Deme Dániel, Abdulfatah Bishr, Telekes András
Onkológia, Szent Lázár Megyei Kórház Salgótarján, Füleki út 54-56., 3100.
Onkológia, Bajcsy-Zsilinszky Kórház Budapest.
Orv Hetil. 2016 Feb 7;157(6):224-9. doi: 10.1556/650.2016.30366.
In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating factor support and 3 cycles of ifosfamide. After 2 years follow up at the age of 24 years, imaging studies did not reveal any local or distant recurrence.
2013年,匈牙利报告了94770例新发癌症患者。滑膜肉瘤占所有癌症的0.05 - 0.1%,因此预计匈牙利每年的发病率为47 - 94例患者。作者报告了一名18岁男性的病史,该患者接受了右上腹壁肿瘤的手术切除,切除范围为R1。在接下来的5个月里,肿瘤最大直径增长到8厘米。组织学检查显示为单相滑膜肉瘤。免疫组织化学显示bcl2、局灶性CD99和高分子量细胞角蛋白呈阳性,而平滑肌肌动蛋白、S100和CD34免疫染色为阴性。由于无法再次手术,给予了六个周期的阿霉素和异环磷酰胺,并在粒细胞集落刺激因子支持下进行治疗,同时对肿瘤床进行60 Gy的放射治疗。经过这些治疗后,计算机断层扫描结果为阴性,患者每3个月进行一次定期影像学检查。在20岁时,患者手术瘢痕处出现了两个肿瘤性病变,大小分别为10毫米和45×10毫米。进行了R0切除、部分肋骨切除和腹壁重建。组织学检查证实为残留的单相滑膜肉瘤。由于存在肠壁穿孔风险,未进行放射治疗。分期正电子发射断层扫描 - 计算机断层扫描结果为阴性。在22岁时,磁共振成像扫描显示无肿瘤复发,但一个月后,超声检查发现手术瘢痕处有一个快速生长的肿瘤性病变,大小为20×20×12毫米。细胞学检查证实为局部复发,荧光原位杂交显示t(x;18)。进行了R0切除和部分网片切除,组织学检查显示为相同的单相滑膜肉瘤。由于存在血管侵犯且切缘较近(1毫米),患者接受了3个周期的辅助化疗(阿霉素和异环磷酰胺),并在粒细胞集落刺激因子支持下进行,随后又进行了3个周期的异环磷酰胺治疗。在24岁时进行了2年随访,影像学检查未发现任何局部或远处复发。