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Presented by Jonathan Epstein, M.D. and prepared by Mark Samols, M.D. Ph.D.
Case 1: A 58 year old male was noted to have a 7 cm renal mass.
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1. Question
Week 521: Case 1
A 58 year old male was noted to have a 7 cm renal mass. A radical nephrectomy was performed.images/samols/0723121a.jpg
images/samols/0723121b.jpg
images/samols/0723121c.jpgCorrect
Answer: Angiomyolipoma
Histology: The lesion has the typical appearance of an angiomyolipoma with admixed mature adipose tissue, numerous vessels which are abnormal in that they have irregularly thickened walls where spindle cells proliferate off the adventitia, along with an associated spindle cell proliferation. The spindle cell proliferation is typical of PEComa cells consisting of cells with delicate fibrillary (spider web) cytoplasm. There are scattered nuclei within these spindle cells that are hyperchromatic yet overall the lesion is not that cellular, lacks mitotic figures, atypical mitotic figures, and necrosis. The atypia is occurring within spindle cells not epithelioid cells.
Discussion: This lesion is typical of angiomyolipoma. It is not uncommon in angiomyolipomas for the spindle cells to have somewhat enlarged hyperchromatic nuclei yet this has no prognostic or any other clinical significance. The variant of angiomyolipoma that is associated with true clinically malignant behavior is epithelioid angiomyolipoma with atypia. These lesions do not even resemble angiomyolipoma but consist of big pink “amoeboid” cells with abundant eosinophilic cytoplasm that resemble more a pleomorphic carcinoma than angiomyolipoma. In epithelioid angiomyolipoma with atypia, features that have been associated with malignant behavior include greater than 2 mitoses per 10 high power field, necrosis, atypical mitotic figures, vascular invasion, and over 70% of the angiomyolipoma having epithelioid features with atypia.
Incorrect
Answer: Angiomyolipoma
Histology: The lesion has the typical appearance of an angiomyolipoma with admixed mature adipose tissue, numerous vessels which are abnormal in that they have irregularly thickened walls where spindle cells proliferate off the adventitia, along with an associated spindle cell proliferation. The spindle cell proliferation is typical of PEComa cells consisting of cells with delicate fibrillary (spider web) cytoplasm. There are scattered nuclei within these spindle cells that are hyperchromatic yet overall the lesion is not that cellular, lacks mitotic figures, atypical mitotic figures, and necrosis. The atypia is occurring within spindle cells not epithelioid cells.
Discussion: This lesion is typical of angiomyolipoma. It is not uncommon in angiomyolipomas for the spindle cells to have somewhat enlarged hyperchromatic nuclei yet this has no prognostic or any other clinical significance. The variant of angiomyolipoma that is associated with true clinically malignant behavior is epithelioid angiomyolipoma with atypia. These lesions do not even resemble angiomyolipoma but consist of big pink “amoeboid” cells with abundant eosinophilic cytoplasm that resemble more a pleomorphic carcinoma than angiomyolipoma. In epithelioid angiomyolipoma with atypia, features that have been associated with malignant behavior include greater than 2 mitoses per 10 high power field, necrosis, atypical mitotic figures, vascular invasion, and over 70% of the angiomyolipoma having epithelioid features with atypia.
