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Presented by Peter Illei, M.D. and prepared by Kara Judson, M.D.
Case 5: This 43-year-old woman had a 3.0 cm incidental right adrenal cyst noted 7 years ago.
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1. Question
Week 263: Case 5
This 43-year-old woman had a 3.0 cm incidental right adrenal cyst noted 7 years ago. Nine months ago she was admitted with a high fever of unknown etiology and CT scan revealed a 5.6 cm right adrenal cyst. Laboratory values including testosterone levels were within normal limits. The patient complained of some weight loss and some right flank pain. The patient had lost approximately 30 pounds in the last several months. She had decreased energy, no libido, and had been treated with testosterone shots. She underwent a right laparascopic adrenalectomy. At gross exam, the adrenal was cystic, measured 6.5 x 6.2 x 3.5 cm and weighed 94.9 g. Upon sectioning, approximately 50cc of non-viscus dark yellow fluid was collected from the cyst. Serial sections showed a multilocular cystic structure of the adrenal gland with one large (~5.0 cm) cystic space largely surrounded by a thin rim of compressed cortex and multiple smaller cysts within the cortical parenchyma.images/4406 case 6 1.jpg
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Answer: Cystic lymphangioma of adrenal gland
Histology: none provided
Discussion: Sections show a large cyst with adjacent small multilocular cystic structures that are separated by thin fibrous septa. The cysts are lined by flattened cells that are CD31 and factor VIII positive and partially CD34 positive, but cytokeratin (AE1/AE3) negative consistent with endothelium. The lumen of the smaller cysts is filled with acellular, homogenous proteinaceous material. These findings are consistent with a cystic lymphangioma of the adrenal gland. Adrenal cysts are space occupying lesions that are bilateral in 5-8% of cases. They are most common in the 5th and 6th decades of life but can be seen at any age (even childhood). They can be divided into four types: parasitic (7%; usually due to echinococcal infection), epithelial (9%), endothelial (45%), and pseudocysts (39%). Epithelial cysts include true glandular or retention cysts, embryonal cysts or cystic neoplasms such as cystic pheochromocytoma. Endothelial cysts can be of lymphangiomatous or angiomatous variants. Pseudocysts are the most common adrenal cysts to present clinically as mass lesion. The histogenesis of adrenal pseudocysts is not clear. In some cases vascular origin is suggested by the flat to cuboidal endothelial lining, whereas other pseudocysts develop following intraadrenal hemorrhage or hemorrhage within an adrenal tumor.
Incorrect
Answer: Cystic lymphangioma of adrenal gland
Histology: none provided
Discussion: Sections show a large cyst with adjacent small multilocular cystic structures that are separated by thin fibrous septa. The cysts are lined by flattened cells that are CD31 and factor VIII positive and partially CD34 positive, but cytokeratin (AE1/AE3) negative consistent with endothelium. The lumen of the smaller cysts is filled with acellular, homogenous proteinaceous material. These findings are consistent with a cystic lymphangioma of the adrenal gland. Adrenal cysts are space occupying lesions that are bilateral in 5-8% of cases. They are most common in the 5th and 6th decades of life but can be seen at any age (even childhood). They can be divided into four types: parasitic (7%; usually due to echinococcal infection), epithelial (9%), endothelial (45%), and pseudocysts (39%). Epithelial cysts include true glandular or retention cysts, embryonal cysts or cystic neoplasms such as cystic pheochromocytoma. Endothelial cysts can be of lymphangiomatous or angiomatous variants. Pseudocysts are the most common adrenal cysts to present clinically as mass lesion. The histogenesis of adrenal pseudocysts is not clear. In some cases vascular origin is suggested by the flat to cuboidal endothelial lining, whereas other pseudocysts develop following intraadrenal hemorrhage or hemorrhage within an adrenal tumor.
