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Abdominal Imaging Jun 2014Angiomyolipoma is the most common benign solid renal neoplasm observed in clinical practice. Once thought to be a hamartoma and almost always diagnosed by the... (Review)
Review
Angiomyolipoma is the most common benign solid renal neoplasm observed in clinical practice. Once thought to be a hamartoma and almost always diagnosed by the imaged-based detection of fat, angiomyolipomas are now known to consist of a heterogeneous group of neoplasms. Although all are considered perivascular epithelioid cell tumors, many display different pathology, imaging features, and clinical behavior. The importance of understanding this group of neoplasms is emphasized by the fact that many types of angiomyolipoma contain little to no fat, and despite being benign, sometimes escape a pre-operative diagnosis. These types of angiomyolipomas can all be considered when encountering a renal mass that is both hyperattenuating relative to renal parenchyma on unenhanced CT and T2-hypointense, features that reflect their predominant smooth muscle component. We review recent developments and provide a radiological classification of angiomyolipomas that helps physicians understand the various types and learn how to both diagnose and manage them.
Topics: Angiomyolipoma; Diagnosis, Differential; Diagnostic Imaging; Embolization, Therapeutic; Humans; Kidney; Kidney Neoplasms; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Ultrasonography
PubMed: 24504542
DOI: 10.1007/s00261-014-0083-3 -
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 -
Medicine Apr 2018Renal angiomyolipoma (AML) is a common benign tumor of the kidney. The main complication of AML is retroperitoneal hemorrhage caused by AML rupture, which can be severe... (Review)
Review
BACKGROUND
Renal angiomyolipoma (AML) is a common benign tumor of the kidney. The main complication of AML is retroperitoneal hemorrhage caused by AML rupture, which can be severe and life threatening. The risk of AML rupture used to be determined by tumor size. However, these criteria have been challenged by series of clinical studies and case reports, suggesting prediction AML rupture based on tumor size is not always reliable.
METHODS
The authors searched PubMed using "angiomyolipoma," "AML," and "rupture" and reviewed relevant studies. The authors investigated the risk factors of AML rupture using the retrieved literature. The authors also summarized current modalities to evaluate and manage AML.
RESULTS
It is established that risk of AML rupture is associated with lesion size. However, genetic abnormality, aneurysm formation, and pregnancy are also risk factors for tumor rupture. Thus, the prediction of AML rupture should be based on a more comprehensive risk assessment system. The management of renal AML and tumor rupture was also discussed in the present paper.
CONCLUSION
The risk of AML rupture is associated with but not exclusive to lesion size. Any decision to intervene AML must be based on multiple factors including risk, symptoms, and auxiliary findings.
Topics: Angiomyolipoma; Hemorrhage; Humans; Kidney Neoplasms; Retroperitoneal Space; Risk Adjustment; Risk Assessment; Risk Factors; Rupture, Spontaneous
PubMed: 29668633
DOI: 10.1097/MD.0000000000010497 -
Acta Otorrinolaringologica Espanola 2014
Topics: Angiomyolipoma; Humans; Male; Middle Aged; Nose Neoplasms
PubMed: 23433716
DOI: 10.1016/j.otorri.2012.10.005 -
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 -
Clinical Journal of the American... Jul 2017Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys,... (Review)
Review
Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys, brain, heart, lungs, and skin. Tuberous sclerosis complex is usually caused by a mutation in either the or gene, resulting in constitutive activation of mammalian target of rapamycin signaling. Currently, mammalian target of rapamycin inhibitors are recommended in adult patients with tuberous sclerosis complex for the treatment of asymptomatic, growing renal angiomyolipoma that are >3 cm in diameter and pediatric or adult patients with brain lesions (subependymal giant cell astrocytoma) that either are growing or are not amenable to surgical resection. Clinical evidence suggests that systemic administration of a mammalian target of rapamycin inhibitor may provide concurrent improvements in multiple lesions and symptoms of tuberous sclerosis complex. With the major paradigm shift in consensus guidelines toward screening at diagnosis and ongoing monitoring and with the recent availability of an effective oral treatment, it is important that nephrologists have a thorough understanding of our role in the management of patients with tuberous sclerosis complex. Because the various manifestations of tuberous sclerosis complex typically emerge at different periods during patients' lifetimes, patients will need to be followed throughout their lives. Unlike brain and cardiac lesions, renal lesions are more likely to emerge as patients age and can grow at any time. Considerations regarding long-term medication administration for the potential control of multiple tuberous sclerosis complex manifestations will need to be addressed; these include the most appropriate starting dose, appropriate doses for tumor shrinkage versus prevention of regrowth, and management of adverse events. Best practices and potential obstacles for nephrologists treating patients with tuberous sclerosis complex who have multiple manifestations are considered.
