Bilateral Renal Angiomyolipomas Research Articles

Two Cases of bilateral diffuse cystic lesion

Lymphangioleiomyomatosis(LAM) is a rare disease of unknown etiology that occurs mainly in woman in her reproductive age. We recently experienced two cases of bilateral diffuse cystic lesion of the lung on chest X-ray and HRCT. The first case, a 26-year-old female, who had been diagnosed with tuberous sclerosis be the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to recently developed hemoptysis. Chest PA showed diffuse ground-glass opacity with radiolucent cystic lesions of various sizes on both lung fields. HRCT showed innumerable small cystic lesions with suspicious diffuse ground-glass opacity on both lung fields. The second case, a 30-year-old female was admitted due to dyspnea and spewing of blood-tinged sputum for 2 weeks, shortly after delivery. Chest PA showed diffuse reticular and ground-glass opacities on both lung field. HRCT showed multiple well-difined and relatively uniform size air cysts with a uniform wall thickness on entire both lung fields, with small amount of right pleural effusion. By thoracoscopic lung biopsy she was diagnosed with pulmonary lymphangioleiomyomatosis. We report these cases with a brief review of the literatures.

RENAL ANGIOMYOLIPOMA ASSOCIATED WITH INFERIOR VENA CAVAL AND RIGHT ATRIAL THROMBUS

A 40-year-old woman with no stigmata of tuberous sclerosis presented with sudden onset of pain in the right flank. Routine laboratory data were normal except for mild anemia. Computerized tomography revealed a ruptured lesion with low attenuation values in the fat range as well as tumor thrombus extending into the right renal vein and inferior vena cava. Magnetic resonance imaging confirmed a right renal mass extending into the right atrium through the right renal vein and inferior vena cava (fig. 1). A 1 cm. tumor was also noted in the left kidney. All findings were diagnostic of bilateral renal angiomyolipoma associated with right renal vein and inferior vena caval invasion as well as right atrial thrombosis and right renal rupture. Conservative treatment resulted in improved laboratory findings and symptoms. However, the potentially lethal nature of an atrial thrombus prompted radical surgery. Right nephrectomy and

Spontaneous subcapsular or perirenal haemorrhage caused by renal tumours. A urological emergency.

Spontaneous perirenal haemorrhage is a rare abdominal emergency most commonly caused by solid renal tumours. The aim of this study was to evaluate the efficacy of different diagnostic methods and treatment modalities. A total of 9 patients (5 women and 4 men) with spontaneous subcapsular or perirenal haemorrhage caused by renal tumours were treated at two Finnish central hospitals over a period of 20 years. All the patients presented with flank pain, often severe and associated with a palpable mass and a reduced haemoglobin concentration. Ultrasonography was abnormal in all cases where it was used, but was able to show the tumour and haemorrhage correctly in only one case (13%). Computed tomography had a sensitivity of 71%. Seven patients underwent extrafascial nephrectomy (5 renal cell cancers, 1 malignant oncocytoma and 1 angiomyolipoma) and two with known tuberous sclerosis and bilateral renal angiomyolipomas were treated by superselective embolization. As these few cases were all individual and were collected over a long period of time, general statements about diagnosis and treatment must be approached critically. It may be concluded, however, that spontaneous perirenal haemorrhage is often a surgical emergency necessitating great efforts in terms of diagnosis and treatment. Computed tomography should be performed on all patients nowadays. If the bleeding is caused by a malignant tumour, extrafascial nephrectomy is the treatment of choice. For those with benign tumours selective embolization should be used.

結節性硬化症に両側性腎血管筋脂肪腫と多発性腎嚢胞を合併した１透析例

結節性硬化症に両側性腎血管筋脂肪腫と多発性腎嚢胞を合併した１透析例

Bilateral renal angiomyolipoma.

Bilateral renal angiomyolipoma is a rare entity, usually associated with tuberous sclerosis. Five cases of bilateral renal angiomyolipoma, all females, with a mean age of 34.6 years, are reported. All patients had symptoms attributable to lesions only on one side, with flank pain and mass the commonest symptoms encountered. Only one case had clinical evidence of stigmata of tuberous sclerosis. The mean size of the lesions on the symptomatic side was 17 cm, while that on the asymptomatic side was 2 cm. Three patients were diagnosed correctly as having angiomyolipomas preoperatively with the use of ultrasonography and computed tomography (CT); two of these were treated with total nephrectomy and the third with partial nephrectomy. The other two cases were seen before the availability of CT and were only diagnosed intraoperatively. Both patients were treated with total nephrectomy. The lesions on the asymptomatic side were kept on close surveillance. Two patients developed an increase in the size of the lesions in the contralateral kidney 1 year and 3 1/2 years after the first surgery. Both patients were treated with partial nephrectomy. All patients are alive at 3-14 years (mean 6.6 years) after operation.

A hepatic tumor associated with bilateral renal angiomyolipomas: A variant of angiomyolipoma?

Angiomyolipoma is usually derived from the kidney and composed of well developed vessels, smooth muscle and fat tissue. The liver is the only extra-renal site of angiomyolipoma. A peculiar type of hepatic tumor accompanied by bilateral renal angiomyolipomas is reported here. The tumor was mostly composed of large epithelioid cells and a small part of hyalinized large vessels and foam cell infiltration. Mature adipose tissue was absolutely absent. Epithelioid tumor cells arranged in an alveolar pattern had abundant glycogen and some diastase-resistant periodic acid-Schiff granules without obvious crystals. Immunohistochemical studies revealed that the epithelioid cells were positive for melanoma specific antibody (HMB-45), S-100 protein, aromatic L-amino acid decarboxylase and focally alpha-smooth muscle actin. Many melanosome- or premelanosome-like, electron-dense granules were observed in these cells. Thus, these cells were characterized by differentiation to both immature melanocytes and smooth muscle cells. These epithelioid cells were similar to some cells in the renal angiomyolipomas of the same patient. The hepatic tumor was considered to be a result of monotonous proliferation of the epithelioid cells seen in renal angiomyolipoma. Differential diagnosis of this tumor was discussed.

