• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1292-1296
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• 2021

Renal epithelioid angiomyolipoma (EAML) is a rare variant of angiomyolipoma (AML), with a prominent epithelioid component. EAML usually presents as a large heterogeneous soft tissue lesion with intratumoral hemorrhage and variable necrosis or cystic changes. We present a case of multiloculated cystic renal EAML mimicking renal cell carcinoma in a 64-year-old female. Intracystic massive hemorrhage, hyperattenuating wall and septa on an unenhanced study, and enlarged intratumoral vessels can be helpful imaging features for distinguishing renal EAML from renal cell carcinoma.

• Journal of the Korean Society of Radiology
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• v.83 no.3
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• pp.559-581
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• 2022

Although renal angiomyolipoma (AML) is a benign tumor, treatment may be necessary occasionally because it can cause potentially life-threatening retroperitoneal hemorrhage. Transarterial embolization (TAE) is a safe and effective treatment option to prevent the hemorrhagic rupture of AMLs and relieve the symptoms caused by enlarged lesions or active bleeding. However, there is no clear consensus regarding the indications for prophylactic TAE in patients with sporadic renal AMLs. In urgent TAE for bleeding AMLs, there is a likelihood of incomplete embolization when the focus is on stabilizing the clinical symptoms. This pictorial essay discusses the patient selection and technical considerations to achieve optimal therapeutic effects as well as the follow-up findings after TAE.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1109-1115
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• 2022

Renal angiomyolipomas (AMLs) are typically solid tumors, but there have been few reports of a rare cystic variant of AML. AML with epithelial cysts, where the epithelial cyst has a cuboidal epithelial lining, account for the majority of them. Next, epithelioid AML (EAML) with cystic changes due to hemorrhage and necrosis, which is composed of epithelioid cells with abundant eosinophilic cytoplasm, have also been reported. These rare cystic types of AML can be mistaken for other cystic tumors, such as cystic renal cell carcinoma, in preoperative imaging. We report the imaging findings of a rare case of EAML with epithelial cysts.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.178-181
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• 2007

Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

• Tuberculosis and Respiratory Diseases
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• v.48 no.5
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• pp.788-793
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• 2000

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involved by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.50 no.2
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• pp.245-251
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• 2001

Recently we have experienced one case of pulmonary lymphangioleiomyomatosis(LAM). A 49 year-old woman visited the outpatient department complaining of longstanding dyspnea, which was aggravated by exercise. Although the chest PA film showed nothing more than a slight increase in interstitial marking, a lung HRCT revealed multiple cystic lesions of a similar size that were scattered through out the whole field in both lungs. An abdominal CT detected an angiomyolipoma located in the midbody of the left kidney. Video-assisted thoracic surgery(VATS) was performed for the pathologic diagnosis. On gross examination of the biopsy lung, a pulmonary LAM was confirmed by a finding of smooth muscle proliferation in the interstitum of the lung. After the final diagnosis, oral medroxyprogesterone was prescribed and she is presently in a stable condition.

• The Journal of the Korean bone and joint tumor society
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• v.11 no.1
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• pp.25-31
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• 2005

Purpose: This study was designed to know the usefulness of the MRI to distinguish lipoma and well differentiated liposarcoma (WDL). Materials and methods: 47 lipomatous tumors with MRI were reviewed among the 107 lipomatous tumors operated in our department. MRI examinations and their corresponding pathology reports were compared to determine sensitivity, specificity, diagnostic ability, positive predictable value and negative predictable value. Statistical analysis was performed to know the relationship between malignancy of the tumor (WDL) with the age and gender of the patients, and location, depth, size and the enhancement of tumors in MRI. Results: Among 28 lipoma in MRI examinations, 26 were proved as lipoma in pathology, and only 6 were WDL from 19 suspicious lesions in MRI, and others were proved as lipoma variants mostly. The varieties of lipoma variants were fibrolipoma, angiolipoma, spindle cell lipoma, lipoblastoma and angiomyolipoma. The sensitivity, specificity, diagnostic ability, positive predictable value and negative predictable value of MRI were 100%, 68 %, 72%, 31% and 100% in WDL, and 90%, 89%, 89%, 93% and 84% in lipoma. Among the variants to distinguish WDL and lipoma, the size of tumor and enhancement in MRI were significant statistically (p<0.05). Conclusion: MRI was highly sensitive in detection of WDL and highly specific in detection of simple lipoma. The size of tumor and enhancement in MRI were significant variants to distinguish WDL and lipoma. When MRI finding is non-specific, it is more likely to represent one of lipoma variants.

• Tuberculosis and Respiratory Diseases
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• v.49 no.2
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• pp.246-252
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• 2000

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 by the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to a 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 fema1e 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-defined 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.