Angiomyolipoma with epithelial cysts (AMLEC): A rare but distinct variant of angiomyolipoma

Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Diagnostic Pathology (Impact Factor: 2.6). 02/2007; 2(1):11. DOI: 10.1186/1746-1596-2-11
Source: PubMed


Angiomyolipoma with epithelial cysts (AMLEC) is a recently described distinct cystic variant of angiomyolipoma (AML). To date 15 cases of AMLEC have been reported in 2 case series. We report the 16th case in a 39-year-old female. Her left kidney tumor was discovered incidentally. Partial nephrectomy was performed. Histologically, the tumor was composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) compact subepithelial mullerian-like AML stroma with admixed chronic inflammation; and 3) muscle-predominant AML with dysmorphic blood vessels exterior to the subepithelial stroma. Immunohistochemically, the subepithelial stroma stained most intensely with HMB-45 and Melan-A, whilst the muscle-predominant AML areas stained most intensely with smooth muscle actin and desmin. Estrogen receptor (ER), progesterone receptor (PR), and CD10 stained most intensely in the subepithelial stroma. The cyst lining was positive for pancytokeratin, but negative for HMB-45, Melan-A, ER, PR, and CD10. The patient is alive with no evidence of disease, 12 months postoperatively, and yearly follow-up CT scans are planned.

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Available from: Anil V Parwani, Oct 13, 2015
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    • "Angiomyolipoma is a benign mesenchymal tumor usually occurring in the kidney. It comprises 2.0% to 6.4% of all renal tumors; however, it represents one of the most common benign renal lesions [14]. It can occur sporadically or in patients with tuberous sclerosis. "
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    ABSTRACT: SUMMARY Introduction: Angiomyolipomas belong to the family of PEComas. They are the most common mesenchymal renal tumours. Classic angiomyolipoma is composed of smooth muscle, thick abnormal blood vessels and mature adipose tissue. A new subtype of angiomyolipoma has been described recently, with 16 cases reported to date, contai- ning macro- and/or microscopic epithelial cysts, surrounded by a «cambium-like» layer and with Müllerian type stroma, which are positive for melanocytic markers, CD10 and oes- trogen and progesterone receptors. These neoplasms are more frequent in women and are usually sporadic, although they are can occur as part of the tuberous sclerosis complex. Clinical findings: Here we report a sporadic case of angiomyolipoma with epithelial cysts occurring in a 30 year old man with untreated gynaecomastia who presented with left ureteral lithiasis. An apparently solid 1.8 cm left renal tumour which contained microscopic epithelial cysts was revealed; morphologically it corresponded to an angiomyo- lipoma with epithelial cysts. Discussion: Although these tumours have a distinct histolology, differential diagnosis may include several different benign entities, such as cystic nephroma, mixed epithelial and stromal tumour (MEST) of the kidney, including PEComa, as well as malignant neo- plasms such as renal cell carcinomas (NOS, multicystic and sarcomatoid), amongst others. Angiomyolipoma with epi- thelial cysts behaves in the same benign fashion as angiom- yolipoma.
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