1/131. Percutaneous nephrolithotomy with renal angiomyolipomas: a rare challenge.Renal angiomyolipomas (AML) are vascular tumors associated with a risk of spontaneous bleeding. Renal trauma may also initiate such hemorrhage. We present a case in which we initially avoided direct puncture and the possible risk of bleeding through extensive renal AMLs and then subsequently performed a direct puncture through the tumors. A 21-year-old obese male patient with tuberous sclerosis and mental retardation presented to our institution with left renal colic and was found to have a staghorn calculus. A CT scan revealed extensive bilateral renal AMLs. The patient had previously undergone renal angioinfarction for an enlarging right-sided AML, and nuclear renography demonstrated 70% function from the left side. The patient had a nephrostomy access created on the morning of a scheduled percutaneous nephrolithotomy under three-dimensional CT guidance. There was no clinically significant bleeding. Intraoperatively, a second access site was required in order to render the patient stone free. This was done using standard biplanar fluoroscopic technique and traversed an AML. Both tracts were balloon dilated prior to placement of a 34F Amplatz sheath. Postoperatively, the patient had an uneventful recovery. A CT scan performed 1 day postoperatively revealed no retroperitoneal collection. This case demonstrates that renal access can be achieved with remarkable accuracy using 3D CT imaging. Furthermore, although this approach seems most prudent in the case of extensive renal AMLs, fluoroscopically guided renal access and dilation to 34F was not associated with bleeding in this patient.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/131. Renal angiomyolipoma resembling gastrointestinal stromal tumor with skenoid fibers.We report an unusual case of renal angiomyolipoma occurring in 68-year-old man. The tumor lacked well-developed vascular and adipose components and was composed almost exclusively of smooth muscle cells. Numerous skenoid-like periodic acid-Schiff-positive globules were interspersed between the tumor cells; the lesion therefore closely resembled a low-grade stromal tumor of the gastrointestinal tract. The HMB45-positive/CD34-negative immunophenotype was essential for the diagnosis of angiomyolipoma. Neither gastrointestinal tumor nor any signs of tuberous sclerosis were found. This lesion should be included in the list of morphologic variations of angiomyolipoma, which may cause diagnostic difficulties.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/131. Composite renal cell carcinoma and angiomyolipoma: a study of the histogenetic relationship of the two lesions.The purpose of the present study was to investigate the possible histogenetic relationship of renal cell carcinoma (RCC) and angiomyolipoma (AMYL) occurring in the same renal nodule by examining two cases of composite RCC and AMYL in patients without stigmata of tuberous sclerosis and by reviewing the medical literature of similar cases. Case 1 represents an epithelioid variant of AMYL with multiple additional nodules of typical AMYL in a surgically removed kidney. The patient subsequently developed a lesion consisting of a mixture of epithelioid variant of AMYL and RCC 24 months later in the retroperitoneum and, an additional 4 months later, in the liver. The RCC cells resembled mononucleated epithelioid cells of the epithelioid AMYL except that they were focally reactive with epithelial membrane antigen (EMA) in the retroperitoneum and focally reactive with both EMA and cytokeratin (CK) in the liver. Case 2 consisted of a typical AMYL admixed with a chromophil cell RCC. A review of the medical literature revealed seven additional cases with histopathological findings similar to this case. All cases had multiple foci of typical AMYL. Immunostaining results are available in five tumors. Chromophil RCC showed variable reactivity with CK and EMA. In addition, RCC in the two cases in the present study also displayed a positive reaction with mucin staining and a positive reactivity with carcinoembryonic antigen. There appears to be a spectrum of histopathological and immunohistochemical changes from the epithelioid variant of AMYL through a mixed epithelioid AMYL/RCC to chromophil RCC in three successive specimens in case 1. Moreover, the intimate admixture of AMYL and RCC and the similar expression of epithelial markers of RCC in the two cases in the present study, as well as other cases in the literature, suggest that some RCC develop from the same precursor cell as AMYL or from a component of AMYL.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/131. tuberous sclerosis associated with multiple hepatic lipomatous tumors and hemorrhagic renal angiomyolipoma.We report a case of tuberous sclerosis associated with hepatic lipomatous tumors and renal angiomyolipomas. Abdominal ultrasonography revealed a high echoic large tumor in the left kidney. A provisional diagnosis of angiomyolipomas of the kidney was made based on computed tomography. Subsequent laparotomy revealed that the extracted tumor was renal angiomyolipoma. It was also revealed that there was an association with hepatic lipomatous tumors thought to be lipomas or angiomyolipomas by liver biopsy. Nearly half of all cases of angiomyolipoma in the kidney are reported as occasional association with tuberous sclerosis complex, but lipomatous tumors in the liver are rare.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
