One of the uncommon urogenital malformations in males, which presents late in the second or third decades of life, is the congenital malformation of the seminal vesicle. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction, and ipsilateral ejaculatory duct obstruction. Very few cases in the literature describe the malignant change in seminal vesicle cyst in Zinner syndrome. To the best of our knowledge, this is the first report of a papillary neoplasm of the seminal vesicle cyst developed in a patient with Zinner syndrome.
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| http://dx.doi.org/10.4103/jiaps.jiaps_84_21 | DOI Listing |
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Zinner Syndrome in a Young Male: A Case Report and Review of the Literature.
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Radiodiagnosis, MNR Medical College and Hospital, Sangareddy, IND.
Zinner syndrome is an extremely uncommon congenital anomaly of the male urogenital tract. It is attributed to an embryological anomaly that arises in the distal segment of the mesonephric or Wolffian duct. It is the inadequate migration of the ureteric bud that contributes to the failure of differentiation of the metanephric blastema, which ultimately results in ipsilateral renal agenesis and atresia of the ejaculatory duct.
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Med J Armed Forces India
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Assistant Professor (Urology), Command Hospital, Central Command, Lucknow, India.
Anomalies of the mesonephric duct are associated with a combination of renal agenesis/dysgenesis, ejaculatory duct obstruction, and seminal vesical cyst, called Zinner syndrome. We present a case of this rare syndrome in a young male patient, who initially presented with urinary symptoms. The relevant embryology and diagnostic modalities are discussed.
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Department of Paediatric Surgery & Urology, Bristol Royal Hospital for Children, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, United Kingdom.
We present the first description of a family in which 2 siblings show alternative expression of CRKL gene deletion as the phenotypes of Zinner (OSVIRA, obstructed seminal vesicle and ipsilateral renal agenesis) and OHVIRA (obstructed hemivagina with an ipsilateral renal anomaly) syndromes. The male infant with Zinner syndrome and his sister aged 5 years with OHVIRA syndrome both have a paternally inherited 703-kb deletion at chromosome 22q11.21 that includes CRKL.
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Department of Radiodiagnosis, Kempegowda Institute of Medical Sciences Hospital and Research Centre, Rajiv Gandhi University of Health Sciences, Bangalore, Karnataka, 560004, India.
Zinner syndrome is a rare congenital malformation of the Wolffian duct with seminal vesicle cyst, ipsilateral renal agenesis and ejaculatory duct obstruction, seen in less than 0.002 % of the male population. An increased awareness among clinicians, urologists, and radiologists is essential, as early diagnosis and management can impact fertility outcomes.
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Department of Urology, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.
Background: Zinner syndrome (ZS) is a congenital malformation characterized by a triad of mesonephric (Wolffian) duct dysplasia, first identified by Zinner in 1914. The classical presentation of ZS includes unilateral renal hypoplasia or dysplasia, ipsilateral seminal vesicle cysts, and obstruction of the ejaculatory duct. This case presents a rare variant of the syndrome, where an ejaculatory duct cyst is observed instead of the typical ipsilateral seminal vesicle cyst.
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