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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...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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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. |
format | Online Article Text |
id | pubmed-10477911 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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