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Revista Da Associacao Medica Brasileira... Aug 2019Angiomyolipoma is one of the most common benign solid renal tumors. We investigated the characteristics of renal angiomyolipomas and the clinical outcomes of patients in...
INTRODUCTION
Angiomyolipoma is one of the most common benign solid renal tumors. We investigated the characteristics of renal angiomyolipomas and the clinical outcomes of patients in the last thirteen years.
METHODS
The medical records of the patients who underwent nephrectomy were reviewed retrospectively from July 2005 to May 2018. The laboratory data, radiology, and pathology reports were recorded. Patients diagnosed with angiomyolipoma were included in the study.
RESULTS
A total of 28 patients were included in the study, eight of them male. The mean age of the patients was 55.89+14.49 years. The patients were treated with open and laparoscopic techniques. Partial nephrectomy was performed in 12 patients(42.85%). After pathological examination, 23 patients were diagnosed as fat rich, four patients as fat poor, and one as epithelioid angiomyolipoma. There were no recurrences in the follow-up 91.21+48.31 months.
CONCLUSION
Angiomyolipoma is a rare renal tumor in daily urology practice. Clinicians must be aware of its complications and manage patients well.
Topics: Adult; Aged; Angiomyolipoma; Female; Humans; Immunohistochemistry; Kidney Neoplasms; Magnetic Resonance Imaging; Male; Middle Aged; Nephrectomy; Retrospective Studies; Tumor Burden
PubMed: 31389508
DOI: 10.1590/1806-9282.65.7.977 -
Urology Feb 2012Angiomyolipoma is a rare benign tumor most commonly found in the kidney and, infrequently, extrarenally. We report a case of pelvic angiomyolipoma in a male patient...
Angiomyolipoma is a rare benign tumor most commonly found in the kidney and, infrequently, extrarenally. We report a case of pelvic angiomyolipoma in a male patient without stigmata of tuberous sclerosis. The patient presented with right retroperitoneal bleeding and was found to have bilateral renal angiomyolipomas as well as a pelvic mass with similar appearance as the other lesions. He underwent urgent embolization of the large right angiomyolipoma and subsequent robot-assisted left laparoscopic partial nephrectomy with simultaneous resection of the pelvic mass, which was well-tolerated. Pathology confirmed what is, to our knowledge, the only reported case of pelvic angiomyolipoma.
Topics: Angiomyolipoma; Embolization, Therapeutic; Flank Pain; Hemorrhage; Humans; Kidney Neoplasms; Male; Middle Aged; Neoplasms, Multiple Primary; Nephrectomy; Pelvic Neoplasms
PubMed: 21722945
DOI: 10.1016/j.urology.2011.04.019 -
Archives of Pathology & Laboratory... Jun 2016Angiomyolipoma with epithelial cysts is a rare mesenchymal tumor of the kidney that enters in the differential diagnosis of adult cystic renal neoplasms. These tumors... (Review)
Review
Angiomyolipoma with epithelial cysts is a rare mesenchymal tumor of the kidney that enters in the differential diagnosis of adult cystic renal neoplasms. These tumors demonstrate a slight female predominance and can present either incidentally or with symptoms, commonly flank pain and hematuria. Unlike conventional angiomyolipoma, this variant is characterized grossly by both solid and cystic areas, and histologically by the presence of single or multiple cysts lined by epithelial cells, a subepithelial "cambium-like" layer of small stromal cells with a prominent capillary vasculature, and a thick exterior wall composed of poorly formed fascicles of smooth muscle and thick-walled dysplastic blood vessels. Tumors show a distinct immunohistochemical profile and are often reactive for melanocytic markers (HMB-45 and Melan-A), as well as estrogen receptor and progesterone receptor. These tumors have an indolent clinical course, with no reports of progression or metastasis in reported cases thus far.
