Muzi M G, Rulli F, Federico F
Cattedra di Chirurgia Generale, Università di Roma Tor Vergata.
Acta Biomed Ateneo Parmense. 2000;71(5):135-40.
Primary angiosarcoma of the spleen is very rare and only 143 cases have previously been reported. The pathogenesis is unknown. The clinical aspects are variable, but loss of weight, anaemia, splenomegaly and liver metastases are frequently present. The age range is generally 18 to 93 years; only four of the reported patients were under 20 (Chen KTK). The prognosis is very poor in any case and survival isn't more than two years: wherever the spleen undergoes spontaneous rupture the survival should be less than six months. Patients with or without metastatic disease may be treated by chemotherapy but with poor results. Radiotherapy is used for the pain from bone metastasis. We report the clinical case concerning a 79-years-old man with liver metastases and a 5-cm lesion in the spleen, where a subcapsular rupture was suspected.
脾脏原发性血管肉瘤非常罕见,此前仅有143例病例报告。其发病机制尚不清楚。临床症状多样,但体重减轻、贫血、脾肿大和肝转移较为常见。发病年龄一般在18至93岁之间;报告的患者中只有4例年龄在20岁以下(陈KTK)。无论如何,预后都非常差,生存期不超过两年:脾脏一旦发生自发性破裂,生存期应少于6个月。有或无转移疾病的患者均可接受化疗,但效果不佳。放疗用于缓解骨转移引起的疼痛。我们报告了一例临床病例,患者为一名79岁男性,有肝转移,脾脏有一个5厘米的病灶,怀疑有包膜下破裂。