Topics: Angiomyolipoma; Antineoplastic Agents; Embolization, Therapeutic; Humans; Kidney Neoplasms; Molecular Targeted Therapy; Mutation; Nephrectomy; Protein Kinase Inhibitors; TOR Serine-Threonine Kinases; Treatment Outcome; Tuberous Sclerosis; Tuberous Sclerosis Complex 1 Protein; Tuberous Sclerosis Complex 2 Protein; Tumor Burden; Tumor Suppressor Proteins
PubMed: 28302901
DOI: 10.2215/CJN.08150816 -
Annals of the Royal College of Surgeons... Jun 2021Angiomyolipoma is a benign solid renal neoplasm. A giant angiomyolipoma is more than 10cm by size, but it can grow to huge proportions. Our case appears to be the third...
Angiomyolipoma is a benign solid renal neoplasm. A giant angiomyolipoma is more than 10cm by size, but it can grow to huge proportions. Our case appears to be the third largest angiomyolipoma and the largest among bilateral giant renal angiomyolipoma in the indexed literature. A 26-year-old man presented with large right abdominal swelling for the past three years, which was occupying his right flank and iliac region, extending beyond the midline. Computed tomography of the abdomen revealed a large well-defined mass in the right side of the abdomen, crossing the midline and measuring 35 × 20 × 12cm. The left kidney showed a similar fatty lesion of 14 × 6cm. The findings were consistent with angiomyolipoma. Further evaluation for tuberous sclerosis by magnetic resonance imaging the brain demonstrated multiple subependymal nodules. Giant renal angiomyolipoma is an uncommon tumour with bilateral giant angiomyolipoma being a rare entity. Preoperative embolisation helps in reducing size of the tumour. In case of giant and bilateral angiomyolipoma, evaluation for tuberous sclerosis should always be done.
Topics: Adult; Angiomyolipoma; Humans; Kidney Neoplasms; Magnetic Resonance Imaging; Male; Neoplasms, Multiple Primary; Tomography, X-Ray Computed; Tuberous Sclerosis; Tumor Burden
PubMed: 33955281
DOI: 10.1308/rcsann.2020.7036 -
International Journal of Surgical... 2016Extrarenal retroperitoneal angiomyolipomas are rare. (Review)
Review
BACKGROUND
Extrarenal retroperitoneal angiomyolipomas are rare.
AIM
To review the literature.
RESULTS
Angiomyolipomas, previously classified as hamartomas, are now classified as benign tumours. Thirty cases of primary retroperitoneal angiomyolipomas have been reported. Diagnosis of the disease upon is based radiological and pathological findings of triphasic features of (a) fat and (b) blood vessels and myoid tissue. Immunohistochemistry tends to be positive for HMB45, MART1, HHF35, calponin, NKI-C3, and CD117. The lesion is common in women. Treatment options have included the following: (a) radical surgical excision of the lesion with renal sparing surgery or radical nephrectomy in cases where malignant tumours could not be excluded and (b) selective embolization of the lesion alone or prior to surgical excision. One case of retroperitoneal angiomyolipoma was reported in a patient 15 years after undergoing radical nephrectomy for angiomyolipoma of kidney and two cases of distant metastases of angiomyolipoma have been reported following radical resection of the tumour.
CONCLUSIONS
With the report of two cases of metastases ensuing surgical resection of the primary lesions there is need for academic pathologists to debate and review angiomyolipomas to decide whether to reclassify angiomyolipomas as slow-growing malignant tumours or whether the reported cases of metastases were de novo tumours or metastatic lesions.
Topics: Actins; Adult; Aged; Aged, 80 and over; Angiomyolipoma; Biomarkers, Tumor; Calcium-Binding Proteins; Diagnosis, Differential; Embolization, Therapeutic; Female; Humans; Immunohistochemistry; Kidney Neoplasms; MART-1 Antigen; Male; Melanoma-Specific Antigens; Microfilament Proteins; Middle Aged; Nephrectomy; Rare Diseases; Retroperitoneal Neoplasms; Sex Factors; Treatment Outcome; gp100 Melanoma Antigen; Calponins
PubMed: 26989509
DOI: 10.1155/2016/6347136 -
Pathologica Oct 2021In this manuscript, we summarize the main features of angiomyolipoma highlighting the recognition of epithelioid angiomyolipoma and the discovery of immunohistochemical... (Review)
Review
In this manuscript, we summarize the main features of angiomyolipoma highlighting the recognition of epithelioid angiomyolipoma and the discovery of immunohistochemical expression of HMB45 in a group of tumors that now are referred to as as PEComas. In this scenario, Dr. Rosai believed in our intuition, demonstrating his intellectual honesty and motivated us with his experience ("") and enthusiasm for the new entities (""). He really pushed the improvement of the knowledge in this field.
Topics: Angiomyolipoma; Humans; Kidney Neoplasms; Male; Perivascular Epithelioid Cell Neoplasms
PubMed: 34837089
DOI: 10.32074/1591-951X-335