Angiomiolipoma renale: Nostra esperienza in 14 casi sottoposti a terapia chirurgica

1997 ◽  
Vol 64 (1_suppl) ◽  
pp. 117-122
Author(s):  
G. Deiana ◽  
D. Belussi ◽  
R. Hurle ◽  
A. Losa ◽  
E. Micheli ◽  
...  
Keyword(s):  
Ct Scan ◽  
Tuberous Sclerosis ◽  
Left Kidney ◽  
Correct Diagnosis ◽  
Diagnostic Error ◽  
Renal Angiomyolipoma ◽  
Retroperitoneal Hemorrhage ◽  
Renal Masses ◽  
Minimum Age ◽  
Operative Ultrasound

Renal angiomyolipoma (AML) is an uncommon hamartomatous benign tumour and may present in an isolated form or associated with tuberous sclerosis (Bourneville's disease). Since there is no specific symptomatology when dimensions are small or medium, discovery is often accidental and the tumour may sometimes grow to a considerable size. In the symptomatic forms or larger tumours, surgery may be indicated due to the risk of retroperitoneal hemorrhage, both spontaneous and secondary to lumbar trauma, however slight. As the tumour is benign, kidney-sparing surgery should be as conservative as possible. For this purpose, ultrasound and CT scan are considered essential for a differential diagnosis between AML and parenchymal renal masses. In our experience with 14 cases (12 women and 2 men; minimum age 31 years, maximum 62 years) of monolateral renal AML (10 right kidney AML and 4 left kidney AML) in patients not suffering from tuberous sclerosis, treated surgically between August 1988 and February 1997, the pre-operative ultrasound and CT scan gave a correct diagnosis in 7 cases, a suggestion of renal carcinoma in 5 cases while leaving doubts in 2 cases. The two methods of investigation therefore proved not to be error-free. Following instrumental diagnosis, 12 patients underwent enucleoresection of the tumour while the remaining two underwent extensive nephrectomy. The presence of hemorrhagic areas with AML was considered to be the frequent cause of pre-operative diagnostic error.

scholarly journals Primitive neuroectodermal tumour of the kidney: An unusual case mimicking renal angiomyolipoma with minimal fat

2015 ◽  
Vol 9 (5-6) ◽  
pp. 337 ◽  
Author(s):  
Jing Xie ◽  
Jin Wen ◽  
Ya-lan Bi ◽  
Han-zhong Li
Keyword(s):  
Ct Scan ◽  
Benign Lesion ◽  
Left Kidney ◽  
Immunohistochemical Analysis ◽  
Renal Angiomyolipoma ◽  
Primitive Neuroectodermal Tumour ◽  
Neuroectodermal Tumour ◽  
Positron Emission ◽  
The Central Nervous System ◽  
Minimal Fat

Primitive neuroectodermal tumour (PNET) is a highly aggressive neoplasm that develops classically in the central nervous system. PNET of the kidney (rPNET) is extremely rare. Recently, a 23-yearold woman complained of left flank pain and intermittent hematuria for 3 months and was admitted to our hospital. A computed tomography (CT) scan and magnetic resonance imaging demonstrated a 5.1 × 4.4-cm heterogenous mass with unconspicuous reinforcement in the upper pole of the left kidney. F18-FDG positron emission tomography CT (PET-CT) revealed the mass as a benign lesion with internal extensive bleeding. Renal angiomyolipoma with minimal fat was diagnosed. Three months later, a CT scan showed that the mass shrank to 3.1 × 2.6 cm and nephron-sparing surgery of the left kidney was performed at the patient’s request. However, histologic features and immunohistochemical analysis confirmed the diagnosis of rPNET. Five cycles of combined chemotherapy were executed. At the 11-month follow-up, the patient showed no evidence of metastasis or recurrence.

scholarly journals A Bladder Wall Angiomyolipoma as a Manifestation of Tuberous Sclerosis: First Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Mehmet Kalkan ◽  
Coşkun Şahin ◽  
Ömer Etlik ◽  
Ergun Uçmaklı
Keyword(s):  
Tuberous Sclerosis ◽  
Left Kidney ◽  
Bladder Wall ◽  
Hypovolemic Shock ◽  
Renal Angiomyolipoma ◽  
Lower Pole ◽  
Immunohistochemical Examination ◽  
First Case ◽  
Wall Mass

