Abstract:
INTRODUCTION:Pasqualini's syndrome is an isolated, incretory functional disorder of the testes with a secondary Leydig cell insufficiency but, unlike Klinefelder's syndrome, with normal spermiogenesis and fertility. This unusual form of hypogonadism is caused by an inherent LH secretion disorder of the pituitary gland. The result of this is the secondary Leydig cell insufficiency and a corresponding peripheral androgen deficiency. In this communication we present the case of a 67-year-old man with Pasqualini's syndrome and a histologically confirmed left seminoma. In view of the current lack of data and our experience, we describe the properties of this uncommon syndrome; therapy, prognosis and possible relationship with seminoma are discussed in more detail. CASE REPORT:The 67-year-old male presented in our outpatient department with the urgent suspicion of a left testicular tumour as a painless swelling of the testis since about three months. In the case history we found a previously diagnosed hypogonadotropic hypogonadism, which is described in the literature as the so-called Pasqualini syndrome. On palpation, the left testis was about fist-size, scirrhous, mildly pressure sensitive and highly suspicious. Sonography of the testes revealed the enlarged left testis to be completely inhomogeneous with hypo- and hyperdense areas and large spatial requirements with unremarkable perfusion. On the basis of the clinically and sonographically supported diagnosis of a tumour of the left testis and inguinal exposure of the testes was performed. Intraoperative histology of a sample confirmed the diagnosis of a testicular tumour in the sense of a seminoma. A high left ablatio testis was carried out. DISCUSSION:The occurrence of testicular tumours of the seminoma type in association with Pasqualini's syndrome has not previously been described in the literature. Thus, we assume that the seminoma had occurred at this age independently of the Pasqualini syndrome. For the treatment of Pasqualini's syndrome not only hCG but also testosterone have been employed with success. The patient must continue therapy for his whole life. The results of interruption tests confirm that it is not a passing ailment but rather a persisting, endogenous disease. PRACTICAL CONCLUSIONS: Since the molecular genetics and genesis of this disease have not been clarified unambiguously, further clinical and experimental studies are necessary in order to better understand the disease. An increased risk of testicular tumours has not yet been observed on account of the small number of cases of Pasqualini's syndrome.
journal_name
Aktuelle Uroljournal_title
Aktuelle Urologieauthors
Zugor V,Dimmler A,Schrott KM,Schott GEdoi
10.1055/s-2006-932206subject
Has Abstractpub_date
2007-07-01 00:00:00pages
320-3issue
4eissn
0001-7868issn
1438-8820journal_volume
38pub_type
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
doi:10.1055/a-0972-0788
更新日期:2019-12-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/a-1018-1449
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/a-0759-0029
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journal_title:Aktuelle Urologie
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1055/s-2004-818544
更新日期:2004-11-01 00:00:00
abstract::Stress urinary incontinence in women is a common problem in Germany, with approx. 5 million women suffering from incontinence symptoms. These numbers are increasing, due to demographic changes; the suspected numbers are even higher. Prior to treatment, an extended diagnostic approach - including urodynamics and cystos...
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
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journal_title:Aktuelle Urologie
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doi:10.1055/s-0043-121483
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1038282
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journal_title:Aktuelle Urologie
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
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journal_title:Aktuelle Urologie
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pub_type: 杂志文章
doi:10.1055/s-0031-1271544
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journal_title:Aktuelle Urologie
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-2005-915619
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-0029-1224657
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-2003-42001
更新日期:2003-09-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
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更新日期:2009-05-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
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更新日期:2018-09-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-2003-37558
更新日期:2003-01-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
doi:10.1055/a-0828-9936
更新日期:2019-04-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
doi:10.1055/s-0035-1550037
更新日期:2015-09-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-0042-103198
更新日期:2016-05-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
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更新日期:2020-04-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-0031-1271392
更新日期:2011-07-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章
doi:10.1055/s-0041-106151
更新日期:2015-11-01 00:00:00
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journal_title:Aktuelle Urologie
pub_type: 杂志文章,评审
doi:10.1055/s-0042-108871
更新日期:2016-12-01 00:00:00