Primary splenic angiosarcoma is an extremely rare and aggressive malignancy that can present with spontaneous splenic rupture and has a poor prognosis.
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Rapid mortality despite surgery in ruptured splenic angiosarcoma; leaves open need for earlier detection strategies in this rare entity.
Primary angiosarcoma of the spleen is an extremely rare malignant neoplasm of vascular origin that often has a poor prognosis. The majority of cases presents with splenic rupture and hemorrhage. The present study retrospectively analyzed the case of 77-year-old female who presented with diffuse abdominal pain and distension. During laparotomy, a huge actively bleeding spleen was identified and a splenectomy was performed. Since an accurate diagnosis could not be achieved by abdominal computed tomography, a pathological examination was performed. The patient and the family refused post-operative adjuvant chemotherapy and radiation therapy. The patient succumbed to uncontrolled hemorrhagic shock and lung metastases at 2 weeks post-surgery.
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Xu et al. (2015) studied this question.
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