TY - JOUR
T1 - Retroperitoneal Sclerosing Angiomyolipoma with Long-Term Follow up
T2 - A Case Report with Unique Clinicopathologic and Genomic Profile
AU - Jia, Liwei
AU - Panwar, Vandana
AU - Parmley, Michelle
AU - Lucas, Elena
AU - Pedrosa, Ivan
AU - Kapur, Payal
N1 - Funding Information:
The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was partially supported by the NIH/NCI Kidney Cancer SPORE grant (P50CA196516) and endowment from Brock Fund for Medical Science Chair in Pathology and Jan and Bob Pickens Distinguished Professorship in Medical Science.
Publisher Copyright:
© The Author(s) 2021.
PY - 2022/2
Y1 - 2022/2
N2 - Sclerosing angiomyolipoma (sAML) is a rare variant of the perivascular epithelioid tumors exhibiting distinct morphology with extensive stromal hyalinization, which makes it challenging to recognize. It often lacks an adipose tissue component and melanocytic markers may be expressed only focally, further posing a diagnostic challenge. Here, we report a case of sAML of the left pararenal retroperitoneum in a 52-year-old woman with 92 months of clinical follow up and discuss the histologic features, immunoprofile, molecular alterations, and differential diagnoses that can aid in the diagnosis of this unique and rare entity.
AB - Sclerosing angiomyolipoma (sAML) is a rare variant of the perivascular epithelioid tumors exhibiting distinct morphology with extensive stromal hyalinization, which makes it challenging to recognize. It often lacks an adipose tissue component and melanocytic markers may be expressed only focally, further posing a diagnostic challenge. Here, we report a case of sAML of the left pararenal retroperitoneum in a 52-year-old woman with 92 months of clinical follow up and discuss the histologic features, immunoprofile, molecular alterations, and differential diagnoses that can aid in the diagnosis of this unique and rare entity.
KW - perivascular epithelioid tumors (PEComas)
KW - sclerosing angiomyolipoma
KW - transcription factor E3 (TFE3) gene rearrangement
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U2 - 10.1177/10668969211021483
DO - 10.1177/10668969211021483
M3 - Article
C2 - 34106015
AN - SCOPUS:85107443882
SN - 1066-8969
VL - 30
SP - 86
EP - 90
JO - International Journal of Surgical Pathology
JF - International Journal of Surgical Pathology
IS - 1
ER -