Inferior Vena Caval Tumor Thrombus from Renal Angiomyolipoma

The authors report a case of bilateral renal angiomyolipoma with tumor thrombus from the right kidney extending to the inferior vena cava. Right nephrectomy and total thrombectomy were performed. Vena caval tumor thrombus rarely occurs in patients with angiomyolipomas. To their knowledge, only 6 cases have been reported in the literature.

Bilateral Renal Angiomyolipomatosis in Tuberous Sclerosis Presenting with Chronic Renal Failure: Case Report and Review of the Literature

A 45-year-old patient with tuberous sclerosis and bilateral renal angiomyolipomas who presented with chronic renal failure is reported. Although bilateral renal angiomyolipomas are commonly associated with tuberous sclerosis, the occurrence of chronic renal failure in the absence of associated cystic disease of the kidneys as seen in this patient is very rare.

Bilateral Renal Angiomyolipoma Associated With Bilateral Renal Vein and Inferior Vena Caval Thrombi

We report a case of bilateral renal angiomyolipoma with thrombosis of the bilateral renal veins and inferior vena cava. Right partial nephrectomy and total thrombectomy were performed. To our knowledge only 5 cases of invasion of the inferior vena cava have been described. We report a case in which the renal veins were involved bilaterally. Treatment modalities are discussed.

Coincidental Renal Cell Carcinoma and Renal Angiomyolipomas in Tuberous Sclerosis

We report a case of bilateral renal multiple angiomyolipomas associated with renal cell carcinoma on one side in a patient with tuberous sclerosis. The difficulties in diagnosis and management of such a condition are discussed.

Bilateral Renal Angiomyolipoma: Involvement of the Contralateral Kidney after 25 Years – An Unusual Case

The diagnosis of renal angiomyolipoma often poses problems, both clinically and pathohistologically. With the increasing refinement of imaging techniques it is becoming potentially possible to recognize these benign neoplasms by means of ultrasound, computed tomography and magnetic resonance imaging. Electron microscopy and histochemical methods are often necessary for the correct pathological diagnosis. The problems involved are presented on the basis of the case history of a patient who suffered from renal angiomyolipoma in both kidneys with an interval of 25 years. The first tumor was diagnosed histologically as a liposarcoma 25 years ago.

Symptomatic Bilateral Renal Angiomyolipomas in a Child

We report a case of bilateral renal angiomyolipomas in a child with Bourneville’s tuberous sclerosis. The case is unusual because of the early age of the patient at which symptoms occurred. The presentation and treatment are discussed, and the literature is reviewed. Renal disease can occur in young patients with tuberous sclerosis and it is of clinical importance to document renal involvement in these children.

Angiomyolipomas and polycystic renal disease in tuberous sclerosis ultrastructural observations

We report the ultrastructure of bilateral renal angiomyolipomas in a case of tuberous sclerosis. The patient also had adult-type polycystic renal disease (Potter type 3) with systemic hypertension. Smooth muscle differentiation was supported by immunofluorescence localization of contractile protein using both smooth muscle and platelet-specific antibodies against myosin. There were cells with ultrastructural features intermediate between mature smooth muscle and fat suggesting origin fro progenitor pericytic cells closely related to vascular endothelium. There was also evidence of secretory or synthetic capacity by some mesenchymal cells raising the possibility that hypertension in part may have been hormonally mediated by tumor. A unifying theory of histogenesis is proposed with special reference to the study of Wassermann regarding embryonal lipogenesis.

Renal Angiomyolipoma in a Case of Tuberous Sclerosis, an Electron Microscopy Study

Electron microscopic study of bilateral renal angiomyolipoma in a case of tuberous sclerosis showed two cell types: smooth muscle cells and fibroblasts. There were no tubular or glomerular elements within the tumor mass. To our knowledge this study represents the first ultrastructural demonstration of smooth muscle cells occurring in the renal lesions of this complex.

Angiomyolipomas in a case of lymphangiomyomatosis syndrome: relationships to tuberous sclerosis.

In 17 of the 31 reported cases of lymphangiomyoma, cystic pulmonary changes have been noted which are similar to those seen in some patients with tuberous sclerosis. The contention that lymphangiomyoma represents a forme fruste of tuberous sclerosis is based on this observation, and is strengthened by reports of lymphangiomyoma in patients with tuberous sclerosis and cystic pulmonary changes. Further support is presented here where bilateral renal angiomyolipomas, virtually confined to patients with tuberous sclerosis, were discovered in association with extensive lymphangiomyoma and cystic pulmonary changes.

Renal angiomyolipoma associated with tuberous sclerosis: Review of the literature

A case of bilateral renal angiomyolipoma associated with tuberous sclerosis is presented. Although in our patient right renal biopsy specimen demonstrated angiomyoma, we believe that the lipomatous element could be found in a larger specimen. Roentgenographic and therapeutic considerations are presented and the different lesions of tuberous sclerosis reviewed. Unlike most patients exhibiting stigmata of tuberous sclerosis, our patient had normal mentality and seizures preceded skin lesions. Fundoscopic examination revealed retinal arteriolitis with an occlusive phenomenon which, to our knowledge, has not been reported previously.