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Comprehensive clinical and pathological analysis of aggressive renal epithelioid angiomyolipoma: report of three cases.

Luo J, Liu B, Wang Y, Li J, Wang P, Chen J, Wang C - Onco Targets Ther (2014)

Bottom Line: It can occur in patients with or without tuberous sclerosis.At times, the lesions may extend into the renal vein and inferior vena cava or metastasize to other organs such as the lung and liver.To clarify the biological nature of EAML, three specific cases that we encountered in clinical practice are analyzed and reported in detail.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Zhejiang University, Zhejiang, People's Republic of China.

ABSTRACT
Renal angiomyolipoma (AML) is recognized as a benign hamartomatous lesion arising in the kidney with no obvious malignant potential. However, epithelioid AML (EAML), a rare variant of AML, is potentially malignant, with aggressive clinical features. It can occur in patients with or without tuberous sclerosis. Because EAML may mimic renal cell carcinoma in imaging studies, differentiation of this tumor from renal cell carcinoma preoperatively is difficult. At times, the lesions may extend into the renal vein and inferior vena cava or metastasize to other organs such as the lung and liver. To clarify the biological nature of EAML, three specific cases that we encountered in clinical practice are analyzed and reported in detail.

No MeSH data available.


Related in: MedlinePlus

Radiological and histopathological findings of epithelioid angiomyolipoma.Notes: (A) A 17 cm left renal mass with a tumor thrombus extending into the main renal vein and inferior vena cava. (B) Tumor thrombus extending into the renal vein (hematoxylin and eosin; magnification, ×100).
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f3-ott-7-823: Radiological and histopathological findings of epithelioid angiomyolipoma.Notes: (A) A 17 cm left renal mass with a tumor thrombus extending into the main renal vein and inferior vena cava. (B) Tumor thrombus extending into the renal vein (hematoxylin and eosin; magnification, ×100).

Mentions: A 41-year-old man was referred to our institution complaining of having had left abdominal fullness for 2 months. The physical examination found a large mass in the left upper abdomen and no evidence of TSC. Routine laboratory investigations were within normal limits, except that urinalysis revealed 2+ blood. CT angiography demonstrated a left renal mass (17.0 cm × 13.6 cm × 9.2 cm) with a tumor thrombus extending into the main renal vein and IVC (Figure 3A). A multifocal tumor ranging in diameter from 0.5 to 1.0 cm was also noted in the right kidney. All findings suggested the diagnosis of bilateral renal AML with the left renal vein and IVC invasion. No metastatic disease was evident. Thus, the patient underwent left radical nephrectomy and IVC thrombectomy.


Comprehensive clinical and pathological analysis of aggressive renal epithelioid angiomyolipoma: report of three cases.

Luo J, Liu B, Wang Y, Li J, Wang P, Chen J, Wang C - Onco Targets Ther (2014)

Radiological and histopathological findings of epithelioid angiomyolipoma.Notes: (A) A 17 cm left renal mass with a tumor thrombus extending into the main renal vein and inferior vena cava. (B) Tumor thrombus extending into the renal vein (hematoxylin and eosin; magnification, ×100).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4043816&req=5

f3-ott-7-823: Radiological and histopathological findings of epithelioid angiomyolipoma.Notes: (A) A 17 cm left renal mass with a tumor thrombus extending into the main renal vein and inferior vena cava. (B) Tumor thrombus extending into the renal vein (hematoxylin and eosin; magnification, ×100).
Mentions: A 41-year-old man was referred to our institution complaining of having had left abdominal fullness for 2 months. The physical examination found a large mass in the left upper abdomen and no evidence of TSC. Routine laboratory investigations were within normal limits, except that urinalysis revealed 2+ blood. CT angiography demonstrated a left renal mass (17.0 cm × 13.6 cm × 9.2 cm) with a tumor thrombus extending into the main renal vein and IVC (Figure 3A). A multifocal tumor ranging in diameter from 0.5 to 1.0 cm was also noted in the right kidney. All findings suggested the diagnosis of bilateral renal AML with the left renal vein and IVC invasion. No metastatic disease was evident. Thus, the patient underwent left radical nephrectomy and IVC thrombectomy.

Bottom Line: It can occur in patients with or without tuberous sclerosis.At times, the lesions may extend into the renal vein and inferior vena cava or metastasize to other organs such as the lung and liver.To clarify the biological nature of EAML, three specific cases that we encountered in clinical practice are analyzed and reported in detail.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Zhejiang University, Zhejiang, People's Republic of China.

ABSTRACT
Renal angiomyolipoma (AML) is recognized as a benign hamartomatous lesion arising in the kidney with no obvious malignant potential. However, epithelioid AML (EAML), a rare variant of AML, is potentially malignant, with aggressive clinical features. It can occur in patients with or without tuberous sclerosis. Because EAML may mimic renal cell carcinoma in imaging studies, differentiation of this tumor from renal cell carcinoma preoperatively is difficult. At times, the lesions may extend into the renal vein and inferior vena cava or metastasize to other organs such as the lung and liver. To clarify the biological nature of EAML, three specific cases that we encountered in clinical practice are analyzed and reported in detail.

No MeSH data available.


Related in: MedlinePlus