Wapshott Taylor, Schammel Christine M G, Schammel David P, Rezeanu Luminita, Lynn Michael
University of South Carolina School of Medicine-Greenville, 607 Grove Road, Greenville, SC 29605, USA.
Pathology Associates, 8 Memorial Medical Court, Greenville, SC 29605, USA.
J Clin Neurosci. 2018 Aug;54:128-135. doi: 10.1016/j.jocn.2018.04.060. Epub 2018 May 21.
Sarcomas make up 1% of all cases of adult cancer, with 5-10% of those classified as undifferentiated pleomorphic sarcomas (UPS/PUS) and 0.1-4.3% primary intracranial sarcomas. Intracranial undifferentiated sarcoma is characterized by an earlier age of onset and generally poorer prognosis compared to extracranial undifferentiated sarcomas. Current therapies involve surgical excision with wide margins and radiotherapy, with minimal data available regarding the efficacy of chemotherapy.
A 79-year-old man with a history of remote superficial bladder cancer presented with a large frontal scalp lesion. A biopsy was initially attempted by a dermatologist in the outpatient setting, but a follow-up CT scan revealed a skull-eroding, enhancing soft tissue lesion. Neurosurgical treatment revealed an undifferentiated sarcoma. The patient underwent adjuvant radiation therapy of 59.4 Gy fractionated over 45 days following surgery. Follow-up brain MRIs at 1-, 6-, 9-, 12-, 15-, 21-, and 27 months after surgery have not shown any indications of local recurrence or tumor metastasis. Despite the high propensity that undifferentiated sarcomas have for recurrence and metastasis and the patient's advanced age, this patient remains uniquely disease-free.
We provide a description of an unusual case and comprehensive literature review of UPS to clarify the hallmarks of the disease, identify the difficulties in diagnosis, and provide a summary of therapies employed in the literature with their corresponding patient outcomes.
肉瘤占成人癌症病例的1%，其中5 - 10%为未分化多形性肉瘤（UPS/PUS），原发性颅内肉瘤占0.1 - 4.3%。与颅外未分化肉瘤相比，颅内未分化肉瘤的特点是发病年龄较早，预后通常较差。目前的治疗方法包括广泛切除手术和放射治疗，关于化疗疗效的可用数据极少。
一名有远处浅表膀胱癌病史的79岁男性出现一个巨大的额部头皮肿物。皮肤科医生最初在门诊尝试进行活检，但后续的CT扫描显示有一个侵蚀颅骨、强化的软组织肿物。神经外科治疗显示为未分化肉瘤。患者在术后45天内接受了总量为59.4 Gy的辅助放疗。术后1、6、9、12、15、21和27个月的脑部MRI随访未显示任何局部复发或肿瘤转移的迹象。尽管未分化肉瘤有很高的复发和转移倾向且患者年龄较大，但该患者仍无疾病复发。
我们描述了一个不寻常的病例，并对UPS进行了全面的文献综述，以阐明该疾病的特征，确定诊断中的困难，并总结文献中采用的治疗方法及其相应的患者预后情况。
