A Case of Renal Synovial Sarcoma Treated with Adjuvant Ifosfamide and Doxorubicin

Abstract

Primary renal synovial sarcomas (SS) are rare tumors of the kidney. Faria et al first described primary renal synovial sarcoma in 1999 ( Mod Pathol 12:94A). In this paper we present a primary renal synovial sarcoma case and review the 41 primary renal synovial sarcoma cases reported to date. Primary renal synovial sarcomas can exist in either a monophasic or a biphasic pattern. The monophasic variant of primary renal synovial sarcoma is more common and tends to have a better prognosis than the biphasic variant. We present in this paper, a 68-year-old woman with primary renal synovial sarcoma. She presented with right flank pain and abdominal distention. Postoperative pathology of the 20 cm mass on magnetic resonance imaging showed histologic and immunochemical features of synovial sarcoma with coexisting spindle and epithelial cells. She underwent adjuvant ifosfamide and doxorubicin chemotherapy and was free of disease at 1 year after diagnosis. As a conclusion, physicians should be aware of the possibility of malignancy in cystic renal masses and that synovial sarcoma is one of the possibilities.

Primary Synovial Sarcoma of the Kidney

“…Primary sarcoma of the kidney is a rare neoplasm that accounts for less than 2% of malignant renal tumors [1,2,3,4,5]. According to our most recent search in the literature, less than 50 cases have been reported up to now [1,2,3].…”

“…According to our most recent search in the literature, less than 50 cases have been reported up to now [1,2,3]. Leiomyosarcoma is the most frequent type compromising 40-60%, followed by rhabdomyosarcoma, chondrosarcoma, liposarcoma, angiosarcoma, hemangiopericytoma, and osteosarcoma [5,6,7,8].…”

“…On the other hand, the monophasic subtype comprises only an epithelial or spindle cell component. However, monophasic SS may be difficult to differentiate from other spindle cell sarcomas [3,4,5]. The differential diagnosis includes leiomyosarcoma, fibrosarcoma, malignant peripheral nerve sheath tumors, primitive neuroectodermal tumors, fibrous tumors, Wilms' tumors, hemangiopericytoma, congenital mesoblastic nephroma, and sarcomatoid renal cell cancer [1,2,3,4,5,6,7,8,9,10,11,12,13,14].…”

“…SSs have been reported in other unusual sites including the thoracic and abdominal wall, head and neck region, retroperitoneum, bone, lung, or prostate [2,3,4,5]. Primary SS of the kidney is an extremely rare tumor, first described by Faria et al in 1999, as cited in Grampurohit et al [2].…”

“…Histopathological diagnosis is difficult, and always needs immunohistochemical stain and cytogenetic study. In differential diagnosis, we should keep in mind metastatic sarcoma, sarcomatoid renal cell carcinoma, and hemangiopericytoma which may have similar histological features [1,2,3,4,5]. We describe a case of primary SS of the kidney, and review the relevant literature.…”