Aggressive Angiomyolipomas: the Clandestine Epithelioid Variant

Maré, Anton; Wickramasinghe, Shehan; Ilie, Victor; Mulcahy, Maurice

doi:10.1159/000442853

Cited by 4 publications

(5 citation statements)

References 10 publications

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“…In all cases of mediastinal angiomyolipoma described to date, patients have been treated with surgical excision or selective arterial embolisation. Whilst a watch and wait approach is often adopted in benign appearing renal tumours measuring less than 4cm, no papers found in the literature advocate radiological monitoring of mediastinal tumours [17,18].…”

Section: Discussionmentioning

confidence: 99%

Locally Invasive Angiomyolipoma of the Mediastinum

2024

J Surg

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Section: Discussionmentioning

confidence: 99%

Locally Invasive Angiomyolipoma of the Mediastinum

2024

J Surg

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“…A subset of renal epithelioid angiomyolipomas may show rhabdoid features. [166][167][168] In such cases, the expression of smooth muscle actin, HMB-45 and Melan A in immunohistochemistry is helpful in making the diagnosis.…”

Section: Differential Diagnosismentioning

confidence: 99%

Sarcomatoid and Rhabdoid Renal Cell Carcinoma

Adeniran,

Shuch,

Humphrey

2024

American Journal of Surgical Pathology

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Renal cell carcinoma (RCC) with sarcomatoid and rhabdoid morphologies has an aggressive biological behavior and a typically poor prognosis. The current 2022 WHO classification of renal tumors does not include them as distinct histologic entities but rather as transformational changes that may arise in a background of various distinct histologic types of RCC. The sarcomatoid component shows malignant spindle cells that may grow as intersecting fascicles, which is reminiscent of pleomorphic undifferentiated sarcoma. The rhabdoid cells are epithelioid cells with eccentrically located vesicular nuclei with prominent nucleoli and large intracytoplasmic eosinophilic inclusions. Studies have shown that RCCs with sarcomatoid and rhabdoid differentiation have distinctive molecular features. Sarcomatoid RCC harbors shared genomic alterations in carcinomatous and rhabdoid components, but also enrichment of specific genomic alterations in the sarcomatoid element, suggesting molecular pathways for development of sarcomatoid growth from a common clonal ancestor. Rhabdoid differentiation also arises through clonal evolution although less is known of specific genomic alterations in rhabdoid cells. Historically, treatment has lacked efficacy, although recently immunotherapy with PD-1/PD-L1/CTLA-4 inhibitors has produced significant clinical responses. Reporting of sarcomatoid and rhabdoid features in renal cell carcinoma is required by the College of American Pathologists and the International Collaboration on Cancer Reporting. This manuscript reviews the clinical, pathologic, and molecular features of sarcomatoid RCC and rhabdoid RCC with emphasis on the morphologic features of these tumors, significance of diagnostic recognition, the molecular mechanisms of tumorigenesis and differentiation along sarcomatoid and rhabdoid lines, and advances in treatment, particularly immunotherapy.

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“…5,6 A rare variant of AML is the eosinophilic AML (eAML); first described by Eble et al, 9 it is characterized by predominant perivascular epithelioid cells and, radiologically, it presents distinct differences from AML but close resemblance to RCC. 10 Fat-poor AMLs with high cellular content, eAMLs show variable enhancement on CT and, on MRI, high cellular content with absence of fat. Pathological bleeding, necrosis, and hyalinization contribute to the heterogeneity, making them difficult to distinguish from RCCs on imaging.…”

Section: Palabras Clavementioning

confidence: 99%

“…Pathological bleeding, necrosis, and hyalinization contribute to the heterogeneity, making them difficult to distinguish from RCCs on imaging. [10][11][12]…”

Section: Palabras Clavementioning

confidence: 99%

“…12 However, it is difficult to identify malignancy in eAMLS, as not all cases with cellular atypia are malignant. 10 The adverse histological features associated with malignancy have been described by two large multicenter retrospective studies 22,23 as: a large epithelioid cell component, severe nuclear atypia, extent of nuclear atypia, mitotic count, presence of atypical mitotic figures, necrosis, presence of lymphovascular invasion, tumor size larger than 7 cm, and renal vein invasion.…”

Section: Histological Classificationmentioning

confidence: 99%

See 1 more Smart Citation

Sporadic Renal Angiomyolipoma: Can We Adopt a Uniform Management Protocol?

Ahmed

Teo

Sami

et al. 2022

Revista Urología Colombiana / Colombian Urology Journal

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Renal angiomyolipomas (AMLs), formerly known as PEComas (tumors showing perivascular epithelioid cell differentiation) are common benign renal masses composed of a varying ratio of fat, blood vessels, and smooth muscles. They are largely asymptomatic and diagnosed incidentally on imaging.The adipose tissue content is the factor that gives AMLs their characteristic appearance on imaging and makes them easily identifiable. However, the fat-poor or fat-invisible varieties, which are difficult to differentiate radiologically from renal cell carcinomas (RCCs), present a diagnostic challenge. It is thus essential to establish the diagnosis and identify the atypical and hereditary cases as they require more intense surveillance and management due to their potential for malignant transformation.Multiple management options are available, ranging from conservative approach to embolization and to the more radical option of nephrectomy. While the indications for intervention are relatively clear and aimed at a rather small cohort, the protocol for follow-up of the remainder of the cohort forming the majority of cases is not well established. The surveillance and discharge policies therefore vary between institutions and even between individual practitioners. We have reviewed the literature to establish an optimum management pathway focusing on the typical AMLs.

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Aggressive Angiomyolipomas: the Clandestine Epithelioid Variant

Cited by 4 publications

References 10 publications

Locally Invasive Angiomyolipoma of the Mediastinum

Locally Invasive Angiomyolipoma of the Mediastinum

Sarcomatoid and Rhabdoid Renal Cell Carcinoma

Sporadic Renal Angiomyolipoma: Can We Adopt a Uniform Management Protocol?

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