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Presented by Dr. Epstein and prepared by Dr. Daniel Miller
A 55 year old female was noted to have a kidney mass
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1. Question
A 55 year old female was noted to have a kidney mass.
Choose the correct diagnosis.
Correct
Answer: C. Rena-adrenal fusion with adrenal cortical adenoma
Histological Description: The nephrectomy specimen shows a nodule and a rim along the renal capsule of pale and eosinophilic cells adjacent to and focally infiltrating the kidney. The cells within the nodule are arranged in compact small nests and cords. The paler cells are notable for finely vacuolated cytoplasm. The nodule despite its infiltrative appearance lacks cytological atypia, mitotic figures, necrosis, or vascular invasion.
Discussion: Renal-adrenal fusion varies in its growth from subcapsular lesions that are plaque-like, wedge-shaped, or spherical to irregular nests deep in the renal parenchyma. The adherent and intrarenal adrenal tissue is composed of adrenal cortical tissue, with no adrenal medullary tissue present. At the base of the subcapsular adrenal tissue, a complete fibrous septum separating the adrenal tissue from the renal parenchyma is infrequently present. In the most cases there are nests of clear cells or eosinophilic cells in direct contact with the renal parenchyma. In some cases, adrenal tissue focally extends into renal parenchyma in an infiltrative manner, as seen in this case. In a minority of cases of renal–adrenal fusion, the normal adrenal architecture is well maintained with identification of the three zones of cortical cells. The large clear cells have a characteristic microvesicular pattern, which differs from the dissolved empty cytoplasm of clear cell renal cell carcinoma (RCC) and the fine lightly eosinophilic cytoplasm of chromophobe RCC. Adrenal cortical tissue is usually positive for inhibin, SF-1, and melan A, and negative for PAX8 and pancytokeratin with the opposite pattern for clear cell renal cell carcinoma. Rarely, neoplasms can arise in ectopic adrenal tissue or in cases of renal–adrenal fusion, where involvement of the kidney should not be mistaken for malignancy in an adrenocortical neoplasm. In this case, the adrenal cortical tissue is too expanded for normal adrenal cortical tissue and since a solitary nodule is consistent with an adrenal cortical adenoma arising in renal-adrenal fusion. The expanded adrenocortical tissue of the adenoma lacks features of malignancy other than infiltration of the kidney, which is not atypical in this setting. The cells have no cytologic atypia, not mitotically active, lack necrosis, and show no lymphovascular invasion.
Reference: Ye H, Yoon GS, Epstein JI. Mod Pathol. 2009 Feb;22(2):175-81.
Incorrect
Answer: C. Rena-adrenal fusion with adrenal cortical adenoma
Histological Description: The nephrectomy specimen shows a nodule and a rim along the renal capsule of pale and eosinophilic cells adjacent to and focally infiltrating the kidney. The cells within the nodule are arranged in compact small nests and cords. The paler cells are notable for finely vacuolated cytoplasm. The nodule despite its infiltrative appearance lacks cytological atypia, mitotic figures, necrosis, or vascular invasion.
Discussion: Renal-adrenal fusion varies in its growth from subcapsular lesions that are plaque-like, wedge-shaped, or spherical to irregular nests deep in the renal parenchyma. The adherent and intrarenal adrenal tissue is composed of adrenal cortical tissue, with no adrenal medullary tissue present. At the base of the subcapsular adrenal tissue, a complete fibrous septum separating the adrenal tissue from the renal parenchyma is infrequently present. In the most cases there are nests of clear cells or eosinophilic cells in direct contact with the renal parenchyma. In some cases, adrenal tissue focally extends into renal parenchyma in an infiltrative manner, as seen in this case. In a minority of cases of renal–adrenal fusion, the normal adrenal architecture is well maintained with identification of the three zones of cortical cells. The large clear cells have a characteristic microvesicular pattern, which differs from the dissolved empty cytoplasm of clear cell renal cell carcinoma (RCC) and the fine lightly eosinophilic cytoplasm of chromophobe RCC. Adrenal cortical tissue is usually positive for inhibin, SF-1, and melan A, and negative for PAX8 and pancytokeratin with the opposite pattern for clear cell renal cell carcinoma. Rarely, neoplasms can arise in ectopic adrenal tissue or in cases of renal–adrenal fusion, where involvement of the kidney should not be mistaken for malignancy in an adrenocortical neoplasm. In this case, the adrenal cortical tissue is too expanded for normal adrenal cortical tissue and since a solitary nodule is consistent with an adrenal cortical adenoma arising in renal-adrenal fusion. The expanded adrenocortical tissue of the adenoma lacks features of malignancy other than infiltration of the kidney, which is not atypical in this setting. The cells have no cytologic atypia, not mitotically active, lack necrosis, and show no lymphovascular invasion.
Reference: Ye H, Yoon GS, Epstein JI. Mod Pathol. 2009 Feb;22(2):175-81.
