Primary synovial sarcoma of kidney: A rare tumor with an atypical presentation

Dassi, Vimal; Das, Krishanu; Singh, Bhupendra; Swain, Sanjaya

doi:10.4103/0970-1591.52932

Cited by 20 publications

(18 citation statements)

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“…Surgical management forms the mainstay of treatment. Although there is no proven benefit of adjuvant chemotherapy, primary synovial sarcoma of kidney may be sensitive to high dose Ifosfamide and Adriamycin based regimens [8].…”

Section: Resultsmentioning

confidence: 99%

Primary Synovial Sarcoma of the Kidney with Inferior Vena Caval Thrombus

Chandrasekaran

Kathiresan

Sundersingh

et al. 2016

Indian J Surg Oncol

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Synovial sarcomas occurs primarily in the soft tissues, mostly in para-articular regions of the extremities. These tumors have been described in other unusual locations like the pleura, lungs, mediastinum, and kidneys. Primary synovial sarcoma of the kidney is a recently described entity; to date, a total of approximately 35 cases have been reported. Only three cases of primary synovial sarcoma of kidney with IVC thrombus have been reported so far. Here we present the case of a 44 year old gentleman, with primary synovial sarcoma of the left kidney with caval thrombus.Keywords Synovial sarcoma . Renal . Tumors . Kidney . IVC . Thrombus Case SummaryA 44 year old gentleman presented with an incidentally detected left renal mass during routine evaluation. On examination, he had a 7*7 cm mass palpable in the left hypochondrium, which was ballotable. CT and MRI scan of abdomen done elsewhere, revealed a 9*9 cm mass lesion involving the upper pole of the left kidney with associated left renal vein thrombosis. Doppler ultrasound at presentation revealed left renal vein thrombosis with no evidence of Inferior vena cava (IVC) thrombosis. His renal parameters were deranged with blood urea 40 mg/ml and serum creatinine 1.7 mg. Renogram revealed glomerular filtration rate of 17.8 ml/min on the left side and 30.7 ml/min on the right side (Figs. 1, 2, 3 and 4).PET CT scan (done without IV contrast in view deranged renal functions) showed a tumor in the upper pole of the left kidney with tumor thrombus in the left renal vein with a SUV of 9.2. He underwent left radical nephrectomy. Intraoperatively, the thrombus was extending into the infrahepatic inferior vena cava and left lumbar vein. The IVC was mobilised, both renal veins were exposed, and slings were passed around these structures for control, taking care not to dislodge the thrombus. Incision performed at the ostium of the tumoral renal vein and the thrombus was completely removed under direct vision.Grossly there was an ill defined white to brown necrotic tumour 10*8*7 cm, involving almost the entire upper pole and with extension into the midpole. Renal vein was dilated with thrombus and the tumor was covered by a thin capsule with perirenal fat and fascia.Microscopy revealed mult-nodular tumor composed of sheets and intersecting bundles of spindle cells with elongated and pleomorphic vesicular nuclei. Microscopic foci of mucinous area was noted. Mitosis was 2 per high power field. Tumor thrombus was adherant to the lumen of the blood vessel. Lymph nodes around the ureter and para aortic nodes were free of tumour. Overall features were suggestive of malignant spindle cell tumor, high grade.Immunohistochemistry study showed strong positive reaction for Vimentin, EMA, CD 99 and bcl-2. 40 % cells showed strong nuclear positivity for KI 67. The tumor cells showed negative uptake for CD10 and Keratin. Histology along with Immunohistochemistry correlation favored Synovial sarcoma, High grade.

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Section: Resultsmentioning

confidence: 99%

Primary Synovial Sarcoma of the Kidney with Inferior Vena Caval Thrombus

Chandrasekaran

Kathiresan

Sundersingh

et al. 2016

Indian J Surg Oncol

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“…SSs usually stain positively for bcl-2, CD99/Mic2, CD56, vimentin, and focally for EMA. However, SSs usually stain negatively for desmin, actin, WT-1, S-100, CD34 and CD31 [1,2,3,4,5,6,7,8,9,10,11,12,13,14]. In the differential diagnosis, the positivity for both CD99 and CD56, and the focal positivity for EMA confirm the diagnosis of SS.…”

Section: Discussionmentioning

confidence: 99%

“…Biphasic, monophasic spindle cell and poorly differentiated variants are histological subtypes of SS. Poorly differentiated SS is composed of sheets of undifferentiated round cells with hyperchromatic nuclei and frequent mitoses, and shows the poorest outcome [8,9,10,11,12,13,14]. Biphasic SS can be diagnosed with the presence of both epithelial and spindle cell components.…”

Section: Discussionmentioning

confidence: 99%

“…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]. …”

Section: Discussionmentioning

confidence: 99%

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Primary Synovial Sarcoma of the Kidney

Özkanli¹,

Yıldırım²,

Zemherı³

et al. 2013

Urol Int

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Synovial Sarcoma (SS) is a soft tissue neoplasm that occurs generally in the proximity of large joints. Here, we report a case of a 45-year-old man who was diagnosed with Primary SS of the kidney which is an extremely rare tumor that accounts for less than 2% of malignant renal tumors. We also review the literature on primary synovial sarcomas of the kidney and focus especially on the renal tumors' differential diagnosis.

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“…There is no clinical or imaging characteristic that can indicate the diagnosis. The diagnosis is difficult due to rarity of tumor and its similar presentations as compared to other renal tumors [4,5]. It mostly affects young people and is associated with bad prognosis and is a rare tumor in location of kidney and so causing diagnostic difficulty and differentiating it from other renal malignant tumors is important which are common like sarcomatoid renal cell carcinoma, sarcomas and hemangiopericytoma Monophasic synovial sarcoma is composed of spindle cells only and there is no epithelial component and biphasic is having spindle cells with admixed epithelial cell component and there may be poorly differentiated type of synovial sarcoma.…”

Section: Introductionmentioning

confidence: 99%

Differentiating Monophasic Synovial Cell Sarcoma of the Kidney from Other Tumors of Kidney and Review of the Literature

Mukherjee¹

2016

Med Clin Rev

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Sarcomas of the kidney could be a clear cell sarcoma or Monophasic Synovial cell sarcoma (MSC) of the kidney is a tumor which is rare in kidney and is associated with poor prognosis. Differentiating this from other tumours is through histology and immunohistochemistry because of its characteristic spindly tumor cells with no epithelial component and immunopositivity for bcl2, CD99 and TEL1. It is important to distinguish because of its poor prognosis and chances of relapse.

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Primary synovial sarcoma of kidney: A rare tumor with an atypical presentation

Cited by 20 publications

References 5 publications

Primary Synovial Sarcoma of the Kidney with Inferior Vena Caval Thrombus

Primary Synovial Sarcoma of the Kidney with Inferior Vena Caval Thrombus

Primary Synovial Sarcoma of the Kidney

Differentiating Monophasic Synovial Cell Sarcoma of the Kidney from Other Tumors of Kidney and Review of the Literature

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