Pitino A, Squillaci S, Spairani C, Cosimi M F, Feyles E, Ricci D, Bardari F, Graziano M, Morabito F, Cesarani F, Garruso M, Belletti M, Beierl K, Murphy K M
Division of Anatomic Pathology, San Giacomo Hospital, Novi Ligure, AL, Italy.
Pathologica. 2011 Oct;103(5):271-8.
Synovial sarcoma (SS) is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features that usually arises in the extremities of young adults. The occurrence of these tumors in the kidney is extremely rare and have been prevalently described in case reports. The objectives of this work were to evaluate the frequency of primary renal synovial sarcomas and the pathologic progression in recognition of this possibly under-diagnosed entity. A comprehensive review of the literature has also been performed with a focus on survival. We report the clinico-pathological features of an intrarenal SS occurring in a 67-year-old man. The tumour, measuring 4 cm in its greatest diameter, completely replaced the cortex and the medulla of the inferior region of the left kidney compressing the iliopsoas muscle. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumour was composed of atypical monotonous vimentin+, CD99+, bcl-2+ spindle cells exhibiting a haphazard fascicular growth pattern and a high mitotic rate (3 to 5 mitoses per HPF). The diagnosis was supported by reverse transcription-polymerase chain reaction which demonstrated SYT-SSX2 gene fusion. The patient was alive with local recurrence of disease 24 months after surgery. Synovial sarcomas occurring in the kidney, in analogy to other sites, tend to have an aggressive biologic behaviour. Despite being extremely uncommon, with only 44 cases reported to date, they should be included in the differential diagnosis of benign and malignant spindle cell tumours of the kidney. This study also emphasizes the importance of a correct pathologic diagnosis for prognostic and therapeutic implications.
滑膜肉瘤（SS）是一种软组织肿瘤，具有明确的组织学、免疫组化和分子特征，通常发生于年轻成年人的四肢。这些肿瘤在肾脏中极为罕见，大多在病例报告中有所描述。本研究的目的是评估原发性肾滑膜肉瘤的发生率以及认识这种可能诊断不足的实体的病理进展情况。我们还对文献进行了全面综述，重点关注生存率。我们报告了一名67岁男性发生的肾内滑膜肉瘤的临床病理特征。肿瘤最大直径为4 cm，完全取代了左肾下部的皮质和髓质，压迫髂腰肌。影像学检查结果与肾细胞癌一致。组织学上，肿瘤由非典型的、单一的波形蛋白阳性、CD99阳性、bcl-2阳性的梭形细胞组成，呈杂乱的束状生长模式，有较高的有丝分裂率（每高倍视野3至5个有丝分裂象）。逆转录聚合酶链反应证实存在SYT-SSX2基因融合，支持该诊断。患者术后24个月仍存活，但有局部疾病复发。与其他部位发生的滑膜肉瘤一样，发生于肾脏的滑膜肉瘤往往具有侵袭性生物学行为。尽管极为罕见，迄今为止仅报道了44例，但在肾脏良恶性梭形细胞瘤的鉴别诊断中应考虑到它们。本研究还强调了正确病理诊断对预后和治疗的重要性。
