An Extremely Rare Anomaly: Unveiling Renal Vein-Originated Leiomyosarcoma

Leiomyosarcoma of renal vein and inferior vena cava

Nutcracker syndrome due to left-sided inferior vena cava compression and treated with superior mesenteric artery transposition

Renal Cell Carcinoma with Thrombus Extension into the Inferior Vena Cava and the Right Atrium: A Case Report

LEFT INFERIOR VENA CAVA IN A LIVING KIDNEY DONOR

Endovascular treatment of obliterative hepatocavopathy with inferior vena cava occlusion and renal vein thrombosis

Objective To discuss the surgical therapy of primary leiomyosarcoma of the inferior vena cava(PIVCLS).Methods Retrospective analysis of was made 5 patients of PIVCLS from Oct 2009 to May 2011 hospitalized in Department of Vascular Surgery,Provincial Hospital Affiliated to Shandong University.All patients underwent surgical resection,combined with reconstruction of bilateral renal vein and distal inferior vena cava using artificial vascular graft.Results Surgical resection was performed successfully in all patients.The mean operation time was 166.6 min,with mean blood loss 1 560 mL.Leiomyosarcoma intruding the inferior vena cava and right renal vein were observed in all patients during operation.The mean size was 12 cm × 10 cm× 8 cm.The diagnosis of PIVCLS in 5 patients was confirmed by postoperative pathologic examination.All patients did not present lower extremity swelling after surgery and discharged from hospital with normal blood (BUN) and (CREA).All patients were administrated with oral warfarin therapy after discharge.No clinical relapse and pulmonary embolism was observed during the follow-up (range 3 months to 12 months).The ultrasound revealed the patency of artificial vascular grafts in all patients.Conclusions Surgical resection combined with reconstruction of bilateral renal vein and distal inferior vena cava using artificial vascular graft is an effective and feasible treatment of PIVCLS.Leiomyosarcoma is completely eliminated and important abdominal viscera are protected well during the procedure because of minimal impact on hemodynamics.The incidence of postoperative pulmonary embolism also decreases obviously. Key words: Inferior vena cava; Leiomyosarcoma; Artificial vascular graft

Primary Leiomyosarcoma of the Inferior Vena Cava: Reports of Infrarenal and Suprahepatic Caval Involvement

Nutcracker Syndrome—How Well Do We Know It?

Intraoperative immediate diagnosis of acute obstruction of tricuspid valve and pulmonary embolism due to renal cell carcinoma with transesophageal echocardiography.

The purpose of this article is to present the CT features in five cases of pathologically verified Inferior vena cava (IVC) leiomyosarcoma. In this retrospective analysis, we reviewed CT features in 5 cases of clinicopathologically confirmed IVC leiomyosarcoma with respect to its location (infra renal, trans renal, supra renal), its extent (with or without involvement of renal vein, hepatic IVC with or without involvement of hepatic vein, right atrial & extra caval extension) and pattern of enhancement. CT guided biopsy was performed in four patients while the last patient underwent successful resection of the tumor. Three male and two female patients (aged 45 to 72 years) were included in the study. Heterogeneously enhancing retroperitoneal mass involving IVC is the most common imaging feature. The intra and extra luminal extension was demonstrated excellently in all patients. IVC leiomyosarcoma is a rare neoplasm often presenting very late with non-specific symptoms. Cross sectional imaging establishes the exact location and extension and plays a vital role in determining the resectibility and planning the management.

Léiomyosarcome de la veine rénale gauche dans un contexte de polyarthrite rhumatoïde sous méthotrexate

Leiomyosarcoma is the second most common primary retroperitoneal malignant tumor in adults, which commonly originates in the inferior vena cava (1). A leiomyosarcoma arising from the renal vein is particularly uncommon. After the first report of Varela and Garro in 1967, only about 30 cases have been reported in the published medical literature (2, 3). The renal vein leiomyosarcoma is grossly classified into three subtypes: completely extravascular; completely intravascular and mixed (1, 4). To the best of our knowledge, there have been only few reports detailing the imaging findings of renal leiomyosarcoma with both extraand intravascular components (1, 4). We report an uncommon case of left renal vein leiomyosarcoma with combined components, focusing on the multidetector computed tomography (MDCT) and MR imaging findings with review of the literature. This case report was approved by the ethics committee at our institution and an informed consent was waived.

