.28067.
The authors present a rare cause of pulmonary hypertension, which occurred in a 57-year-old woman. Postmortem examination discovered an adenocarcinoma with bronchioloalveolar growth pattern as a cause of severe dyspnoea, extreme pulmonary hypertension, and chronic cor pulmonale. The tumour involved all lobes of the lung. In the hilar lymph node metastasis was detectable. Histology showed tumour cell emboli in branches of the pulmonary arteries, intimal fibrosis, which was associated with fibrin precipitation and involvement of lymphatic vessels showing lymphangiosis carcinomatosa. The intrapulmonary dissemination of the adenocarcinoma could be caused by the isolated haematogenous dissemination via the thoracic duct. The authors discuss the possible pathomechanism of pulmonary hypertension and the way of tumour cell dissemination in the lung. They highlight the histological changes, which accompany the syndrome of pulmonary tumour thrombotic microangiopathy. In this case, which can be regarded rarely published in the literature, they emphasise the differential diagnostic questions.
作者报告了一例罕见的肺动脉高压病例,患者为一名57岁女性。尸检发现,细支气管肺泡生长模式的腺癌是导致严重呼吸困难、重度肺动脉高压和慢性肺源性心脏病的原因。肿瘤累及肺的所有叶。在肺门淋巴结可检测到转移。组织学检查显示肺动脉分支中有肿瘤细胞栓子、内膜纤维化,伴有纤维蛋白沉淀,且淋巴管受累表现为癌性淋巴管炎。腺癌的肺内播散可能是通过胸导管的孤立血行播散所致。作者讨论了肺动脉高压可能的发病机制以及肿瘤细胞在肺内的播散方式。他们强调了伴随肺肿瘤血栓性微血管病综合征的组织学变化。在这个文献中很少发表的病例中,他们强调了鉴别诊断问题。