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A Four-Year Follow-up Study of Renal Epithelioid Angiomyolipoma: A Multi-Center Experience and Literature Review.

Lei JH, Liu LR, Wei Q, Song TR, Yang L, Yuan HC, Jiang Y, Xu H, Xiong SH, Han P - Sci Rep (2015)

Bottom Line: For treatment, surgery resulted in satisfactory short-term prognosis.In conclusion, EAML is a tumor with malignant potential.Once diagnosed, integrated approaches, including surgery, chemotherapy, and targeted therapy, should be considered; a close follow-up regimen is necessary for cases that met: 1) tumor size>9 cm, 2) tumor thrombus formation in the vein, 3) epithelioid cells>70% or atypia cells>60%, and 4) necrosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, West China Hospital, Sichuan University.

ABSTRACT
In this study, we systematically explored the clinical manifestations, diagnosis, treatment, and prognosis of renal epithelioid angiomyolipoma (EAML) retrospectively by analyzing data of 52 patients diagnosed with EAML at four centers. Our results showed that the onset of EAML was usually inconspicuous, and so no obvious symptoms or signs had occurred in most patients at diagnosis. Its diagnoses always depended on postoperative pathological examination. The immunohistochemical (IHC) results [HMB45 ( + ), cytokeratin (-), and S100 (-)] could be used to differentiate EAML from other malignancies such as renal cell cancer (RCC) and sarcomas. For treatment, surgery resulted in satisfactory short-term prognosis. The long-term prognosis of patients with EAML was poor, particularly when a large size, a high percentage of epithelioid component, tumor thrombus formation, and necrosis were present. In conclusion, EAML is a tumor with malignant potential. Once diagnosed, integrated approaches, including surgery, chemotherapy, and targeted therapy, should be considered; a close follow-up regimen is necessary for cases that met: 1) tumor size>9 cm, 2) tumor thrombus formation in the vein, 3) epithelioid cells>70% or atypia cells>60%, and 4) necrosis.

No MeSH data available.


Related in: MedlinePlus

HE staining. The ranges of vision are filled with abundant epithelioid cells, with red cytoplasm and large nuclei; nucleoli were visible, with a certain degree of pleomorphism and mitotic figures. Original magnification ×400.
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f1: HE staining. The ranges of vision are filled with abundant epithelioid cells, with red cytoplasm and large nuclei; nucleoli were visible, with a certain degree of pleomorphism and mitotic figures. Original magnification ×400.

Mentions: Among these three, two had renal hilum and para-aortic lymph node metastasis at diagnosis. During the follow-up, one of the two patients with lymph node metastasis was diagnosed with hepatic and lung metastasis 12 months postoperatively, and died 17 months later (Fig. 1); one patient with renal hilus node metastasis at diagnosis suffered local recurrence 10 months postoperatively and died from serious cancer cachexia 4 months later (Fig. 2); the third case (Fig. 3) and all the others did not suffer any signs of progression and live well.


A Four-Year Follow-up Study of Renal Epithelioid Angiomyolipoma: A Multi-Center Experience and Literature Review.

Lei JH, Liu LR, Wei Q, Song TR, Yang L, Yuan HC, Jiang Y, Xu H, Xiong SH, Han P - Sci Rep (2015)

HE staining. The ranges of vision are filled with abundant epithelioid cells, with red cytoplasm and large nuclei; nucleoli were visible, with a certain degree of pleomorphism and mitotic figures. Original magnification ×400.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4419536&req=5

f1: HE staining. The ranges of vision are filled with abundant epithelioid cells, with red cytoplasm and large nuclei; nucleoli were visible, with a certain degree of pleomorphism and mitotic figures. Original magnification ×400.
Mentions: Among these three, two had renal hilum and para-aortic lymph node metastasis at diagnosis. During the follow-up, one of the two patients with lymph node metastasis was diagnosed with hepatic and lung metastasis 12 months postoperatively, and died 17 months later (Fig. 1); one patient with renal hilus node metastasis at diagnosis suffered local recurrence 10 months postoperatively and died from serious cancer cachexia 4 months later (Fig. 2); the third case (Fig. 3) and all the others did not suffer any signs of progression and live well.

Bottom Line: For treatment, surgery resulted in satisfactory short-term prognosis.In conclusion, EAML is a tumor with malignant potential.Once diagnosed, integrated approaches, including surgery, chemotherapy, and targeted therapy, should be considered; a close follow-up regimen is necessary for cases that met: 1) tumor size>9 cm, 2) tumor thrombus formation in the vein, 3) epithelioid cells>70% or atypia cells>60%, and 4) necrosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, West China Hospital, Sichuan University.

ABSTRACT
In this study, we systematically explored the clinical manifestations, diagnosis, treatment, and prognosis of renal epithelioid angiomyolipoma (EAML) retrospectively by analyzing data of 52 patients diagnosed with EAML at four centers. Our results showed that the onset of EAML was usually inconspicuous, and so no obvious symptoms or signs had occurred in most patients at diagnosis. Its diagnoses always depended on postoperative pathological examination. The immunohistochemical (IHC) results [HMB45 ( + ), cytokeratin (-), and S100 (-)] could be used to differentiate EAML from other malignancies such as renal cell cancer (RCC) and sarcomas. For treatment, surgery resulted in satisfactory short-term prognosis. The long-term prognosis of patients with EAML was poor, particularly when a large size, a high percentage of epithelioid component, tumor thrombus formation, and necrosis were present. In conclusion, EAML is a tumor with malignant potential. Once diagnosed, integrated approaches, including surgery, chemotherapy, and targeted therapy, should be considered; a close follow-up regimen is necessary for cases that met: 1) tumor size>9 cm, 2) tumor thrombus formation in the vein, 3) epithelioid cells>70% or atypia cells>60%, and 4) necrosis.

No MeSH data available.


Related in: MedlinePlus