Renal vein leiomyosarcoma is a rare tumor whose clinical and imaging features can significantly overlap with those of advanced primary renal neoplasms, particularly renal cell carcinoma. IRB approval was obtained for a retrospective review of clinical and imaging information for five patients with the pathologically proven diagnosis of renal vein leiomyosarcoma at two institutions. Among the five patients, two were female and three male; average age 58.6 (range 42-77). Three patients presented with flank or abdominal pain, one with pulmonary embolism, and one with weight loss and fatigue. Mass size ranged from 4 to 10cm. Four masses were left-sided and one right-sided. Tumors involved and expanded the renal vein in all cases, with tumor extension to the renal hilum in one case, through the renal hilum into renal parenchyma in two cases, and into inferior vena cava in two cases. All masses demonstrated contrast enhancement, which was solely peripheral in one case. In all cases, extrarenal tumor volume was greater than renal parenchymal tumor volume. When renal masses are predominantly or exclusively intravascular, consideration should be given to the diagnosis of renal vein leiomyosarcoma because preoperative biopsy results could significantly alter management.

Objective To discuss the lymph node metastasis of chromophobe renal cell carcinoma (chRCC)in children. Methods One case of chRCC was reported. A eleven-year-old boy was admitted to our hospital on November 2, 2017 and presented gross hematuria 4 weeks. No mass was palpable in the abdomen and he felt tenderness in the left back. The ultrasound showed a solid mass in the lower pole of the left kidney. There were a lot of tortuous blood vessels in the tumor. Contrast enhanced CT suggested a mass of 4.5 cm×6.3 cm×4.9 cm. The left renal artery and vein were thickened. There was no enlarged lymph nodes were seen in both examinassions. During the operation, the tumor was located in the lower pole of the kidney. The frozen section diagnosis was benign renal tumor. Nephron-sparing surgery was performed. Results The pathology showed that the tumor cells had well-defined cell borders and characteristic peri-nuclear halos surrounded by eosinophilic cytoplasm that was positive for Hale's colloidal iron stain. Immunohistochemical studies were positive for EMA(+ ), Ki-67(5%+ ), CK19(+ ), CK(+ ), CD10(+ ), CD117(+ ), CD56(+ ). Fluorescence in situ hybridization showed absence of TFE3 protein, a strong immune marker of Xp11.2/TFE renal cell carcinoma. 1 year after the operation, contrast-enhancement magnetic resonance showed long T1 signal near the left renal hilum and the mass showed slight enhancement. ChRCC with regional lymph node metastasis hereby was considered. Intraoperatively, an approximately 2.5 cm×2.0 cm×2.0 cm mass was seen near the the left renal hilum. The tumor did not involve left kidney, abdominal aorta and inferior vena cava. The mass was completely removed and intro-operative fast-frozen pathology revealed chRCC. Nephron-sparing surgery was performed. Postoperative pathology report chRCC local lymph node metastasis . Follow-up at six months after resection, there was no recurrence or metastatic disease identified. Conclusions chRCC is rare in children and clinical stage is low with a good prognosis. Local recurrence and distant metastasis is rare. Key words: Chromophobe renal cell carcinoma; Children; Metastasis; Prognosis

A 63-year-old male, with history of hypertension, diabetes mellitus and hypercholesterolemia, underwent an ultrasound scan for prostate hypertrophy, which revealed an abdominal aortic aneurysm (AAA). An abdominal CT scan confirmed the presence of a 5.5 cm diameter AAA and disclosed duplication of the inferior vena cava (IVC) (Panel A; arrows). The right-sided IVC followed the normal IVC anatomy, whereas the left-sided IVC originated in the pelvis from the left common iliac vein, ascended along the posterior-lateral aspect of the abdominal aorta, passed anterior to the AAA neck where it joined the left renal vein and then entered the right-sided IVC. At surgery, the left-sided IVC (Panel B: black arrow left-sided IVC; white arrow left renal vein; bent arrow left spermatic vein) was dissected free of the AAA neck and the AAA was replaced with a 20 mm Dacron tube graft. The postoperative course was uneventful and the patient was discharged on the seventh postoperative day. Duplication of the IVC is estimated to occur in 2 3% of autopsy series. The abnormality is caused by failure of regression of the caudal left supracardinal vein during the first embryologic trimester, which results in persistence of both left and right supracardinal veins. Several variations of double IVC have been described. The left-sided IVC may pass anterior or posterior to the aorta or may ascend along the lateral aspect of the aorta and empty into the left renal vein. The left-sided IVC can also be poorly developed or segmental. The presence of double IVC poses hazards to the surgeon during abdominal aortic surgery. Preoperative diagnosis is desirable and can be made on a CT scan. Ultrasound scan and venography of the vena cava is rarely recommended. It should be noted, however, that CT scans are routinely performed for patients scheduled for AAA repair but not for patients undergoing reconstruction for aortoiliac occlusive disease. Familiarity with the anatomy of the most common types of major venous anomalies is therefore mandatory for all vascular surgeons to reduce the risk of severe venous hemorrhage associated with these anomalies.