Primary kidney malignant epithelioid angiomyolipoma: Two cases report and review of literature

Rationale: Epithelioid angiomyolipoma (EAML) is a subtype of angiomyolipoma with malignant potential. A diagnosis of malignant EAML of the kidney is based on extrarenal metastasis, and predicting early transformation is difficult. To propose criteria for indicators of malignant transformation, herein we report 2 cases and review 17 cases reported in the literature (2000–2017). Patient concerns: Tumor of the kidney was determined in 2 patients, and tissues after nephrectomy were pathologically, histologically, and immunochemically examined. Diagnosis: Malignant EAML. Intervention: The 2 present patients were treated with nephrectomy only. Outcomes: Case 1 involved a 48-year-old woman with a 7.5-cm solid mass in the right kidney who underwent nephrectomy. CT detected a mass in the liver after 13 months, which was speculated to be metastasis from the kidney lesion. Case 2 involved a 62-year-old man with a 7-cm cystic solid mass in the left kidney who accepted nephrectomy and at 10 months post-surgery lived with no disease. Both cases presented a large tumor, atypical epithelioid cells, mitotic figures, and necrosis; tested positive for melanocytic markers (HMB45, MelanA). Lessons: The literature review of malignant EAML led to the identification of 8 malignant features: size ≥5 cm; metastasis; infiltration; necrosis; ≥50% atypical epithelioid cells; cytologic atypia; atypical mitosis; and vessel invasion. The co-existence of at least 5 of these is propos...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research