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Case report


Renal Epithelioid Angiomyolipoma Associated with Pulmonary Lymphangioleiomyomatosis: Imaging Findings.

Athina C TsiliAlexandra NtorkouMaria I Argyropoulou
Date of Submission: 13-Feb-2017, Date of Acceptance: 26-Mar-2017, Date of Web Publication: 22-May-2017.
Corresponding Author:
Corresponding Author

Athina C Tsili

Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina 45110, Greece.
E-mail: a_tsili@yahoo.gr, atsili@cc.uoi.gr

Corresponding Author:
Corresponding Author

Athina C Tsili

Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina 45110, Greece.
E-mail: a_tsili@yahoo.gr, atsili@cc.uoi.gr

DOI: 10.4103/jcis.JCIS_14_17 Facebook Twitter Google Linkedin

ABSTRACT


Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. There are few reports in the English literature addressing on the imaging findings of renal EAMLs, which are considered nonspecific. We present the sonographic, computed tomographic, and magnetic resonance imaging findings of a renal EAML in a pregnant woman with concomitant pulmonary lesions indicative of LAM, without stigmata of TS. We conclude the importance of considering EAML as a possible diagnosis in the presence of a large renal mass with high cellular content and small amounts of fat in the coexistence of pulmonary LAM.
Keywords: Perivascular Epithelioid Cell Tumors, Pulmonary Lymphangioleiomatosis, Renal Angiomyolipoma, Renal Epithelioid Angiomyolipoma

Cited in 2 Documents

  1. Yu-Jing Huang, Zong-Pei Jiang, Yu-Ping Chen, Jin-Quan Wu, Jia-Li Huang, Yan-Qiang Chen and Meng-Jun Liang (2019) Malignancy of renal angiomyolipoma from tuberous sclerosis complex with TSC2 mutation. Chinese Medical Journal 132(1):103. doi: 10.1097/CM9.0000000000000026
  2. Jianguo Zhu, Haige Li, Liucheng Ding and Hongyong Cheng (2018) Imaging appearance of renal epithelioid angiomyolipoma. Medicine 97(1):e9563. doi: 10.1097/MD.0000000000009563

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