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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

Histopathological findings of epithelioid angiomyolipoma.Notes: (A) Microscopically, the tumor was composed of epithelioid cells, blood vessels, and adipose tissue (hematoxylin and eosin; magnification, ×100). (B) Higher magnification shows that epithelioid cells exhibited pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (hematoxylin and eosin; magnification, ×200). (C) Tumor cells displayed strongly positive immunoreactivity for HMB-45 (magnification, ×200). (D) Immunohistochemically, the tumor cells showed positive reactivity for MART1 (magnification, ×200).Abbreviation: MART1, melanoma antigen recognized by T-cells 1.
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f2-ott-7-823: Histopathological findings of epithelioid angiomyolipoma.Notes: (A) Microscopically, the tumor was composed of epithelioid cells, blood vessels, and adipose tissue (hematoxylin and eosin; magnification, ×100). (B) Higher magnification shows that epithelioid cells exhibited pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (hematoxylin and eosin; magnification, ×200). (C) Tumor cells displayed strongly positive immunoreactivity for HMB-45 (magnification, ×200). (D) Immunohistochemically, the tumor cells showed positive reactivity for MART1 (magnification, ×200).Abbreviation: MART1, melanoma antigen recognized by T-cells 1.

Mentions: A 27-year-old man with no stigmata of tuberous sclerosis complex (TSC) was referred with left flank dull pain and abdominal fullness for 5 days. Physical examination revealed a large mass in the left upper abdomen. Computed tomography (CT) scan demonstrated multiple masses in both kidneys. Of special interest was a large heterogeneous mass (15.5 cm × 13.0 cm × 10.9 cm) that occupied the upper middle portion of the left kidney (Figure 1). The average diameter was calculated by taking the average of three cases according to the longest diameter of the tumor (the third case taking the longest diameter of the primary tumor in the kidney). No metastatic disease was evident. All findings were diagnostic of bilateral renal AML, and a left nephrectomy was performed. Pathological analysis showed that the left renal tumor was composed predominantly (50%–60%) of epithelioid cells, with smooth muscle, blood vessels, and adipose tissue accounting for the remainder. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (Figure 2A and B). Immunohistochemical studies showed the tumor cells to be positive for human melanosome-associated protein (HMB-45) (Figure 2C) and melanoma antigen recognized by T-cells 1 (MART1) (Figure 2D).


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)

Histopathological findings of epithelioid angiomyolipoma.Notes: (A) Microscopically, the tumor was composed of epithelioid cells, blood vessels, and adipose tissue (hematoxylin and eosin; magnification, ×100). (B) Higher magnification shows that epithelioid cells exhibited pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (hematoxylin and eosin; magnification, ×200). (C) Tumor cells displayed strongly positive immunoreactivity for HMB-45 (magnification, ×200). (D) Immunohistochemically, the tumor cells showed positive reactivity for MART1 (magnification, ×200).Abbreviation: MART1, melanoma antigen recognized by T-cells 1.
© Copyright Policy
Related In: Results  -  Collection

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

f2-ott-7-823: Histopathological findings of epithelioid angiomyolipoma.Notes: (A) Microscopically, the tumor was composed of epithelioid cells, blood vessels, and adipose tissue (hematoxylin and eosin; magnification, ×100). (B) Higher magnification shows that epithelioid cells exhibited pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (hematoxylin and eosin; magnification, ×200). (C) Tumor cells displayed strongly positive immunoreactivity for HMB-45 (magnification, ×200). (D) Immunohistochemically, the tumor cells showed positive reactivity for MART1 (magnification, ×200).Abbreviation: MART1, melanoma antigen recognized by T-cells 1.
Mentions: A 27-year-old man with no stigmata of tuberous sclerosis complex (TSC) was referred with left flank dull pain and abdominal fullness for 5 days. Physical examination revealed a large mass in the left upper abdomen. Computed tomography (CT) scan demonstrated multiple masses in both kidneys. Of special interest was a large heterogeneous mass (15.5 cm × 13.0 cm × 10.9 cm) that occupied the upper middle portion of the left kidney (Figure 1). The average diameter was calculated by taking the average of three cases according to the longest diameter of the tumor (the third case taking the longest diameter of the primary tumor in the kidney). No metastatic disease was evident. All findings were diagnostic of bilateral renal AML, and a left nephrectomy was performed. Pathological analysis showed that the left renal tumor was composed predominantly (50%–60%) of epithelioid cells, with smooth muscle, blood vessels, and adipose tissue accounting for the remainder. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm (Figure 2A and B). Immunohistochemical studies showed the tumor cells to be positive for human melanosome-associated protein (HMB-45) (Figure 2C) and melanoma antigen recognized by T-cells 1 (MART1) (Figure 2D).

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