Renal epithelioid angiomyolipoma: Imaging characteristics in nine cases with radiologic-pathologic correlation and review of the literature

Adam T. Froemming, Jennifer Boland, John Cheville, Naoki Takahashi, Akira Kawashima

Research output: Contribution to journalReview article

39 Citations (Scopus)

Abstract

OBJECTIVE. The purpose of this study was to describe the imaging features of renal epithelioid angiomyolipoma (EAML), a rare subtype of angiomyolipoma, with clinical and pathologic correlation. MATERIALS AND METHODS. This study was a retrospective review of nine cases from a single institution in which total resection and preoperative imaging were performed and the diagnosis of EAML was made. Imaging included CT (nine cases), MRI (five cases), and ultrasound (one case), and the images were reviewed in consensus by two radiologists. Patient demographics, disease associations, presentation, and outcomes were determined by chart review. RESULTS. The patients were nine women and one man (mean age, 42 years). Two patients had tuberous sclerosis complex. The size of the nine EAMLs ranged from 1.4 to 22 cm (mean, 7.8 cm). Six lesions had minor components of fat identifiable at imaging. The contrast enhancement pattern was heterogeneous in eight lesions, five of which contained cysts, necrosis, and hematoma. Four presentations were acute hemorrhage, with ruptured EAML in three of the four. Five tumors extended into the renal sinus. Two tumors were locally invasive. One patient had metastatic disease at presentation with epithelioid tumor identified in a single lymph node. The follow-up periods ranged from 0 to 89 months, and there was one case of suspected but not yet proved recurrence. CONCLUSION. Renal EAML can have a range of imaging appearances and can be indistinguishable from renal cell carcinoma and angiomyolipoma with minimal fat. EAML can be considered when a mass is found that has small foci of macroscopic fat without calcification or when acute hemorrhage of a renal mass occurs.

Original languageEnglish (US)
JournalAmerican Journal of Roentgenology
Volume200
Issue number2
DOIs
StatePublished - Feb 2013

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Angiomyolipoma
Kidney
Fats
Hemorrhage
Neoplasms
Tuberous Sclerosis
Renal Cell Carcinoma
Hematoma
Cysts
Necrosis
Lymph Nodes
Demography
Recurrence

Keywords

  • Epithelioid angiomyolipoma
  • Malignant angiomyolipoma
  • Renal mass

ASJC Scopus subject areas

  • Medicine(all)
  • Radiology Nuclear Medicine and imaging

Cite this

Renal epithelioid angiomyolipoma : Imaging characteristics in nine cases with radiologic-pathologic correlation and review of the literature. / Froemming, Adam T.; Boland, Jennifer; Cheville, John; Takahashi, Naoki; Kawashima, Akira.

In: American Journal of Roentgenology, Vol. 200, No. 2, 02.2013.

Research output: Contribution to journalReview article

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N2 - OBJECTIVE. The purpose of this study was to describe the imaging features of renal epithelioid angiomyolipoma (EAML), a rare subtype of angiomyolipoma, with clinical and pathologic correlation. MATERIALS AND METHODS. This study was a retrospective review of nine cases from a single institution in which total resection and preoperative imaging were performed and the diagnosis of EAML was made. Imaging included CT (nine cases), MRI (five cases), and ultrasound (one case), and the images were reviewed in consensus by two radiologists. Patient demographics, disease associations, presentation, and outcomes were determined by chart review. RESULTS. The patients were nine women and one man (mean age, 42 years). Two patients had tuberous sclerosis complex. The size of the nine EAMLs ranged from 1.4 to 22 cm (mean, 7.8 cm). Six lesions had minor components of fat identifiable at imaging. The contrast enhancement pattern was heterogeneous in eight lesions, five of which contained cysts, necrosis, and hematoma. Four presentations were acute hemorrhage, with ruptured EAML in three of the four. Five tumors extended into the renal sinus. Two tumors were locally invasive. One patient had metastatic disease at presentation with epithelioid tumor identified in a single lymph node. The follow-up periods ranged from 0 to 89 months, and there was one case of suspected but not yet proved recurrence. CONCLUSION. Renal EAML can have a range of imaging appearances and can be indistinguishable from renal cell carcinoma and angiomyolipoma with minimal fat. EAML can be considered when a mass is found that has small foci of macroscopic fat without calcification or when acute hemorrhage of a renal mass occurs.

AB - OBJECTIVE. The purpose of this study was to describe the imaging features of renal epithelioid angiomyolipoma (EAML), a rare subtype of angiomyolipoma, with clinical and pathologic correlation. MATERIALS AND METHODS. This study was a retrospective review of nine cases from a single institution in which total resection and preoperative imaging were performed and the diagnosis of EAML was made. Imaging included CT (nine cases), MRI (five cases), and ultrasound (one case), and the images were reviewed in consensus by two radiologists. Patient demographics, disease associations, presentation, and outcomes were determined by chart review. RESULTS. The patients were nine women and one man (mean age, 42 years). Two patients had tuberous sclerosis complex. The size of the nine EAMLs ranged from 1.4 to 22 cm (mean, 7.8 cm). Six lesions had minor components of fat identifiable at imaging. The contrast enhancement pattern was heterogeneous in eight lesions, five of which contained cysts, necrosis, and hematoma. Four presentations were acute hemorrhage, with ruptured EAML in three of the four. Five tumors extended into the renal sinus. Two tumors were locally invasive. One patient had metastatic disease at presentation with epithelioid tumor identified in a single lymph node. The follow-up periods ranged from 0 to 89 months, and there was one case of suspected but not yet proved recurrence. CONCLUSION. Renal EAML can have a range of imaging appearances and can be indistinguishable from renal cell carcinoma and angiomyolipoma with minimal fat. EAML can be considered when a mass is found that has small foci of macroscopic fat without calcification or when acute hemorrhage of a renal mass occurs.

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