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Presented by Pedram Argani, M.D. and prepared by Sharon Swierczynski, M.D., Ph.D.
Case 3: 62-year-old male with an adrenal mass
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1. Question
Week 153: Case 3
62-year-old male with an adrenal mass/images/091503case3fig1.jpg
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/images/091503case3fig5.jpgCorrect
Answer: Ganglioneuroma
Histology: This intra-adrenal lesion contains mature ganglion cells, with surrounding satellite cells; abundant Schwann cells forming fascicles that simulate a smooth muscle tumor; and adipose tissue at the periphery. The Schwann cell component is strongly immunoreactive for S100 protein, while HMB 45 is negative.
Discussion: Angiomyolipoma differs from the current lesion in that it lacks ganglion cells, would label for HMB 45, and would have dysplastic vessels within. This lesion abuts the adjacent normal adrenal medulla, but does not qualify as a composite pheochromocytoma, since this component is non-neoplastic. The fatty tissue at the periphery of this lesion, when involved by Schwann cells, simulates a sclerosing liposarcoma, and this potentially could have been a big problem on a needle biopsy. Attention to the remainder of the lesion, particularly the ganglion cells, and realization that the fat is only at the periphery of this lesion allows the proper interpretation.
Incorrect
Answer: Ganglioneuroma
Histology: This intra-adrenal lesion contains mature ganglion cells, with surrounding satellite cells; abundant Schwann cells forming fascicles that simulate a smooth muscle tumor; and adipose tissue at the periphery. The Schwann cell component is strongly immunoreactive for S100 protein, while HMB 45 is negative.
Discussion: Angiomyolipoma differs from the current lesion in that it lacks ganglion cells, would label for HMB 45, and would have dysplastic vessels within. This lesion abuts the adjacent normal adrenal medulla, but does not qualify as a composite pheochromocytoma, since this component is non-neoplastic. The fatty tissue at the periphery of this lesion, when involved by Schwann cells, simulates a sclerosing liposarcoma, and this potentially could have been a big problem on a needle biopsy. Attention to the remainder of the lesion, particularly the ganglion cells, and realization that the fat is only at the periphery of this lesion allows the proper interpretation.
