Kas József, Csekeő Attila, Fehér Csaba, Vágvölgyi Attila, Grmela Gábor, Varga Judit, Rozgonyi Zsolt, Soltész Ibolya, Fillinger János, Pápai Zsuzsanna, Lahm Erika, Vadász Pál
Mellkassebészet, Országos Korányi Pulmonológiai Intézet Budapest, Pihenő út 1., 1529.
Pulmonológia, Fejér Megyei Szent György Oktató Kórház Székesfehérvár.
Orv Hetil. 2018 Jan;159(4):149-153. doi: 10.1556/650.2018.30925.
Infrequent solitary fibrous tumours of the pleura are associated with hypoglycaemia only in a few percent of the cases; this condition is called Doege-Potter syndrome, named after its first descriptors. Our 63 years old male patient has previously undergone clinical treatment for intrathoracic fluid accumulation on the left side caused by a giant tumour-like mass in the lower left lobe detected by CT scan. In the course of further investigations performed due to increasing load-induced dyspnoea, lung core biopsy verified low grade sarcoma in the tumour. Tumour board suggested surgery. The patient was transferred from the intensive care unit into the operation theater due to increasing dyspnoea and repeated hypoglycaemic periods in rapidly worsening general condition. Pneumonectomy and removal of the tumour was performed on the left side. Histology showed solitary fibrous tumour of the pleura corresponding to Doege-Potter syndrome. The patient was discharged without complications and underwent adjuvant chemotherapy due to pleural dissemination of the tumour observed intraoperatively. One year after surgery the patient underwent surgical removal of a locally recurrent tumour. In spite of repeated chemotherapy local and multiplex contralateral pulmonary progression was observed. Three-year survival was noted from the time of the first surgery. Orv Hetil. 2018; 159(41): 149-153.
罕见的胸膜孤立性纤维瘤仅在少数病例中与低血糖有关;这种情况被称为多伊格 - 波特综合征,以其最初的描述者命名。我们63岁的男性患者此前因CT扫描发现左下叶巨大肿瘤样肿块导致左侧胸腔积液而接受了临床治疗。由于负荷诱导的呼吸困难加重而进行的进一步检查过程中,肺芯活检证实肿瘤为低级别肉瘤。肿瘤委员会建议手术。由于呼吸困难加剧且全身状况迅速恶化,患者反复出现低血糖期,从重症监护病房转入手术室。在左侧进行了肺切除术和肿瘤切除术。组织学显示为符合多伊格 - 波特综合征的胸膜孤立性纤维瘤。患者无并发症出院,因术中观察到肿瘤胸膜播散而接受辅助化疗。手术后一年,患者接受了局部复发性肿瘤的手术切除。尽管进行了反复化疗,但仍观察到局部和对侧肺部多处进展。自首次手术起记录到三年生存率。《匈牙利医学周报》。2018年;159(41):149 - 153。