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Presented by Dr. Jonathan Epstein and prepared by Dr. Jennifer Bynum
Case 2: A 30 year old female with ureteropelvic junction obstruction underwent a nephrectomy for a nonfunctioning kidney.
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Question 1 of 1
1. Question
Week (624): Case 2
A 30 year old female with ureteropelvic junction obstruction underwent a nephrectomy for a nonfunctioning kidney.
Correct
Answer: Retention cysts with Mullerian stroma
Histology: Scattered in the kidney are cysts separated by uninvolved kidney. The tubules are lined by hobnail cells. Surrounding the cysts is cellular stroma resembling endometrial stroma cuffed by a circumferential layer of smooth muscle.
Discussion: Both AMLEC and MEST shares some features with this case in that they both often have cysts and Mullerian stroma. However, these lesions are neoplasms and form solitary masses without intervening stroma as seen in the current case. In contrast to endometriosis, the lining of the cysts in this case is hobnail consistent with flattened atrophic renal tubular epithelium. Immunohistochemistry could be misleading as the epithelium lining the cysts would be PAX8 positive and the stroma positive for ER and PR. It has been recognized that in addition to the tumors listed above, mullerian stroma may be seen associated with obstructive changes. It is frequently see around dilated renal pelvic epithelium or collecting ducts and is considered to represent a metaplastic phenomenon.
Reference(s): Tickoo SK, Gopolan A, Tu JJ, et al. Estrogen and progesterone-receptor-positive
stroma as a non-tumorous proliferation in kidneys: a possible metaplastic response
to obstruction. Modern Pathology 2008; 21, 60–65.Incorrect
Answer: Retention cysts with Mullerian stroma
Histology: Scattered in the kidney are cysts separated by uninvolved kidney. The tubules are lined by hobnail cells. Surrounding the cysts is cellular stroma resembling endometrial stroma cuffed by a circumferential layer of smooth muscle.
Discussion: Both AMLEC and MEST shares some features with this case in that they both often have cysts and Mullerian stroma. However, these lesions are neoplasms and form solitary masses without intervening stroma as seen in the current case. In contrast to endometriosis, the lining of the cysts in this case is hobnail consistent with flattened atrophic renal tubular epithelium. Immunohistochemistry could be misleading as the epithelium lining the cysts would be PAX8 positive and the stroma positive for ER and PR. It has been recognized that in addition to the tumors listed above, mullerian stroma may be seen associated with obstructive changes. It is frequently see around dilated renal pelvic epithelium or collecting ducts and is considered to represent a metaplastic phenomenon.
Reference(s): Tickoo SK, Gopolan A, Tu JJ, et al. Estrogen and progesterone-receptor-positive
stroma as a non-tumorous proliferation in kidneys: a possible metaplastic response
to obstruction. Modern Pathology 2008; 21, 60–65.
