Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series

Saoud, Ragheed; Kristof, Tanya W.; Judge, Clark; Chumbalkar, Vaibhav; Antic, Tatjana; Eggener, Scott E.; Modi, Parth K.

doi:10.1016/j.urolonc.2021.09.010

Cited by 10 publications

(14 citation statements)

References 41 publications

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“…11 High-grade features such as increased number of mitotic figures, atypical mitoses, necrosis, LVI, and large tumor size have been reported in cases where epithelioid AML had exhibited malignant behavior. 2,6,[11][12][13][14][15][16] Immunohistochemical studies are helpful in narrowing down the differential diagnosis. Renal oncocytoma is positive for PAX8 and CD117 and shows no to focal expression of KRT7.…”

Section: Discussionmentioning

confidence: 99%

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Oncocytoma-Like Angiomyolipoma of the Kidney: A Closer Look into the Clinicopathologic, Immunohistochemical, and Molecular Characteristics of a Rare Entity with Review of the Literature

et al. 2023

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Introduction. Angiomyolipoma (AML) is a mesenchymal neoplasm that belongs to the perivascular epithelioid cell tumor family (PEComa). AMLs can be subtyped into several patterns dependent on cell type, morphology, and tissue composition. One of the patterns, oncocytoma-like AML is a rare entity with only three cases published in the literature. Case presentation. We present a case of a previously healthy 29-year-old woman who underwent a left partial nephrectomy secondary to a 4.6 cm heterogeneous renal neoplasm. Gross examination demonstrated a well-circumscribed renal mass. Modified Giemsa stain preparation showed oncocytic cells in syncytial pattern with ample granular cytoplasm and round nuclei with prominent nucleoli. Histology assessment showed an oncocytic neoplasm with interspersed adipose tissue. The tumor exhibited tubular architecture with the tubules lined by eosinophilic epithelioid cells with nuclear atypia and prominent nucleoli. Thick blood vessels with emanating epithelioid cells were present. High-grade histology features were not identified. The tumor cells were positive for HMB-45 and SMA and negative for PAX8, keratins, KIT, and vimentin. A diagnosis of oncocytoma-like AML was rendered. Next-generation sequencing (NGS) and RNA fusion were performed. NGS revealed no pathogenic variants and RNA fusion identified no rearrangements. Chromosomal copy number alterations were present in the long arm of chromosome 1 (1p) and chromosome 22. Conclusions. We describe and discuss the clinical, cytomorphologic, histologic, and molecular findings of oncocytoma-like AML, a rare renal neoplasm, and provide a review of the literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Oncocytoma-Like Angiomyolipoma of the Kidney: A Closer Look into the Clinicopathologic, Immunohistochemical, and Molecular Characteristics of a Rare Entity with Review of the Literature

et al. 2023

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“…The diagnosis of a renal EAML can be challenging and elusive as there are no pathognomonic clinical manifestations or imaging characteristics. Specifically, EAMLs are often difficult to distinguish radiographically and histologically from other tumors such as renal cell carcinomas (RCCs), leading to potential misdiagnoses ( 4 , 5 ). Nonetheless, indicators of a potential EAML diagnosis can be gleaned from radiologic findings.…”

Section: Discussionmentioning

confidence: 99%

Massive Malignant Epithelioid Angiomyolipoma of the Kidney

Tessone

Lichtbroun

Srivastava

et al. 2022

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Renal angiomyolipomas (AMLs) are a subset of perivascular epithelioid cell neoplasms (PEComas) that are associated with tuberous sclerosis complex (TSC). Epithelioid angiomyolipomas (EAMLs) are a rare variant of AML with more aggressive propensities. EAMLs with malignant potential can be difficult to distinguish from relatively benign AMLs and other renal tumors. Although there are no established criteria for predicting EAML malignancy, there are proposed histologic parameters that are associated with higher tumor risk. EAML can be treated with surgical resection as well as mTOR inhibitors. Here, we present a unique case of a patient with a 36-cm renal EAML metastatic to the lungs that was treated with complete surgical resection of the primary lesion and mTOR inhibition.

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“…25 Recently, it has been documented that the "conventional pan-melanoma cocktail" can be used to diagnose EAML because the melanocyte markers HMB-45, Melan-A, SOX10, and tyrosinase can be expressed in some EAML, and because S100 is usually not expressed in EAML, it can be used to differentiate from melanoma, but this method requires further testing. [35][36][37][38] In addition to the classic immunohistochemical markers mentioned above, specific immunohistochemical markers of PEComa have been explored in the recent years, and it has been found that cathepsin K and at least one muscle marker are expressed in all PEComas. 39 This is consistent with the results of Caliò et al's study that suggested that cathepsin K may be related to TFE3 rearrangement and mTOR pathway activation and may be used as a target for drug therapy in the future.…”

Section: Histopathological Examinationmentioning

confidence: 99%

“…Compared to EAML, RCC is positive for epithelial markers but negative for melanocytic markers 25 . Recently, it has been documented that the “conventional pan‐melanoma cocktail” can be used to diagnose EAML because the melanocyte markers HMB‐45, Melan‐A, SOX10, and tyrosinase can be expressed in some EAML, and because S100 is usually not expressed in EAML, it can be used to differentiate from melanoma, but this method requires further testing 35–38 . In addition to the classic immunohistochemical markers mentioned above, specific immunohistochemical markers of PEComa have been explored in the recent years, and it has been found that cathepsin K and at least one muscle marker are expressed in all PEComas 39 .…”

Section: Diagnosis Of Renal Epithelioid Angiomyolipomamentioning

confidence: 99%

Advancements in the diagnosis and treatment of renal epithelioid angiomyolipoma: A narrative review

Yang

Liang

2022

The Kaohsiung J of Med Scie

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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.

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Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series

Cited by 10 publications

References 41 publications

Oncocytoma-Like Angiomyolipoma of the Kidney: A Closer Look into the Clinicopathologic, Immunohistochemical, and Molecular Characteristics of a Rare Entity with Review of the Literature

Oncocytoma-Like Angiomyolipoma of the Kidney: A Closer Look into the Clinicopathologic, Immunohistochemical, and Molecular Characteristics of a Rare Entity with Review of the Literature

Massive Malignant Epithelioid Angiomyolipoma of the Kidney

Advancements in the diagnosis and treatment of renal epithelioid angiomyolipoma: A narrative review

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