Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma

Schaal, Carlos H; Navarro, Fábio C. P.; Neto, Francisco A. Moraes

doi:10.1590/s1677-55382004000300006

Cited by 30 publications

(30 citation statements)

References 5 publications

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“…Primary synovial sarcomas of kidney have shown to have aggressive clinical course and poor outcomes [6,7]. There are no guidelines for the treatment of primary synovial sarcoma of kidney due to the rarity of cases.…”

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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“…There are no tell-tale specific clinical or radiological features of this tumor in kidney so diagnosis is extremely difficult and is very important to differentiate due to bad prognosis. Histologically monophasic synovial sarcomas may be difficult to differentiate this from sarcomatoid tumors originating in the kidney, especially sarcomatoid renal cell carcinoma, retroperitoneal sarcomas, malignant peripheral nerve sheath tumor, primitive neuroectodermal tumors involving the kidney [6][7][8][9][10]. Immunohistochemistry with TEL1, bcl2, CD99 and vimentin is helpful.…”

Section: Discussionmentioning

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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“…In a case reported by Schaal et al response to ifosfamide and adriamycin is demonstrated (24). However, in surgical managing cystic renal mass, extreme care must be taken to avoid cyst puncture during tumor mobilization or excision and adequate margin of normal parenchyma must be maintained (25).…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis of primary renal synovial sarcoma is unclear due to the limited number of reported cases (2,3). However from previously published data, renal synovial sarcomas are believed to have aggressive clinical courses and poor outcomes (4,5).…”

Section: Introductionmentioning

confidence: 99%

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

Özkan

Mertsoylu²,

Özardalı

2011

Intern. Med.

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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.

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Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma

Cited by 30 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

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

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

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