A 21-year-old female patient admitted to the emergency department complaining of left side pain. Hypovolemic shock, which was probably caused by retroperitoneal bleeding from left sided renal angiomyolipoma, was developed. Abdominal computed tomography showed multiple fat containing lesions in different, regions including right bladder wall, lower pole of left kidney, and right kidney. Some lesions compatible with tuberous sclerosis such as angiofibromas, Shagreen patches, myocardial, and brain hamartomas were also detected. Bladder wall mass showing intra- and extravesical extensions was seen at exploration. We removed the tumor completely preserving bladder trigone. Angiomyolipoma located at lower pole of left kidney was also removed. Diagnosis of bladder angiomyolipoma was confirmed by the immunohistochemical examination. Recurrent or residual mass was not detected at the three-months-follow-up. We report the first case of bladder angiomyolipoma confirmed by histopathologically as a tuberous sclerosis.

scholarly journals Huge Renal Angiomyolipoma in a Child with Tuberous Sclerosis Complex: A Diagnostic and Therapeutic Dilemma

2021 ◽  
Vol 5 (0-2) ◽  
pp. 26
Author(s):  
Sharifah NurDurrah Binti Syed Mudzhar ◽  
Mohd Yusran Othman
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Left Kidney ◽  
Abdominal Distension ◽  
Bleeding Risk ◽  
Subependymal Giant Cell Astrocytoma ◽  
Renal Angiomyolipoma ◽  
Cardiac Rhabdomyoma ◽  
Contralateral Kidney ◽  
Life Threatening

Tuberous Sclerosis Complex (TSC) is a rare neuro-cutaneous disorder that is associated with the development of benign hamartomas including renal angiomyolipoma (RAML). TSC associated RAML are usually asymptomatic, but it carries a life-threatening bleeding risk. We are sharing a case of a 5-year-old girl who was diagnosed to have TSC with associated subependymal giant cell astrocytoma, cardiac rhabdomyoma and autism. She presented with a history of worsening abdominal distension over 3 weeks duration and clinically noted to be pale with a ballotable left flank mass. Ultrasound and CT scan found to have multiple RAML in both kidneys with a huge mass on the left side. The mass represented a huge RAML (8cm) with aneurysmal formation with suspicion of intratumoral bleeding. The option of conservative management with mammalian target of rapamycin inhibitor followed with partial nephrectomy has been questioned with its life-threatening risk of bleeding and inability to do biopsy to rule out the possibility of renal cell carcinoma. Decision for nephrectomy was then made clearer following a MAG-3 scan which revealed only 11% differential function of the left kidney. She underwent a total left nephrectomy uneventfully and intraoperatively noted to have an enlarging lesion as compared to the previous imaging; 15cm in largest diameter. Histopathological finding was consistent with multifocal angiomyolipoma with intratumoral haematoma. Decision for nephrectomy in TSC-associated RAML need to be justified carefully in view of its risk of losing the contralateral kidney following the disease progression which may end up with life-long renal replacement therapy.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S26

scholarly journals Wunderlich’s Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma

2017 ◽  
Vol 18 ◽  
pp. 1309-1314 ◽  
Author(s):  
Sara Catarino Santos ◽  
Liliana Duarte ◽  
Fernando Valério ◽  
Júlio Constantino ◽  
Jorge Pereira ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Renal Angiomyolipoma ◽  
Retroperitoneal Hemorrhage

scholarly journals Giant Bilateral Angiomyolipoma Associated with Tuberous Sclerosis of Bourneville: Exceptional Case and Therapeutic Dilemma

2021 ◽  
pp. 17-21
Author(s):  
Benatta Mahmoud
Keyword(s):  
Serum Creatinine ◽  
Tuberous Sclerosis ◽  
Abdominal Mass ◽  
Left Kidney ◽  
Skin Lesions ◽  
Treatment Modalities ◽  
Surveillance Program ◽  
Abdominal Ultrasonography ◽  
Renal Masses ◽  
Cardiac Rhabdomyoma

Angiomyolipoma (AML) is a mesenchymal tumour composed of variable proportions of adipose tissue and vascular and smooth muscle elements. It can cause potentially life-threatening complications. This report aims to describe a bilateral angiomyolipoma associated with tuberous sclerosis of Bourneville; the second aim is to discuss the treatment modalities of this disease. A 51-year-old woman with abdominal mass and characteristic triad: Epilepsy, mental retardation and skin lesions (adenoma) (Fig. 1), with no notion of hematuria or abdominal pain, abdominal ultrasonography and computed tomography showing bilateral renal masses of 28.4×17 cm on the left kidney and 22× 11.7 cm on the right, respectively (Fig. 2-3). Serum creatinine was 13.4g/l. Body-scan imaging finds cardiac rhabdomyoma (Fig. 4) and brain calcifications (Fig. 5). the patient underwent surveillance with scan imaging every month, and at the last control, she was asymptomatic, serum creatinine was still normal. The management of giant bilateral AML is a complex and multifactorial decision. Patients can knowingly choose an active surveillance program, even with giant AML, but the economic situation and mental status can limit the therapeutic choice.

