Primary renal synovial sarcoma：a case report and literature review
10.3760/cma.j.issn.1000-6702.2014.07.007
- VernacularTitle:原发性肾滑膜肉瘤一例报告并文献复习
- Author:
Deqing ZHANG
;
Yaofeng ZHU
;
Hui MENG
;
Jun WANG
;
Zunlin ZHOU
;
Benkang SHI
- Publication Type:Journal Article
- Keywords:
Kidney neoplasms;
Synovial sarcoma;
Sunitinib;
Diagnosis;
Treatment
- From:
Chinese Journal of Urology
2014;(7):498-501
- CountryChina
- Language:Chinese
-
Abstract:
Objective To improve the diagnosis and therapy of primary renal synovial sarcoma . Methods A single case of primary renal synovial sarcoma was reported .A 54-year-old female presented with left flank pain for six days .Enhanced CT scan revealed a nearly-circular mass with the diameter of 2.0 cm in the superior portion of the left kidney .It showed low and high density with punctate calcification .There were low and high density in left kidney around .A possible diagnosis of left renal tumor with rupturing was given prior to exploratory operation .During the operation , a large hematoma was found on the lateral peri-re-nal fascia, which adjoined with other organs .The hematoma was opened and the 1 000 ml dated clot and blood were removed .The tissue inside the hematoma showed fish meat like .The fast pathologic examination showed sarcomatoid tissue , then the case underwent radical left nephrectomy . Results Gross pathologic examination showed a ashen and fish-liked tumor of 3.0 cm×2.5 cm×1.5 cm with multiple sites of necrosis and hemorrhage .The histopathological examination revealed the tumor cells were spindle shaped .The tumor cells organized as bundle with indistinct border line and larger quantity of mitotic phase , which were positive for BCL-2, vimentin, EMA, CD79a and SMA, but negative for CD34, CK and CD10.A diagnosis of renal syn-ovial sarcoma was then determined .Targeted therapy with sunitinib ( 37.5 mg po qd ) was carried out postopreratively .There was no recurrence and metastasis during the six months following up . Conclusions Primary renal synovial sarcoma is an extremely rare entity .It is very difficult to diagnose through clinical symptoms and imaging .The accurate diagnosis is based on pathology or genetic and molecular tests .Radical nephrectomy and adjuvant therapy are the preferred strategy for the treatment .The prognosis is poor .Targeted therapy with sunitinib may be effective .
