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Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Adult female with kidney tumor.
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Question 1 of 1
1. Question
Adult female with kidney tumor.
Correct
Correct: B.
Histology: This tumor is composed of a spindle cells with abundant light eosinophilic cytoplasm, arranged in a patternless pattern. There are foci of significant nuclear pleomorphism.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epithelioid AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.Incorrect
Correct: B.
Histology: This tumor is composed of a spindle cells with abundant light eosinophilic cytoplasm, arranged in a patternless pattern. There are foci of significant nuclear pleomorphism.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epithelioid AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.
