Ashmore D L, Dasgupta D
Hull Royal Infirmary, Hull and East Yorkshire Hospitals NHS Trust, Hull, UK.
Ann R Coll Surg Engl. 2020 May;102(5):e105-e106. doi: 10.1308/rcsann.2020.0035. Epub 2020 Apr 1.
Primary malignant fibrous histiocytoma, now classified as pleomorphic undifferentiated sarcoma, is the most common soft-tissue sarcoma in adult life. Primary splenic pleomorphic undifferentiated sarcoma is extremely rare and aggressive, and is associated with a poor prognosis; only 14 cases of splenic pleomorphic undifferentiated sarcoma have been documented in the English literature. We discuss a case of a 56-year-old woman with iron-deficiency anaemia, early satiety and left upper-quadrant pain, who was preoperatively diagnosed with a large splenic cyst following thorough investigation. This was excised in an elective procedure. Unfortunately, histology confirmed splenic pleomorphic undifferentiated sarcoma. Following a review and summary of the literature, we discuss key differentials between splenic cysts and splenic pleomorphic undifferentiated sarcoma. This case highlights that iron-deficiency anaemia is unusual in splenic cysts and more sinister causes must be considered.
原发性恶性纤维组织细胞瘤，现归类为多形性未分化肉瘤，是成人中最常见的软组织肉瘤。原发性脾脏多形性未分化肉瘤极为罕见且具有侵袭性，预后较差；英文文献中仅记载了14例脾脏多形性未分化肉瘤病例。我们讨论了一例56岁女性患者，该患者患有缺铁性贫血、早饱感和左上腹疼痛，经过全面检查后术前诊断为巨大脾囊肿。该囊肿在择期手术中被切除。不幸的是，组织学检查证实为脾脏多形性未分化肉瘤。在对文献进行回顾和总结后，我们讨论了脾囊肿与脾脏多形性未分化肉瘤之间的关键鉴别要点。该病例强调，缺铁性贫血在脾囊肿中并不常见，必须考虑更严重的病因。
