Kamocki Zbigniew, Steward Agnieszka, Zaręba Konrad Piotr, Kukliński Adam, Kędra Boguław
Department of Medical Pathomorphology, Medical University of Bialystok, Poland.
Contemp Oncol (Pozn). 2013;17(2):218-21. doi: 10.5114/wo.2013.34628. Epub 2013 Apr 29.
Splenic angiosarcoma has been presented as an extremely rare malignant tumour. Amongst the rarest of all neoplasms, primary splenic angiosarcoma comprises 0.14-0.25 of all annually reported cases per one million persons Splenic angiosarcoma was first described in 1879 by T. Langerhans. The clinical symptoms and diagnostic values associated with splenic angiosarcoma are extremely variable. Nonetheless, majority of the patients (75%) complain of abdominal pain, and a quarter to one-third present with rupture of the involved organ. We hereby report our experience with two splenic angiosarcoma cases, which despite being similar in their diagnosis, nonetheless present with an entirely different clinical picture. Our first case, whereby the patient presented with a liver that was also affected by the angiosarcoma of the spleen. In the second case however, although the patient did show evidence of metastasis to the abdominal cavity as well as the liver, she also suffered from primary adenocarcinoma of the colon. To our knowledge, no previous article has made mention of primary splenic angiosarcoma existing independently of another primary neoplasm. Both may be found in common practice, and ought to be highlighted for their significance as such.
脾血管肉瘤是一种极为罕见的恶性肿瘤。在所有肿瘤中最为罕见，原发性脾血管肉瘤占每年每百万人口报告病例的0.14 - 0.25。脾血管肉瘤于1879年由T. 朗格汉斯首次描述。与脾血管肉瘤相关的临床症状和诊断价值差异极大。尽管如此，大多数患者（75%）主诉腹痛，四分之一到三分之一的患者出现受累器官破裂。我们在此报告两例脾血管肉瘤病例的经验，这两例尽管诊断相似，但临床表现却完全不同。我们的第一例病例中，患者的肝脏也受到了脾血管肉瘤的影响。然而在第二例病例中，尽管患者确实有腹腔及肝脏转移的证据，但她还患有原发性结肠癌。据我们所知，此前没有文章提及原发性脾血管肉瘤独立于另一种原发性肿瘤而存在。这两种情况在常规实践中都可能出现，因此其重要性应予强调。
