A right renal angiomyolipoma with IVC thrombus and pulmonary embolism

Angiomyolipomas (AML) are benign lesions, commonly found in the kidney, where they may be single or multiple. There is a high association with tuberous sclerosis or lymphangioleiomyomatosis. When found as a solitary lesion, they are usually found in females in the forties to fifties age group. One of the rare complications is that of involvement of the lymph nodes and vascular spread via the inferior vena cava. We review the available literature and present a case of invasive AML with fat embolism.     

Rapidly growing renal angiomyolipoma during pregnancy with tumor thrombus into the inferior vena cava: a case report

A case of renal angiomyolipoma rapidly growing during pregnancy with tumor thrombus into the inferior vena cava is reported. Angiomyolipoma in a 31-year-old woman was incidentally found by ultrasonography. CT scan revealed a fat-containing tumor in the right kidney with 4 cm in diameter. The patient was followed at outpatient clinic without any treatment. Fifteen months later, the post-delivery follow-up CT scan showed that tumor size had grown up to 11 cm in diameter. Although laparoscopic right nephrectomy was tried, open transperitoneal right nephrectomy was performed because the tumor thrombus extending into the inferior vena cava was found during the laparoscopic procedure. Pathological diagnosis was angiomyolipoma of the kidney. There are several reports that indicate angiomyolipoma grows rapidly during pregnancy. Our case demonstrates that careful follow-up is required for angiomyolipoma in women with possible conception and delivery.     

Computerized tomographic demonstration of inferior vena caval tumor thrombus from renal angiomyolipoma

A case of renal angiomyolipoma with gross venous tumor thrombus extension into the inferior vena cava is presented. This fatty tumor thrombus is visualized easily by computerized tomography and is confirmed by inferior venacavography, surgery and histology. Macroscopic renal vein and vena caval tumor thrombi rarely occur in patients with angiomyolipoma and may be diagnosed preoperatively by the demonstration of abundant fat within the tumor components.     

A case of renal angiomyolipoma with bone formation

A case of renal angiomyolipoma with bone formation is reported. A 61-year-old woman was admitted to our hospital with one month history of fever. About 15 cm in diameter mass was palpated in her right lower abdomen. Plain abdominal roentgenography, ultrasonography and computed tomography showed marked calcification in the cephalic portion of the right kidney, tumors surrounding right kidney and another tumor in the right lower abdomen. All these tumors and the kidney constituted a big mass continuously, and no fatty component was demonstrated. Total excision of the mass including the kidney was performed. The mass was 30 X 24 X 10 cm in size and 3,240 g in weight. Histologically, the tumor was compatible to renal angiomyolipoma. But, radiopaque shadows which looked like calcification were disclosed to be bones, and fatty tissues were not seen so much. Therefore, the name "benign mesenchymoma" was thought to be more acceptable than angiomyolipoma in this case. Preoperative diagnosis of renal angiomyolipoma is the demonstration of fatty densities in the tumor, and the characteristic extrarenal development should also be taken into consideration. Furthermore, the bone formation of angiomyolipoma, which is very rare might be an important finding to angiomyolipoma.     

A case report of retroaortic left renal vein with tumor thrombus of renal cell carcinoma

A 75-year-old woman was referred to our department for evaluation of a left renal tumor. Computed tomography and other imaging studies demonstrated a left renal mass and tumor extension into the left renal vein passing caudally behind the aorta. We clinically diagnosed the tumor as renal cell carcinoma (RCC) associated with a retroaortic left renal vein thrombus, and performed a radical nephrectomy. Pathological examination of the surgical specimen showed a grade 2, clear cell carcinoma with a renal vein thrombus and negative surgical margin. Retroaortic left renal vein is a rare anomaly with a prevalence of 1.8–2.4%. RCC associated with a retroaortic left renal vein thrombus is rarer still. To our knowledge, this is only the third case report to describe an RCC associated with a tumor thrombus in the retroaortic left renal vein.     

Renal angiomyolipoma with a tumor thrombus extending into the right atrium--a case report]

We present a case of spontaneous rupture of renal angiomyolipoma with a tumor thrombus extending from the right renal vein and inferior vena cava to the right atrium. A 41-year-old woman, previously in good health, was referred to our hospital with right flank pain. Computed tomography showed fat densities in both tumor and thrombus. Other imaging examinations also demonstrated a large right renal mass (18 cm in diameter), a long tumor thrombus (13 cm in length) and a small left renal tumor (1.5 cm in diameter). Right nephrectomy and en-bloc removal of the intra caval and intracardiac tumor thrombus were performed on cardiopulmonary bypass. It was pathologically diagnosed as an angiomyolipoma without tuberous sclerosis. At present, three years after surgery the patient is doing well, showing neither metastasis nor increase of the left renal angiomyolipoma. To our knowledge, our case seems to be the 3rd case report of renal angiomyolipoma with a tumor thrombus extending to the right atrium. We conclude that renal angiomyolipoma even with an intra cardiac tumor thrombus can be resected safely and successfully.     

