Renal Leiomyosarcoma Treated With Partial Nephrectomy

Cocuzza, Marcello; Arap, Sami; Lucon, Antônio Marmo; Saldanha, Luiz Balthazar

doi:10.1590/s1807-59322005000400013

Cited by 11 publications

(6 citation statements)

References 9 publications

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“…Wang et al reported NSS in a patient with sarcoma with a disease-free interval of 42 months 5 . Cocuzza et al suggested that renal sarcomas with a diameter of 5 cm may be considered for NSS 9 . Satoh et al reported a case of cystic renal leiomyosarcoma, which was managed by partial nephrectomy with a normal follow up of 44 months 10 .…”

Section: Discussionmentioning

confidence: 99%

Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically

et al. 2019

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Spontaneous retroperitoneal haemorrhage also called Wunderlich Syndrome (WS) may be caused by various aetiologies. One of the most common causes is renal tumour. Renal sarcoma is a rare cause of WS, and renal sarcoma in itself is a rare entity. In the era of nephron-sparing surgery, optimum management of primary renal sarcoma remains a dilemma as there are limited number of cases available in the literature. Nevertheless, radical nephrectomy remains the recommended treatment, keeping in mind the aggressiveness of the tumour. We report a case of primary undifferentiated renal sarcoma, which presented as WS, and which was managed by partial nephrectomy.

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Section: Discussionmentioning

confidence: 99%

Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically

et al. 2019

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“…Local recurrence is reported in 40% of the cases and distant metastases are primarily to the lungs, followed by liver and bones. [15][16][17][18] Metastatic tumors are frequently high grade tumors. No metastases were reported in any of our cases.…”

Section: Discussionmentioning

confidence: 99%

Primary renal leiomyosarcoma: a rare entity

Beigh¹,

Bilal²,

Sheikh³

et al. 2018

Int J Res Med Sci

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Background: Primary leiomyosarcoma of the kidney is an exceptionally rare tumor with an aggressive behavior. However, reported literature is very limited. Since the prognosis for a renal sarcoma is extremely poor, differentiation from sarcomatoid renal cell carcinoma (RCC) is necessary. Histopathology and immunohistochemistry are the only modes of diagnosing these sarcomas as they have no specific diagnostic features clinically and radiologically. Objectives was to evaluate the clinicopathological pattern of leiomyosarcomas arising from kidney.Methods: This study was conducted in the Department of Pathology, Government Medical College, Srinagar. It was a retrospective study done over a period of 3 years, July 2014 to June 2017. A total of 4 patients, who underwent surgery and were diagnosed with primary LMS of the kidney, were included in the present study.Results: The study was done to evaluate the clinicopathological pattern of 4 cases of primary renal LMS diagnosed at a tertiary care hospital. Age of the patients ranged from 35-64 years with a mean age of 53.5 years. Sex ratio of 1:1 was observed. Major presenting symptom was flank pain (75%) followed by mass abdomen (50%). Also, one of the patients presented with spontaneous rupture of kidney. Radical nephrectomy was done in all patients. On gross examination tumor had originated from renal pelvis (50%) in 2 patients, renal vein (25%) in one patient whereas, renal capsule (25%) appeared to be the site of origin in other patient. Histopathological examination and immunohistochemistry proved the lesion as primary leiomyosarcoma of kidney.Conclusions: Being a rare tumour, renal leiomyosarcoma needs to be considered in the differential diagnosis of renal masses even in patients younger than 40 years.

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“…[1][2][3][4][5] Management is not well-established due to rare presentations and limited literature. 6,7 The presentation is often subtle, ranging from abdominal pain, palpable masses, constitutional symptoms or even Budd-Chiari syndrome.…”

Section: Discussionmentioning

confidence: 99%

Leiomyosarcoma of the inferior vena cava with renal metastasis: An unusual case and diagnostic challenge

Chan

Kroczak

Drachenberg

2014

CUAJ

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Leiomyosarcomas are exceedingly rare malignant tumours with a very poor prognosis. We present a 78-year-old patient with a small renal and paracaval mass. Surgery included right open partial nephrectomy and resection of the paracaval mass to treat the presumed renal cell carcinoma. Pathology revealed the tumour to be leiomyosarcoma (LMS). This is only the second documented report of an inferior vena cava LMS presenting along with an ipsilateral renal mass. Case reportA 78-year-old male with a history of intermediate-risk prostate cancer treated with neoadjuvant and adjuvant androgen deprivation therapy, as well as external beam radiotherapy 1 year ago, presented at a follow-up appointment with significant right-sided flank and abdominal pain. Physical examination revealed tenderness in the right flank without detectable peripheral lymphadenopathy. Routine blood work was normal and prostate serum antigen (PSA) was 0.91 μg/L. Computed tomography (CT) and magnetic resonance imaging revealed an enhancing exophytic lesion off the inferior pole of the right kidney measuring 2.1 cm in diameter (Fig. 1a), as well as a 1.8-cm mass anterior to inferior vena cava (IVC) (Fig. 1b) suspicious for an enlarged lymph node. The right renal vein did not appear to be involved. These lesions were not likely metastatic prostate cancer since PSA was 0.91 μg/L. Consultation with urology was initiated for further management of the renal lesion. A positron emission tomography scan was ordered to assess the activity of the caval lymph node. It revealed a hypermetabolic IVC lymph node, as well as several bilateral hypermetabolic hilar lung abnormalities.A bone scan revealed no skeletal metastasis. Repeat CT scan showed growth of the IVC node to 2.2 cm and bilateral pulmonary nodules. The suspicious renal mass remained stable at 1.8 cm.The patient agreed to an open partial nephrectomy and retroperitoneal lymph node dissection as the anterior caval mass was not amendable to biopsy. During the preoperative period, another CT scan showed the IVC node had increased in size to 2.7 cm, while the renal mass remained stable. The renal mass was removed from the right kidney. Intraoperatively, the anterior caval mass involved the IVC and about 30% of the circumference was resected. Pathology revealed a high grade 2.3-cm renal mass and a 3.8-cm caval leiomyosarcoma (LMS) (Fig. 2a). Diagnosis was confirmed with positive smooth muscle cell markers for actin, desmin and vimentin (Fig. 2b). Based on pathology, the final diagnosis was primary IVC LMS with metastasis to the ipsilateral kidney.Postoperatively the patient was seen by the medical oncology team; the patient declined any adjuvant therapy. The patient developed multifocal pulmonary metastases 3 years after his surgery and succumbed to his disease a year later. DiscussionLeiomyosarcomas are extremely rare and often aggressive malignant tumours that are associated with a dismal prognosis.1-5 Management is not well-established due to rare presentations and limited literature. 6,7 The present...

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Renal Leiomyosarcoma Treated With Partial Nephrectomy

Cited by 11 publications

References 9 publications

Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically

Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically

Primary renal leiomyosarcoma: a rare entity

Leiomyosarcoma of the inferior vena cava with renal metastasis: An unusual case and diagnostic challenge

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