Królczyk Jarosław, Fornal Maria, Janicki Kazimierz, Wilkołek Piotr, Krochin Marek, Tracz Wiesława
I Katedra i Kliniki Chorób Wewnetrznych Collegium Medicum Uniwersytetu Jagiellońskiego w Krakowie, 31-531 Kraków, ul. Sniadeckich 10.
Przegl Lek. 2003;60(2):123-5.
The case of a young 32 year old male with a primary cardiac angiosarcoma is reported. The neoplasm manifested itself by a quickly increasing cardiac tamponade but without metastases. The nonradical resection of the tumor was made because of local invasion with tumor tissue. The patient was followed-up for 3 years after the surgery and no recurrence of the malignant process was observed. Cardiac angiosarcoma is a very rare malignant tumor of soft tissues. In spite of significant progress in clinical treatment, for a patient diagnosed antemortem with cardiac angiosarcoma, the long-term expectations are usually very poor. Commonly known risk factors for this group of neoplasms (haemangioma of skin, chroniclymphedema, chronic post-tuberculosis pleurisy, X-ray, thorium dioxide) cannot be easily associated with the primary cardiac angiosarcoma cases. The search for chromosomal anomalies and gene mutations leading to cardiac angiosarcoma is ongoing. There is hope that recently obtained evidence for mutation of the p53gen, will provide a better understanding of this heart neoplasia.
报告了一例32岁原发性心脏血管肉瘤的年轻男性病例。该肿瘤表现为迅速加重的心包填塞,但无转移。由于肿瘤组织局部浸润,进行了肿瘤的非根治性切除。术后对患者进行了3年随访,未观察到恶性病变复发。心脏血管肉瘤是一种非常罕见的软组织恶性肿瘤。尽管临床治疗取得了显著进展,但对于生前诊断为心脏血管肉瘤的患者,长期预后通常很差。这组肿瘤常见的危险因素(皮肤血管瘤、慢性淋巴水肿、慢性结核后胸膜炎、X射线、二氧化钍)不易与原发性心脏血管肉瘤病例相关联。导致心脏血管肉瘤的染色体异常和基因突变的研究正在进行中。希望最近获得的p53基因发生突变的证据,将有助于更好地了解这种心脏肿瘤。