5/131. angiomyolipoma of the parotid gland: a case report.angiomyolipoma is a hamartomatous process that most frequently occurs as a single lesion or multiple foci in the kidneys of patients affected by tuberous sclerosis. angiomyolipoma can also arise in extrarenal sites, among which the liver is the most frequently recorded. Only rare cases of angiomyolipoma located in the head and neck region (ear and oral and nasal cavity) have been described. The purpose of the present article is to report a case of angiomyolipoma of the parotid gland. A 68-year-old woman appeared for treatment with a slow-growing nodule located in her right parotid gland. Ultrasound examination revealed a heterogeneous nodule with well-defined margins. The nodule was surgically removed by total parotidectomy and showed the characteristic appearance of angiomyolipoma, with an admixture of fat smooth muscle cells, and tortuous, thick-walled blood vessels. Careful physical examination of the patient failed to reveal features of tuberous sclerosis. angiomyolipoma should be considered in the differential diagnosis of mesenchymal lesions involving the salivary gland.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
6/131. Mucocutaneous angiomyolipoma. A report of 2 cases arising in the nasal cavity.OBJECTIVE: angiomyolipoma (AML) is a distinctive tumor that usually occurs in the kidney and rarely in the liver. angiomyolipoma of other sites is extremely rare, and, to our knowledge, only 2 cases have been described in the nasal cavity. We present 2 additional cases of AML of the nasal cavity and discuss the difference between them and renal AML. case reports AND PATHOLOGIC FINDINGS: Two tumors in a 66-year-old man and an 88-year-old woman without tuberous sclerosis are described. They showed 20-mm, well-circumscribed, polypoid shapes. Histologically, they were composed of mature smooth muscle cells, fat cells, and various-sized blood vessels. In addition, aggregated small lymphocytes were noted. Neither epithelioid smooth muscle cells nor HMB45 immunoreactivity was seen in either case. CONCLUSIONS: The clinicopathologic features of AML of the nasal cavity are distinct from renal and hepatic AML and are common to those of AML arising in the skin and oral and pharyngeal mucosa. The term mucocutaneous angiomyolipoma is thought to be appropriate to express these characteristic tumors.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/131. Pulmonary angiomyolipoma and multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis.A 36-year-old woman with a long-standing diagnosis of tuberous sclerosis was found dead. A yellow-tan 0.4 cm-diameter pulmonary tumor was identified at autopsy which had typical microscopic features of an angiomyolipoma (AML). Immunohistochemical stains showed reactivity for actin, but not HMB-45, Melan-A, and tyrosinase (despite reactivity of the patient's renal AML for HMB-45 and Melan-A), perhaps owing to the small size of the lesion and the sometimes focal nature of the reactivity for these markers. Additional lung nodules proved to be multifocal micronodular pneumocyte hyperplasia. This report highlights the first occurrence of a pulmonary angiomyolipoma in the setting of tuberous sclerosis.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
8/131. Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: report of a case.tuberous sclerosis is a hereditary autosomal-dominant disease characterized by hamartomas that can develop in any organ. We report herein the case of a 34-year-old female with tuberous sclerosis and a huge abdominopelvic mass that started growing quickly 2 years after its diagnosis. The patient had undergone several previous operations for hydrocephalus and cerebral tubers, and a nephrectomy for right renal angiomyolipoma. On admission, she was in poor general health with renal failure, severe anemia, and weight loss. A laparotomy revealed that the tumor occupied the pelvis, the lower and part of the upper abdomen, and was hypervascularized, with an extremely irregular surface covered in nodules, vegetations, and areas of hemorrhagic necrosis. The development of the mass and the impossibility of recognizing the internal genital organs led us to assume that the formation had originated from these. Frozen-section examination indicated an undifferentiated tumor that had not been completely resected. Her postoperative course was complicated by bronchopneumonia and progressive renal failure. The patient died 10 days after surgery due to cardiorespiratory failure. A histological diagnosis of epithelioid angiomyolipoma was confirmed. Although it is presently impossible to determine whether angiomyolipoma with predominant epithelioid cells is more aggressive than typical angiomyolipoma, it definitively demonstrated local aggressive behavior in this patient.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
9/131. angiomyolipoma of the large intestine: report of a case.A case of an angiomyolipoma of the large intestine occurring in a 55-year-old man without evidence of tuberous sclerosis is reported. Endoscopically, the lesion resembled a sessile adenomatous polyp. The tumor measured 1 cm. Histologic examination revealed a lesion composed predominantly of spindle and epithelioid cells with significant nuclear atypia. Mitoses were rare. The tumor was strongly positive for HMB-45, CD68, vimentin, desmin, and smooth muscle actin. Rare scattered cells reacted with CD34. No residual tumor was found in the resected colon.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/131. Lymphangiectasia within angiomyolipoma in a tuberous sclerosis patient.This is a presentation of a case of tuberous sclerosis with multifocal hamartomas in the retroperitoneum, both kidneys, and the liver. Lymphangiectasia and numerous ectatic lymphatic channels penetrating through the retroperitoneal angiomyolipoma were demonstrated by lymphography and computed tomography. The findings in our study may document the presence of degenerative change in the lymphatic system--not just in elastic vessels--an association which has not yet been reported in the radiological literature.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
| | Next -> |