Non-ischemic tumor enucleation for renal angiomyolipoma: a case report

A case of renal angiomyolipoma, successfully treated with non-ischemic tumor enucleation, is reported. A 16-year-old Japanese female visited another hospital with a chief complaint of general fatigue. She was diagnosed with angiomyolipoma of the right kidney, 7 cm in the long axis, which developed exteriorly at the lower pole. A nephrectomy was recommended. The patient visited us for a second opinion. We judged that nephron-sparing surgery was applicable to this case. The patient underwent non-ischemic tumor enucleation using a microwave tissue coagulator via retroperitoneal approach. The patient was discharged from our hospital 9 days after the surgery. Since a preoperative diagnosis with renal angiomyolipoma can be obtained relatively easily, maximum efforts for nephron-sparing surgery should be made.     

Exophytic renal angiomyolipoma. A case report

A case of a large exophytic renal angiomyolipoma is presented. Despite the features on excretory urography suggestive of an extrarenal mass, computed tomography provided information concerning the origin and pathological nature of the lesion.     

A case of angiomyolipoma of the renal sinus

The case of a 63-year-old woman with a relatively rapidly growing angiomyolipoma (AML) originating from the right renal sinus is presented. The tumor had enlarged to more than fivefold in volume (7.4 × 6.7 × 10.1 cm) in the 7 years following its detection (5.6 × 3.4 × 4.6 cm) and had significantly pushed aside the duodenum and the inferior vena cava. The tumor was surgically resected together with the right kidney, and pathological examination revealed AML originating from the renal sinus. An AML involving or originating from the renal sinus is exceedingly rare; only 14 cases have been reported. Rapidly growing AMLs of the renal sinus are difficult to differentiate from liposarcoma because of similar findings in imaging studies. It should be noted that AML of the renal sinus can be a differential diagnosis of retroperitoneal fatty mass. Furthermore, an enlarged AML in the renal sinus may push aside other organs, such as the ileum, pancreas, and major vessels. Clinicians should determine whether these patients should be placed under active surveillance or be treated with invasive measures, such as radical nephrectomy.     

A case of renal angiomyolipoma with marked cystic degeneration and pseudoaneurysm

A 59-year-old woman consulted our hospital for a left renal mass which she had been aware of for 4 years. The tumor was in the lower portion of the left kidney. It was a cystic tumor whose wall was enhanced on computed tomography and magnetic resonance imaging. The lesion 3.0 cm in diameter, which was enhanced equally to the aorta, was found in a part of the wall. It was thought to be a pseudoaneurysm by renal angiography. We suspected a cystic renal cell carcinoma because of the plural feeding arteries and tumor staining, and performed left total nephrectomy. Pathological diagnosis was angiomyolipoma with few fat components. To our knowledge only 3 previous cases of renal angiomyolipoma with marked cystic degeneration have been reported in Japan. In all 3 cases, it was difficult in preoperative diagnosis to distinguish angiomyolipoma with cystic degeneration from cystic renal cell carcinoma complicated cyst. Moreover, this is the first reported case of renal angiomyolipoma with marked cystic degeneration and pseudoaneurysm.     

A case report of left renal cell carcinoma with tumor thrombus extending into the right atrium

A case report of left renal cell carcinoma with tumor thrombus extending into the right atrium is reported. A 76-year-old woman was found to have a left renal tumor with tumor thrombus extending into the inferior vena cava and right atrium by computed tomographic-scanning. Left nephrectomy and removal of an intra-atrial tumor thrombus were performed under a cardiopulmonary bypass. The post-operative course was uneventful and the patient was discharged from the hospital 22 days postoperatively. The pathological diagnosis was clear cell carcinoma. After surgery, the patient received interferon-gamma. However, the patient developed lung metastases 26 months after the operation and is currently being observed while receiving interferon-alpha.     

Malignant renal epithelioid angiomyolipoma with an inferior vena cava and right atrium thrombus

The epithelioid variant type of angiomyolipomas presents malignant characteristics compared to classic angiomyolipomas. Epithelioid angiomyolipomas (eAMLomas) are easy to misdiagnose as renal cell carcinoma due to similar histologic and radiographic findings. Until recently, only a few cases of distant metastases of eAMLoma were reported. Herein, we describe a 69-year-old woman with renal eAMLoma and tumor thrombus metastasis to the renal vein, inferior vena cava, and right atrium. She received a left radical nephrectomy combined with tumor thrombus excision and systemic chemotherapy. Using this case, we remind readers of the aggressive behavior and poor prognosis of eAMLomas and review the literature on the optimal treatment strategy.     

Pancreatic neuroendocrine tumor with splenic vein tumor thrombus: A case report

INTRODUCTION

Pancreatic neuroendocrine tumors (PNET) are rare, often indolent malignancies. PNET are classified as functional or nonfunctional based on the secretion of hormones without a negative feedback loop; the latter account for up to 60% of PNET. Although PNET are associated with a better prognosis compared to pancreatic adenocarcinomas, they are often diagnosed in advanced stages, making them a significant source of morbidity for patients. Here we present a rare case of venous tumor thrombus arising from a nonfunctional PNET.

