Renal epithelioid angiomyolipoma: MRI findings

Zhong, Yan; Shen, Yanguang; Pan, Jingjing; Wang, Yingwei; An, Yunxia; Guo, Aitao; Ma, Lu; Ye, Huiyi; Wang, Haiyi

doi:10.1007/s11547-017-0788-9

Cited by 12 publications

(6 citation statements)

References 30 publications

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“…When a fat-containing solid mass growing out of kidney is seen, it is with high probability an angiomyolipoma. On T1 weighted MRI the tumor is hyperintense relative to the renal parenchyma and isointense relative to fat [56,57].…”

Section: Differential Diagnosismentioning

confidence: 99%

Adrenal myelolipoma: a comprehensive review

et al. 2017

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The average age for its diagnosis was 51 years, and no gender difference was observed. The average size of tumors was 10.2 cm. Congenital adrenal hyperplasia was associated to 10% of all cases analyzed, while other adrenal hypersecretory disorders (cortisol, aldosterone) were found in 7.5% of cases. Computed tomography and magnetic resonance imaging can be reliably used for its differential diagnosis. If the diagnosis of an adrenal myelolipoma is unambiguous, and no associated symptoms or hormonal activity are established, surgical intervention is usually not necessary.

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Section: Differential Diagnosismentioning

confidence: 99%

Adrenal myelolipoma: a comprehensive review

et al. 2017

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“…Nonetheless, indicators of a potential EAML diagnosis can be gleaned from radiologic findings. In a study by Zhong et al, renal EAMLs displayed certain characteristics on MRI such as large size (mean diameter of 7.1 cm), exophytic growth, minimal macroscopic fat, microscopic fat, enlarged vessels, massive hemorrhage, and hypointensity on T2 weighted imaging ( 6 ). Furthermore, another study of nine EAML cases indicated that the radiologic finding of a lipid-poor mass without calcification should similarly raise clinical suspicion for a renal EAML diagnosis ( 7 ).…”

Section: Discussionmentioning

confidence: 99%

Massive Malignant Epithelioid Angiomyolipoma of the Kidney

Tessone

Lichtbroun

Srivastava

et al. 2022

jkcvhl

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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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“…[ 23 ] MRI imaging features are a low-to-high T1WI signal (with acute hemorrhage), low T2WI signal with diffusion restriction (due to lesion hypercellularity), lack of fat, distinct margins, and marked heterogeneous enhancement. [ 2 , 24 ] Ultrasonography typically shows hypoechoic mass with heterogeneous peak enhancement or the presence of pseudocapsule. [ 25 , 26 ] In our current report, radiological imaging findings described a heterogeneous soft tissue mass located in the left kidney without fat or calcification.…”

Section: Discussionmentioning

confidence: 99%

Imaging appearance of renal epithelioid angiomyolipoma

Zhu

Li²,

Ding³

et al. 2018

Medicine

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Rationale:Epithelioid angiomyolipoma (EAML) is an extremely rare disease. It commonly occurs in middle-aged females and mainly involves the kidney. Histological and immunohistochemical examination play important roles in differentiating EAML from renal cell carcinoma (RCC) and poor-fat angiomyolipoma (AML).Patient concerns:Here, We report the imaging phenotype, as well as the pathological findings of a case of EAML in a 39-year-old female.Diagnoses:Preoperative noncontrast computed tomography (CT) scan revealed a 6.0 × 5.2 × 7.0 cm soft tissue mass with necrosis, located in the left kidney. On contrast-enhanced CT images, aprogressive enhancement pattern was observed. CT angiography did not show any enlarged vessels or vascular malformation. Abdominal MRI showed a well-circumscribed solid mass with a heterogeneous signal on T1-weighted and T2-weighted images. Ultrasonography of the abdomen demonstrated a hypoechoic mass with abundant blood flow. This patient underwent radical nephrectomy. The pathologic diagnosis was EAML.Interventions:This patient underwent operative resection of the tumor. The resection margins were negative for the neoplastic proliferation and no distant metastases were found. The patient did not receive advanced radiotherapy or chemotherapy.Outcomes:Four months after surgery, the follow-up CT scan did not reveal any local recurrence or distant metastases.Lessons:This case adds to the experience with EAML by summarizing its imaging characteristics as well as reviewing the literature. Additionally, we described the state-of-the-art management of the management of this rare tumor.

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Renal epithelioid angiomyolipoma: MRI findings

Abstract: Although MRI appearances of renal EAML were various, some MRI characteristics may contribute to suggest the possibility of renal EAML.

Cited by 12 publications

References 30 publications

Adrenal myelolipoma: a comprehensive review

Adrenal myelolipoma: a comprehensive review

Massive Malignant Epithelioid Angiomyolipoma of the Kidney

Imaging appearance of renal epithelioid angiomyolipoma

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