Prevalence and natural history of sporadic non-tuberous sclerosis associated renal angiomyolipoma

2013 ◽  
Vol 34 (S 01) ◽  
Author(s):  
A Fittschen ◽  
A Akinli ◽  
W Kratzer ◽  
S Oeztuerk ◽  
MM Haenle ◽  
...  
Keyword(s):  
Natural History ◽  
Tuberous Sclerosis ◽  
Renal Angiomyolipoma ◽  
History Of

scholarly journals High-throughput screening of circRNAs reveals novel mechanisms of tuberous sclerosis complex-related renal angiomyolipoma

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yang Zhao ◽  
Hao Guo ◽  
Wenda Wang ◽  
Guoyang Zheng ◽  
Zhan Wang ◽  
...  
Keyword(s):  
Lipid Metabolism ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Regulatory Network ◽  
High Throughput Screening ◽  
Kidney Tissue ◽  
Cell Types ◽  
The Body ◽  
Differentially Expressed ◽  
Renal Angiomyolipoma

Abstract Objective Tuberous sclerosis complex (TSC) is a rare autosomal dominant disease characterized by lesions throughout the body. Our previous study showed the abnormal up-regulation of miRNAs plays an important part in the pathogenesis of TSC-related renal angiomyolipoma (TSC-RAML). circRNAs were known as important regulators of miRNA, but little is known about the circRNAs in TSC-RAMLs. Methods Microarray chips and RNA sequencing were used to identify the circRNAs and mRNAs that were differently expressed between the TSC-RAML and normal kidney tissue. A competitive endogenous RNA (ceRNA) regulatory network was constructed to reveal the regulation of miRNAs and mRNAs by the circRNAs. The biological functions of circRNA and mRNA were analyzed by pathway analysis. Microenvironmental cell types were estimated with the MCP-counter package. Results We identified 491 differentially expressed circRNAs (DECs) and 212 differentially expressed genes (DEGs), and 6 DECs were further confirmed by q-PCR. A ceRNA regulatory network which included 6 DECs, 5 miRNAs, and 63 mRNAs was established. Lipid biosynthetic process was significantly up-regulated in TSC-RAML, and the humoral immune response and the leukocyte chemotaxis pathway were found to be down-regulated. Fibroblasts are enriched in TSC-RAML, and the up-regulation of circRNA_000799 and circRNA_025332 may be significantly correlated to the infiltration of the fibroblasts. Conclusion circRNAs may regulate the lipid metabolism of TSC-RAML by regulation of the miRNAs. Fibroblasts are enriched in TSC-RAMLs, and the population of fibroblast may be related to the alteration of circRNAs of TSC-RAML. Lipid metabolism in fibroblasts is a potential treatment target for TSC-RAML.

The contrast-enhanced CT scan in the diagnosis of isodense subdural hematoma

1979 ◽  
Vol 50 (1) ◽  
pp. 64-69 ◽  
Author(s):  
Fong Y. Tsai ◽  
James E. Huprich ◽  
Hervey D. Segall ◽  
James S. Teal
Keyword(s):  
Cerebral Cortex ◽  
Ct Scan ◽  
Subdural Hematoma ◽  
Correct Diagnosis ◽  
Content Type ◽  
Subdural Hematomas ◽  
Contrast Enhanced Ct ◽  
Contrast Enhanced ◽  
Enhanced Ct ◽  
Fine Print

✓ The authors review 29 cases of surgically-proven isodense subdural hematomas examined by non-contrast and contrast-enhanced computerized tomography scans. Three types of isodense collections were noted: homogeneous isodense collections, mixed-density collections, and gravitational layering within subdural collections. Contrast enhancement within the cerebral cortex, cortical vessels, and subdural membranes led to the correct diagnosis in each case. Contrast-enhanced scans are essential for the evaluation of isodense subdural hematomas.

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