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肾脏原发性滑膜肉瘤:肾肿大的一种罕见鉴别诊断。

Primary synovial sarcoma of kidney: a rare differential diagnosis of renomegaly.

作者信息

Modi Gaurang, Madabhavi Irappa, Panchal Harsha, Anand Asha, Patel Apurva, Parikh Sonia, Revannasiddaiah Swaroop

机构信息

Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat 830016, India.

Department of Radiotherapy, Government Medical College, Haldwani, Nainital, Uttarakhand 263129, India.

出版信息

Case Rep Pathol. 2014;2014:657497. doi: 10.1155/2014/657497. Epub 2014 Dec 24.

Abstract

Synovial sarcomas (SS) are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC) due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.

摘要

滑膜肉瘤(SS)被归类为软组织肉瘤的一个亚组,主要影响年轻成年人的四肢。原发性肾滑膜肉瘤是非常罕见的肿瘤,预后较差。尽管它们具有特征性的组织学和免疫组织化学(IHC)表现,但由于发病率低,很难对其进行诊断。有时需要进行染色体重排研究来确诊。我们报告一例41岁男性,因左肾肿物被转诊至我们的癌症中心进行评估。腹部CT扫描显示左肾有一个大肿物,包绕主动脉。肾肿物活检显示为低分化肉瘤,免疫组织化学检查波形蛋白、CD99和BCL2呈阳性,AE1、上皮膜抗原和白细胞共同抗原呈阴性。由于肾肿物包绕主动脉,该患者临床上无法手术。因此,随后给他采用异环磷酰胺和阿霉素进行姑息化疗。根据实体瘤疗效评价标准(RECIST),腹部CT显示化疗3个周期后有部分缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00c3/4290144/353832c73bfa/CRIPA2014-657497.001.jpg

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