Topics: Angiomyolipoma; Biomarkers, Tumor; Diagnosis, Differential; Humans; Immunohistochemistry; Kidney Neoplasms
PubMed: 27232352
DOI: 10.5858/arpa.2015-0170-RS -
The Korean Journal of Internal Medicine Nov 2020
Topics: Angiomyolipoma; Female; Hematoma; Hemorrhage; Humans; Kidney; Kidney Neoplasms; Middle Aged
PubMed: 32229794
DOI: 10.3904/kjim.2020.053 -
Kidney International Sep 2004Renal angiomyolipomata can exist as an imaging curiosity or represent a life-threatening condition. There are likely over 10 million people world-wide who have a renal... (Review)
Review
Renal angiomyolipomata can exist as an imaging curiosity or represent a life-threatening condition. There are likely over 10 million people world-wide who have a renal angiomyolipomata and approximately one tenth of these people also have tuberous sclerosis complex. The optimum treatment of angiomyolipomata is focused on sparing renal tissue and has included no intervention, both total and partial nephrectomy, and embolization. As basic science investigation into the biology of angiomyolipomata progresses, there is even hope for successful drug therapy. Because these renal lesions can be associated with other organ system dysfunction, a number of medical specialists become involved in the care of affected patients. The purpose of this article is to update the nephrologist on the molecular understanding of renal angiomyolipomata and for the possibilities of pharmacologic therapy in the future.
Topics: Angiomyolipoma; Animals; Humans; Kidney; Kidney Neoplasms
PubMed: 15327383
DOI: 10.1111/j.1523-1755.2004.00838.x -
Journal of the American Association of... Nov 2013To describe the role of mammalian target of rapamycin (mTOR) inhibition in the treatment of tuberous sclerosis complex (TSC) patients with renal angiomyolipoma in... (Review)
Review
PURPOSE
To describe the role of mammalian target of rapamycin (mTOR) inhibition in the treatment of tuberous sclerosis complex (TSC) patients with renal angiomyolipoma in relation to available clinical data and clinical practice guidance for the nurse practitioner (NP).
DATA SOURCES
A review of the scientific literature, key clinical congresses, and key clinical trials.
CONCLUSIONS
TSC-associated renal angiomyolipomas have a propensity to grow over time and predispose patients to serious and life-threatening consequences. Surgery or invasive interventional therapies may not be the most optimal treatments because of the multiple, bilateral growth pattern of TSC-associated renal angiomyolipomas. Targeted therapies, such as mTOR inhibitors, which have demonstrated efficacy in maintaining and reducing renal angiomyolipoma size, are of great benefit to patients.
IMPLICATIONS FOR PRACTICE
Treatment with everolimus, an oral mTOR inhibitor, offers patients a noninvasive pharmacotherapeutic treatment option. The NP, as a key member of the healthcare team overseeing TSC patients, must be knowledgeable about the safety and efficacy of mTOR inhibitors as their use in the patient population increases.
Topics: Angiomyolipoma; Everolimus; Humans; Immunosuppressive Agents; Kidney Neoplasms; Sirolimus; TOR Serine-Threonine Kinases; Tuberous Sclerosis
PubMed: 24170533
DOI: 10.1002/2327-6924.12081 -
Advancements in the diagnosis and treatment of renal epithelioid angiomyolipoma: A narrative review.The Kaohsiung Journal of Medical... Oct 2022Renal epithelioid angiomyolipoma (EAML) is a unique subtype of angiomyolipoma that contains a variety of cytoplasmic-rich, eosinophilic cytoplasm epithelioid cells in... (Review)
Review
Renal epithelioid angiomyolipoma (EAML) is a unique subtype of angiomyolipoma that contains a variety of cytoplasmic-rich, eosinophilic cytoplasm epithelioid cells in addition to mature adipocytes, hyaline thick-walled vessels, and smooth muscle-like spindle cells. In recent years, increasing evidence has shown that EAML is a potentially malignant tumor. Due to the lack of typical clinical manifestations and imaging features, it is difficult to diagnose before surgery, and the diagnosis mainly depends on postoperative histopathological examination. With the advancement of pathological diagnostic techniques, more EAML cases has been discovered, but clinicians still lack a comprehensive understanding of EAML. This review comprehensively describes some pathological and clinical features of EAML, with special attention to the pathogenesis and treatment of malignant EAML in order to assist with clinical diagnosis and treatment.
Topics: Angiomyolipoma; Epithelioid Cells; Humans; Kidney Neoplasms
PubMed: 36056704
DOI: 10.1002/kjm2.12586 -
Cold Spring Harbor Molecular Case... Apr 2022Tuberous sclerosis complex (TSC) is an inheritable disorder characterized by the formation of benign yet disorganized tumors in multiple organ systems. Germline...
