*Department of Pathology and Urology, University of Miami Miller School of Medicine, Miami, FL Departments of †Pathology ‡Urology §Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD.
Am J Surg Pathol. 2015 Sep;39(9):1219-25. doi: 10.1097/PAS.0000000000000426.
Dedifferentiated liposarcoma (DDL) of the spermatic cord is rare, with limited data regarding morphology and prognosis. We analyzed the morphologic spectrum of DDL of the spermatic cord and its clinical significance in 42 patients from 1990 to 2014. The median age of the patients was 70.5 years (range: 43 to 90 y). The median tumor size was 10.9 cm (range: 3 to 30 cm). Six cases were low-grade DDL, 28 were high-grade DDL, and in 8 cases both coexisted. Five cases had osteosarcoma, and 2 had leiomyosarcoma differentiation. Low-grade DDL had a fibrosarcoma-like (n=11), myxofibrosarcoma-like (n=2), and fibrosarcoma and inflammatory-like (n=1) pattern. High-grade DDL had a fibrosarcoma-like (n=22), undifferentiated pleomorphic sarcoma-like (n=7), myxofibrosarcoma and undifferentiated pleomorphic sarcoma-like (n=4), fibrosarcoma and undifferentiated pleomorphic sarcoma-like (n=2), and myxofibrosarcoma-like (n=1) pattern. Resection margins were positive in 22, negative in 13, and unknown in 7 cases. MDM2 was positive in 24/24 cases. Two patients received neoadjuvant radiotherapy (1 with chemotherapy). Patients received postoperative radiation (n=13), reexcision (n=5) with radiation (n=3), chemotherapy (n=2), chemotherapy and radiation (n=1), no therapy (n=11), and unknown (n=6). Follow-up information was available in 31 patients (mean: 54.2 mo; median: 38.5 mo; range: 2 to 180 mo). Seven patients developed local recurrence. Two patients had systemic metastases and succumbed to disease. Recurrence developed at an average of 40.9 months (median: 24 mo; range: 12 to 100 mo) after resection. Average follow-up in patients without recurrence was 45.2 months (median: 25 mo; range: 2 to 180 mo) (P=0.87). Margin status, size, and grade did not correlate with recurrence (P>0.05). Thus, DDL of the spermatic cord includes a spectrum of morphologic patterns. Complete excision is difficult at first attempt. Local recurrence is common, and relatively few patients develop systemic disease.
精原细胞瘤去分化脂肪肉瘤（DDL）较为罕见，其形态学和预后的数据资料有限。我们分析了 1990 年至 2014 年间 42 例精原细胞瘤去分化脂肪肉瘤的形态学谱及其临床意义。患者的中位年龄为 70.5 岁（范围：43 至 90 岁）。肿瘤的中位大小为 10.9cm（范围：3 至 30cm）。6 例为低度去分化脂肪肉瘤，28 例为高度去分化脂肪肉瘤，8 例同时存在两种类型。5 例发生骨肉瘤，2 例发生平滑肌肉瘤分化。低度去分化脂肪肉瘤具有纤维肉瘤样（n=11）、黏液纤维肉瘤样（n=2）和纤维肉瘤伴炎症样（n=1）形态。高度去分化脂肪肉瘤具有纤维肉瘤样（n=22）、未分化多形性肉瘤样（n=7）、黏液纤维肉瘤和未分化多形性肉瘤样（n=4）、纤维肉瘤和未分化多形性肉瘤样（n=2）和黏液纤维肉瘤样（n=1）形态。22 例切缘阳性，13 例切缘阴性，7 例切缘情况未知。24/24 例 MDM2 阳性。2 例患者接受新辅助放疗（1 例联合化疗）。术后患者接受放疗（n=13）、再次切除（n=5）联合放疗（n=3）、化疗（n=2）、化疗和放疗（n=1）、未治疗（n=11）和未知（n=6）。31 例患者的随访信息可用（平均：54.2 个月；中位数：38.5 个月；范围：2 至 180 个月）。7 例患者出现局部复发。2 例发生全身转移并死于疾病。切除后平均 40.9 个月（中位数：24 个月；范围：12 至 100 个月）出现复发。无复发患者的平均随访时间为 45.2 个月（中位数：25 个月；范围：2 至 180 个月）（P=0.87）。切缘状态、肿瘤大小和分级与复发无关（P>0.05）。因此，精原细胞瘤去分化脂肪肉瘤包括一系列形态学模式。初次切除很难完全切除。局部复发较为常见，发生全身疾病的患者相对较少。