PRESENTATION OF CASE

A 44-year-old woman was referred for evaluation and treatment of a possible tail of pancreas PNET discovered during work-up for a 9 year history of intermittent subcostal pain. Previous endoscopic ultrasound with fine needle aspiration revealed a 3.5 cm × 3 cm mass, with cytological diagnosis of neuroendocrine tumor. Patient was scheduled for laparoscopic distal pancreatectomy. During surgery the mass was found to encase the splenic vein leading the surgeon to perform an en bloc distal pancreatectomy and splenectomy. Pathologic analysis revealed a 1.8 cm × 5 cm tumor thrombus lodged in the splenic vein.

DISCUSSION

Nonfunctional PNET usually present in advanced stages and can be associated with venous tumor thrombi. Preoperative imaging may not accurately predict the presence of venous tumor thrombi.

CONCLUSION

En bloc resection of primary tumor, involved organs and thrombus is the recommended treatment option and often results in long term survival. New multi-modality strategies are needed for detection of venous involvement in nonfunctional PNET to better assist with preoperative planning and counseling.     

Mass-forming intrahepatic cholangiocarcinoma with portal vein tumor thrombus and bile duct tumor thrombus: A case report

IntroductionWe report the first case of mass-forming intrahepatic cholangiocarcinoma (ICC) with portal vein tumor thrombus (PVTT) and bile duct tumor thrombus (BDTT), where the extrahepatic bile duct was preserved with thrombectomy.Presentation of caseA 70-year-old male. Magnetic resonance imaging (MRI) showed the tumor extending from the hepatic hilum to the left hepatic duct with complete obstruction of the left hepatic duct and a defect at the left portal vein. We planned to perform extended left lobectomy, lymph node dissection, extra hepatic bile duct resection and reconstruction based on the diagnosis of mass-forming ICC with left portal vein and left hepatic duct infiltration (cT3N0M0 Stage III). Intraoperative cholangiography revealed a crab claw-like filling defect at the left hepatic duct, which suggested tumor thrombus. Accordingly, we performed thrombectomy. The margin of the left hepatic duct was tumor negative, so we performed extended left lobectomy, lymph node dissection and thrombectomy. Pathologically, the tumor was diagnosed as ICC (pT4N0M0 Stage IVA, vp3, b3). Tumors in the left hepatic duct and left portal vein proved to be tumor thrombus. The postoperative course was uneventful. He is doing well without recurrence.DiscussionThrombectomy is performed for hepatocellular carcinoma (HCC) with tumor thrombus. Furthermore, extrahepatic bile duct resection and reconstruction are recommended for ICC. In this case, intraoperative cholangiography was effective for precisely diagnosing. Thrombectomy could reduce surgical stress and prevent complications.ConclusionsThrombectomy can be a valid option for ICC with tumor thrombus, as well as for HCC.     

A case of renal tumor with erythrocytosis

A case of renal tumor with secondary erythrocytosis and acute occlusion of the left common iliac artery is reported. The patient was a 73-year-old male who complained of right upper abdominal pain. Laboratory investigation at admission revealed erythrocytosis. Radiological examinations including excretory urography, angiography and computed tomographic (CT) scan showed that the hypervascular tumor was related to the right kidney with pulmonary metastases. Transabdominal echography revealed a tumor thrombus in the inferior vena cava. The tumor was not surgically removed, and IFN-alpha (HLBI) at a dose of 3 x 10(6) units was administered intramuscularly for 6 consecutive days every week and UFT at a dose of 3 capsules was given by oral route daily. About two months later, acute occlusion of the left common iliac artery with necrosis of the left toes occurred, and the left lower extremity was amputated. The high haematocrit resulted in an increase of blood viscosity which caused acute arterial occlusive disease. The association between renal tumor and erythrocytosis is discussed.     

Giant renal angiomyolipoma in a case with pulmonary lymphangiomyomatosis

A 27-year-old female with pulmonary lymphangioleiomyomatosis was referred to us because of the right large renal angiomyolipoma (AML), 10 cm in diameter, which was found by abdominal CT during close investigation of her pulmonary disease. About 6 months earlier she had received operation for pneumothorax and the histological examination disclosed pulmonary lymphangioleiomyomatosis (LAM). Clinically she does not have tuberous sclerosis. Partial nephrectomy was performed. The renal tumor arose from the lower part of the kidney with somewhat exophytic growth. She is now followed at outpatient clinic without recurrence of renal AML. Pulmonary LAM often associates with renal AML. Although pulmonary LAM is a progressive disease and has a poor prognosis, because of the risk of the rupture of renal AML, large renal AMLs should be treated. Recently several cases have been reported for whom nephron-sparing surgery was performed. This case is 10th report of LAM, which is associated with AML in Japan.     

A case of giant renal angiomyolipoma treated by partial nephrectomy

A 38-year-old woman visited our hospital complaining of decreased appetite and sensation of pressure on her abdomen. Computed tomographic scan revealed right giant renal angiomyolipoma. Partial nephrectomy was performed. The resected specimen weighed 970 grams. The histological diagnosis was consistent with angiomyolipoma. Partial nephrectomy was performed because the connection between the tumor and the kidney was in a small range and the tumor was detached easily from the surrounding tissue.