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Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review

Epithelioid angiomyolipoma (EAML) is a rare type of mesenchymal angiomyolipoma with potential malignancy in the kidney that can cause lymph node metastases, local recurrence, and distant metastases. Herein, we describe a case of EAML in the right kidney of a 51-year-old man who was admitted to the h...

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Autores principales: Zhang, Jun, Wang, Wen-Juan, Chen, Li-Hong, Wang, Ning, Wang, Ming-Wen, Liu, Hao, Pang, Li-Juan, Jiang, Han-Guo, Qi, Yan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10477911/
https://www.ncbi.nlm.nih.gov/pubmed/37675231
http://dx.doi.org/10.3389/fonc.2023.1207536
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author Zhang, Jun
Wang, Wen-Juan
Chen, Li-Hong
Wang, Ning
Wang, Ming-Wen
Liu, Hao
Pang, Li-Juan
Jiang, Han-Guo
Qi, Yan
author_facet Zhang, Jun
Wang, Wen-Juan
Chen, Li-Hong
Wang, Ning
Wang, Ming-Wen
Liu, Hao
Pang, Li-Juan
Jiang, Han-Guo
Qi, Yan
author_sort Zhang, Jun
collection PubMed
description Epithelioid angiomyolipoma (EAML) is a rare type of mesenchymal angiomyolipoma with potential malignancy in the kidney that can cause lymph node metastases, local recurrence, and distant metastases. Herein, we describe a case of EAML in the right kidney of a 51-year-old man who was admitted to the hospital with a right abdominal mass. Computed tomography revealed a heterogeneously enhanced mass with blurred margins, which was considered a malignant tumor. A radical nephrectomy was then performed. Two years later, the patient developed liver metastases from EAML and was administered sintilimab combined with bevacizumab. The patient survived after 6 months of follow-up. Histologically, the tumors showed clear boundaries and no obvious capsules. The tumor tissue mainly consisted of epithelioid tumor cells, thick-walled blood vessels, and a small amount of adipose tissue. Tumor cells with lipid vacuoles and acinar areas were large, round, polygonal, eosinophilic, or transparent in the cytoplasm. The enlarged and hyperchromatic nuclei were accompanied by distinct nucleoli and pathological mitosis. These histopathological findings resembled those of renal cell carcinoma, and immunohistochemical analysis was performed. The tumor cells were diffusely positive for HMB45, Melan-A, CK20, vimentin antibodies, and TFE3, suggesting that the tumor originated from perivascular epithelioid cells, excluding renal cell carcinoma. The Ki-67 index was 10%. These histopathological features were observed in liver mass puncture tissues. We also summarized 46 cases of EAML with distant metastasis and explored the clinicopathological features of EAML to improve the treatment of the disease. EAML is often ignored in the clinical setting, leading to metastasis and recurrence. Therefore, EAMLs require long-term follow-up, and timely detection of recurrent disease can improve the prognosis.
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spelling pubmed-104779112023-09-06 Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review Zhang, Jun Wang, Wen-Juan Chen, Li-Hong Wang, Ning Wang, Ming-Wen Liu, Hao Pang, Li-Juan Jiang, Han-Guo Qi, Yan Front Oncol Oncology Epithelioid angiomyolipoma (EAML) is a rare type of mesenchymal angiomyolipoma with potential malignancy in the kidney that can cause lymph node metastases, local recurrence, and distant metastases. Herein, we describe a case of EAML in the right kidney of a 51-year-old man who was admitted to the hospital with a right abdominal mass. Computed tomography revealed a heterogeneously enhanced mass with blurred margins, which was considered a malignant tumor. A radical nephrectomy was then performed. Two years later, the patient developed liver metastases from EAML and was administered sintilimab combined with bevacizumab. The patient survived after 6 months of follow-up. Histologically, the tumors showed clear boundaries and no obvious capsules. The tumor tissue mainly consisted of epithelioid tumor cells, thick-walled blood vessels, and a small amount of adipose tissue. Tumor cells with lipid vacuoles and acinar areas were large, round, polygonal, eosinophilic, or transparent in the cytoplasm. The enlarged and hyperchromatic nuclei were accompanied by distinct nucleoli and pathological mitosis. These histopathological findings resembled those of renal cell carcinoma, and immunohistochemical analysis was performed. The tumor cells were diffusely positive for HMB45, Melan-A, CK20, vimentin antibodies, and TFE3, suggesting that the tumor originated from perivascular epithelioid cells, excluding renal cell carcinoma. The Ki-67 index was 10%. These histopathological features were observed in liver mass puncture tissues. We also summarized 46 cases of EAML with distant metastasis and explored the clinicopathological features of EAML to improve the treatment of the disease. EAML is often ignored in the clinical setting, leading to metastasis and recurrence. Therefore, EAMLs require long-term follow-up, and timely detection of recurrent disease can improve the prognosis. Frontiers Media S.A. 2023-08-22 /pmc/articles/PMC10477911/ /pubmed/37675231 http://dx.doi.org/10.3389/fonc.2023.1207536 Text en Copyright © 2023 Zhang, Wang, Chen, Wang, Wang, Liu, Pang, Jiang and Qi https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Zhang, Jun
Wang, Wen-Juan
Chen, Li-Hong
Wang, Ning
Wang, Ming-Wen
Liu, Hao
Pang, Li-Juan
Jiang, Han-Guo
Qi, Yan
Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title_full Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title_fullStr Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title_full_unstemmed Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title_short Primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
title_sort primary renal malignant epithelioid angiomyolipoma with distant metastasis: a case report and literature review
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10477911/
https://www.ncbi.nlm.nih.gov/pubmed/37675231
http://dx.doi.org/10.3389/fonc.2023.1207536
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