Tuberous sclerosis complex (TSC) is an inheritable disorder characterized by the formation of benign yet disorganized tumors in multiple organ systems. Germline mutations in the (hamartin) or more frequently (tuberin) genes are causative for TSC. The malignant manifestations of TSC, pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML), may also occur as independent sporadic perivascular epithelial cell tumor (PEComa) characterized by somatic mutations. Thus, discerning TSC from the copresentation of sporadic LAM and sporadic AML may be obscured in TSC patients lacking additional features. In this report, we present a case study on a single patient initially reported to have sporadic LAM and a mucinous duodenal adenocarcinoma deficient in DNA mismatch repair proteins. Moreover, the patient had a history of Wilms' tumor, which was reclassified as AML following the LAM diagnosis. Therefore, we investigated the origins and relatedness of these tumors. Using germline whole-genome sequencing, we identified a premature truncation in one of the patient's alleles. Using immunohistochemistry, loss of tuberin expression was observed in AML and LAM tissue. However, no evidence of a somatic loss of heterozygosity or DNA methylation epimutations was observed at the locus, suggesting alternate mechanisms may contribute to loss of the tumor suppressor protein. In the mucinous duodenal adenocarcinoma, no causative mutations were found in the DNA mismatch repair genes , or Rather, clonal deconvolution analyses were used to identify mutations contributing to pathogenesis. This report highlights both the utility of using multiple sequencing techniques and the complexity of interpreting the data in a clinical context.
Topics: Adenocarcinoma; Angiomyolipoma; Female; Humans; Kidney Neoplasms; Leukemia, Myeloid, Acute; Male; Tuberous Sclerosis; Tuberous Sclerosis Complex 2 Protein; Tumor Suppressor Proteins
PubMed: 35483879
DOI: 10.1101/mcs.a006182 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Dec 2022Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear.... (Review)
Review
Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear. Moreover, it is often misdiagnosed. One patient with renal AML complicated with renal vein, IVC, and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University, who was a 35-year-old female, without any previous medical history, presented with right low back pain for more than 3 years. Computed tomography (CT) scan showed irregular lobulated fatty density mass in the right kidney, renal vein, IVC, and right atrium. The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components. Radical resection of the right kidney and removal of tumor thrombus were performed, and there was no recurrence 1 year after the operation. It is rare for renal AML to grow along the renal vein, IVC, and extend to the right atrium. Imaging examination is extremely important, and the CT findings of this case are characteristic, but the diagnosis eventually depends on pathological and immunohistochemical examinations.
Topics: Female; Humans; Adult; Vena Cava, Inferior; Angiomyolipoma; Atrial Fibrillation; Kidney Neoplasms; Embolism; Heart Atria; Leukemia, Myeloid, Acute
PubMed: 36748389
DOI: 10.11817/j.issn.1672-7347.2022.220044 -
Cancer Control : Journal of the Moffitt... Oct 2006Lymphangioleiomyomatosis (LAM) is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to the formation of... (Review)
Review
BACKGROUND
Lymphangioleiomyomatosis (LAM) is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics, and abdominal tumors. It primarily affects women.
METHODS
The authors present a review of large series, registries, and protocols to highlight the prevalence, pathology, clinical features, diagnosis, and treatment options for patients with LAM.
RESULTS
LAM commonly presents with progressive breathlessness or with recurrent pneumothorax, chylothorax, or sudden abdominal hemorrhage. Computed tomography (CT) scans show numerous thin-walled cysts throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show decreased forced expiratory volume in 1 second (FEV1) and diffusion capacity for carbon monoxide (DLCO). Exercise testing shows gas-exchange abnormalities, ventilatory limitation, and hypoxemia that may occur with near-normal lung function.
CONCLUSIONS
No effective treatment currently exists for this progressive disorder. However, recent progress in cancer and smooth muscle cell biology and a better understanding of the factors regulating angiogenesis and lymphangiogenesis may provide a foundation for the development of new therapeutic strategies.
Topics: Angiomyolipoma; Disease Progression; Exercise Tolerance; Humans; Lung Neoplasms; Lymphangioleiomyomatosis; Neoplasm Staging; Prevalence; Respiratory Function Tests; Sex Factors; Tomography, X-Ray Computed
PubMed: 17075565
DOI: 10.1177/107327480601300405