A unique radiological pentad of mesonephric duct abnormalities in a young man presenting with testicular swelling.

Cystic dysplasia of the rete testis with associated ipsilateral renal agenesis is a very rare congenital abnormality, mainly described in the paediatric literature. In addition, the presence of a seminal vesicle cyst with ipsilateral renal agenesis is a rare but well-described entity. We present an adult who was found to have a unique pentad of mesonephric duct abnormalities on radiological imaging, including cystic dysplasia of the rete testis, seminal vesicle cyst, ipsilateral renal agenesis, partial hemitrigonal development and epididymal dilatation. We review and discuss the relevant literature of this case and discuss in detail the embryological basis for this unusual combination of findings.

Fetal gonadoblastoid testicular dysplasia: a focal failure of testicular development.

Fetal gonadoblastoid testicular dysplasia (FGTD) is an extremely rare lesion, which, in its original description, appeared in association with hydrops fetalis and other malformations. Its phenotype strongly resembles gonadoblastoma, although in contrast with that rare tumor, FGTD is not associated with the intersexual states or gonadal dysgenesis that accompany such neoplasm. Two reports described an association of FGTD and a morphologically similar lesion with Walker-Warburg syndrome. However, we have not confirmed such an observation, although a nonspecific muscle disorder was found in one of the examples we describe in this article. Here we study 2 additional cases, providing a detailed topographical, histomorphological, and immunophenotypical analysis. A review of all 5 previously described cases is conducted. The features of this lesion support the notion that a focal defect in testicular development is its most likely pathogenesis.

[Increased risk of testicular dysgenesis?]. / Økt forekomst av utviklingsfeil i testiklene?

The latest figures from the Cancer Registry of Norway show that Norway has the highest incidence rate of testicular cancer in the world. They also show that the incidence rate continues to increase, as it has for the last decades in the western world. The reasons for this increase, which might also be true for urogenital abnormalities in men and reduced sperm quality, are uncertain. Data suggest, however, that these anomalies originate in foetal life, and that contributing factors are genetic, pregnancy-related and environmental. The potential importance of environmental factors must be taken seriously, and the authorities must take action to strengthen the research in this area.

Proliferation and functional maturation of Sertoli cells, and their relevance to disorders of testis function in adulthood.

Disorders of testicular function may have their origins in fetal or early life as a result of abnormal development or proliferation of Sertoli cells. Failure of Sertoli cells to mature, with consequent inability to express functions capable of supporting spermatogenesis, is a prime example. In a similar way, failure of Sertoli cells to proliferate normally at the appropriate period in life will result in reduced production of spermatozoa in adulthood. This review focuses on the control of proliferation of Sertoli cells and functional maturation, and is motivated by concerns about 'testicular dysgenesis syndrome' in humans, a collection of common disorders (testicular germ-cell cancer, cryptorchidism, hypospadias and low sperm counts) which are hypothesized to have a common origin in fetal life and to reflect abnormal function of Sertoli (and Leydig) cells. The timing of proliferation of Sertoli cells in different species is reviewed, and the factors that govern the conversion of an immature, proliferating Sertoli cell to a mature, non-proliferating cell are discussed. Protein markers of maturity and immaturity of Sertoli cells in various species are reviewed and their usefulness in studies of human testicular pathology are discussed. These markers include anti-Mullerian hormone, aromatase, cytokeratin-18, GATA-1, laminin alpha5, M2A antigen, p27(kip1), sulphated glycoprotein 2, androgen receptor and Wilms' tumour gene. A scheme is presented for characterization of Sertoli-cell only tubules in the adult testis according to whether or not there is inherent failure of maturation of Sertoli cells or in which the Sertoli cells have matured but there is absence, or acquired loss, of germ cells. Functional 'de-differentiation' of Sertoli cells is considered. It is concluded that there is considerable evidence to indicate that disorders of maturation of Sertoli cells may be a common underlying cause of human male reproductive disorders that manifest at various life stages. This recognition emphasizes the important role that animal models must play to enable identification of the mechanisms via which failure of proliferation and maturation of Sertoli cells can arise, as this failure probably occurs in fetal life.

[Unusual testicular tumor: an ectopic spleen]. / Tumeur testiculaire inhabituelle: une rate ectopique.

The authors report a case of discontinuous splenogonadal fusion diagnosed after left orchidectomy. Ectopic spleen in the scrotum is a rare congential anomaly frequently associated with other anomalies, especially limb defects. The diagnosis is difficult. Preoperative isotope scanning and intraoperative pathological examination can be performed to avoid unnecessary orchidectomy.

Ectopic adrenals in a sirenomelic fetus.

A sirenomelic malformation was diagnosed in a fetus from the first pregnancy of a 25-year-old woman, and it was aborted at the 24th week of gestation. The fetus represented a sympus monopus. The head, organs of the neck, the thorax, and the upper part of abdominal cavity were normally formed. The kidneys, the ureters, the urinary bladder and the urethra were not developed. Adrenals of discoid shape and normal size were present in the normal position. The large bowel ended blindly in the sigmoid colon. Both testes were found in their normal position in the pelvis. Two symmetrical structures, resembling ovaries in shape and colour, were found near the testes. Histological examination established that these two organs were accessory (aberrant) adrenals.

Transverse testicular ectopia with persistent müllerian duct syndrome.

Transverse testicular ectopia is rarely associated with persistent müllerian duct syndrome. The ninth pediatric case of transverse testicular ectopia with persistent müllerian duct syndrome is reported. The clinical and operative findings and treatment are discussed. The importance of abdominal exploration in the presence of two gonads in one inguinal side and the avoidance of dissection of müllerian structures has been stressed.

[Supernumerary testis and homolateral varicocele. Apropos of a case, study of spermatogenesis and review of the literature]. / Testicule surnuméraire et varicocèle homolatéral. A propos d'une observation, étude de la spermatogénèse et revue de la littérature.

Polyorchidism is a rare abnormality. A case is reported of a small scrotal supernumerary testis associated with a large varicocele. A torsion of this testis probably occurred and the vascular pedicle seemed atrophic. Despite abnormal fertility with oligozoospermy the accessary testis was removed. A review of relevant literature is made giving details of embryology of this anomaly.

Ectasia of the rete testis with ipsilateral renal agenesis.

Scrotal masses are a common problem in children. Cystic lesions usually are found to be hydroceles, while solid masses suggest the possibility of neoplasia. A rare cause of a cystic scrotal mass is ectasia of the rete testis. A review of the literature reveals only 1 case similar to the patient herein described. Both patients had agenesis of the ipsilateral kidney, which explained on the basis of the embryologic overlapping of the genitourinary system. Our patient bears clinical similarity to the ACI rat and correlation is made with this animal model system. Excision of the multiloculated rete testis cyst, rather than orchiectomy, appears to be curative.