Leiomyosarcoma of Renal Vein - A Rare Case Report

Ojha, Sandeep; Nilkanthe, Ramarao; Valecha, Jyoti; Meenai, Farah Jalaly; Haritwal, Amit

doi:10.7860/jcdr/2017/24684.9632

Cited by 4 publications

(5 citation statements)

References 11 publications

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“…A few cases of LMS of the renal vein have been reported so far [ 5 , 7 , 10 ]. It is more frequent in women (3F/1M), the mean age of presentation is 57 years (range 27–88 years), and it is predominantly located in the left side (60%) [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Female preponderance is supported by the theory that estrogenic stimulation leads to growth and proliferation of smooth muscle tumors [ 5 ]. The more frequent involvement of the left renal vein is suggested by its longer length compared to the right renal vein [ 5 , 7 ]. In our case, the patient was a male, and the tumor involved rather the right renal vein.…”

Section: Discussionmentioning

confidence: 99%

“…Local recurrence is reported in 40% of the cases, and distant metastases are primarily to the lungs, followed by the liver and bones [ 10 , 11 ]. However, it has been reported that tumors originating from IVC show a more aggressive course than those arising from the renal vein [ 5 , 13 ]. Studies performed at Memorial Sloan Kettering, New York, showed that the major prognostic factor is total surgical resection.…”

Section: Discussionmentioning

confidence: 99%

“…Primary LMS of the renal vein are rare with only 67 cases reported worldwide [ 3 ]. They occur predominantly in females with a peak in the fifth and sixth decades [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation

Fekkar¹,

Elouazzani²,

Jahid³

et al. 2021

Case Reports in Pathology

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Primary leiomyosarcomas (LMS) of vascular origin are rare tumors, and more than half of the cases arise in the inferior vena cava (IVC). Primary LMS of the renal vein are extremely rare tumors with only a few cases reported in the literature. Their diagnosis is made only by pathological features. Histologically, they are made of atypical spindle-shaped cells arranged in long intersecting fascicles. Tumor cells stain positive for myogenic markers in immunohistochemistry. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. Because of insufficient histological data and follow-up, the prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor. We report here an exceptional case of a huge LMS of the right renal vein mimicking a primitive renal cell carcinoma, occurring in a 56-year-old male patient.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Primary LMS of the renal vein are rare with only 67 cases reported worldwide [ 3 ]. They occur predominantly in females with a peak in the fifth and sixth decades [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation

Fekkar¹,

Elouazzani²,

Jahid³

et al. 2021

Case Reports in Pathology

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“…[6] Still rarer is the tumor arising from renal vein and only about 35 cases have been reported in the literature. [7] This tumor was first described by López Varela and Pereira Garro in 1967. [8] Over a span of the last two decades, literature on renal leiomyosarcoma is sparse with few isolated case reports, [1][2][3][4] and a multi-institutional data is relatively lacking.…”

Section: Discussionmentioning

confidence: 99%

Primary Renal Leiomyosarcoma Arising from Renal Vein: A Case Report of Rare Entity with Review of Literature

Kundu

Chandra

Punia

et al. 2019

Indian Journal of Medical and Paediatric Oncology

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Primary renal sarcomas are extremely rare neoplasms accounting for about 1% of all malignant renal neoplasms. Among all the sarcomas of kidney, leiomyosarcoma is the most common histotype. The histogenesis of these tumors is unclear, and presumably, they arise either from renal capsule or smooth muscle of renal vessels or renal pelvis. We report a case of a 65-year-old woman, who presented with intermittent abdominal pain for 1 year. The correct diagnosis was established only after the histopathological analysis of the resected specimen. The poor prognosis borne by these tumors mandates a thorough evaluation of all renal masses with unusual gross and histomorphology.

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Clinical experience with the treatment of retroperitoneal vascular leiomyosarcoma originating from large veins

et al. 2021

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Background Retroperitoneal vascular leiomyosarcoma (RVLMS) is an extremely rare disease in clinical practice, and it has poor prognosis. This article is to explore the diagnosis and treatment of RVLMS and present our experience. Methods Data of RVLMS patients were continuously collected in our hospital from August 2018 to February 2020: two males and two females with a median age of 56 (min–max = 33–61) years were included. Patients in whom paraganglioma could not be excluded were asked to take phenoxybenzamine before surgery. A multi-disciplinary team (MDT) meeting had been held and surgery was recommended. The operation procedures varied based on the tumor location, shape, and stage, and the core steps were "exposure of the retroperitoneum and tumor, identification of vital blood vessels, blocking the bloodstream, complete removal of the tumor and tumor thrombus, and release of blood flow". A Satinsky clamp was used to partially block the blood vessels. Follow-up was conveyed by revisits and phone calls. Results One patient underwent open surgery, and three patients underwent laparoscopic surgery, one of whom underwent conversion to open surgery. The procedures were finished successfully, with a median operative time of 314.5 (min–max = 224–467) mins. The median amount of intraoperative bleeding was 550 (min–max = 200–1500) ml, and three patients had transfusion during the operation. The mass was irregular in shape, with a median maximum size of 7.45 (min–max = 4.2–10.7) cm, and the pathological examination confirmed RVLMS, which has spindle-shape, high mitotic activity and atypia. One week after the operation, the median serum creatinine level was 85 (min–max = 70–99) µmol/L. The median follow-up time was 16 (min–max = 13–21) months, and 1 case reported asymptomatic recurrence. Conclusion Uncharacteristic manifestations and imaging features contribute to the problematic diagnosis of RVLMS. Comprehensive preoperative evaluation and careful surgical planning are essential. Multicenter research is needed in the future to reach a dominant consensus.

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Leiomyosarcoma of Renal Vein - A Rare Case Report

Cited by 4 publications

References 11 publications

Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation

Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation

Primary Renal Leiomyosarcoma Arising from Renal Vein: A Case Report of Rare Entity with Review of Literature

Clinical experience with the treatment of retroperitoneal vascular leiomyosarcoma originating from large veins

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