Testicular Lymphangiectasis in Noonan’s Syndrome

Nistal, Manuel; Paniagua, Ricardo; Bravo, M. P.

doi:10.1016/s0022-5347(17)50612-6

Cited by 17 publications

(9 citation statements)

References 9 publications

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“…Congenital testicular lymphangiectasia in children is associated with normal development of testicular tubules (Nistal et al. 1990); the same authors have described testicular lymphangiectasis as part of Noonan's syndrome in which there was reduced spermatogenic activity (Nistal et al. 1984).…”

Section: Discussionmentioning

confidence: 99%

Bilateral Lymphangiomatous Testicular Lesions in a Lamb

Brown

Smith

Bazely

et al. 2008

Reprod Domestic Animals

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Bilateral testicular enlargement was recognized in a young lamb; both gonads were largely replaced by a mass composed of multiple cystic spaces. Positive immunohistochemical staining for factor VIII-related protein indicated that the cysts were lined by endothelial cells. The precise nature and origin of the lesions is unclear; they might represent hamartomas or benign tumours or may be a result of lymphatic obstruction.

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Section: Discussionmentioning

confidence: 99%

Bilateral Lymphangiomatous Testicular Lesions in a Lamb

Brown

Smith

Bazely

et al. 2008

Reprod Domestic Animals

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“…The fact that the neonatal testicular tissue implanted in castrated male adult rat spleen showed very similar changes to this glandular change in a period between the occurrence of the Leydig cell hyperplasia and the induction of granulosa cell tumors (3, personal communication) suggests that gonadotropin or sex hormones influence the morphological alteration of the Sertoli cell. The mechanical damage to the seminiferous tubule including loss of normal Leydig cells by the ICT may be a factor to facilitate the metaplastic change in the Sertoli cells (9). On the contrary, it is well known that many chemicals induce degeneration of seminiferous tubules when given to experimental animals.…”

Section: Resultsmentioning

confidence: 99%

Glandular Changes Associated with the Spontaneous Interstitial Cell Tumor of the Rat Testes

et al. 1987

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Testes of untreated F344 and Wistar rats in the control groups of carcinogenicity studies were histologically examined, and the histopathological characteristics and histogenesis of glandular changes in these testes were studied. In 266 testes of 2-year-old F344 rats, 263 had interstitial cell tumors (ICTs) (98.9%) and 39 had glandular changes (14.7%). These glandular changes were also found in 1 out of 38 1-year-old F344 rat testes (2.6%), and 3 in 154 2-year-old Wistar rat testes (1.9%). The changes were observed exclusively in the interstitial cell tumors (ICTs). These glandular changes showed variation in size, shape and number. They were composed oftubules or cysts lined by a layer ofcuboidal or columnar epithelial cells, which had terminal bars, occasionally PAS-and alcian blue-positive brush borders, and rarely, alcian blue-positive cytoplasmic vacuoles. Serial sections revealed that the changes were not connected with the rete testes, but with the degenerative seminiferous tubules involved in the ICTs lined by a layer of flat endothelial-like cells. The findings suggest that the lesions constitute metaplastic changes of the Sertoli cells.

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“…Disorder of lymph vessel formation is frequently reported with Noonan syndrome (11, 12). Although lymphedema secondary to disorder of lymph vessel formation is reported in up to 20% of Noonan syndrome patients (3), PLE is rarely reported with Noonan syndrome (10).…”

Section: Discussionmentioning

confidence: 99%

Late-onset Lymphedema and Protein-losing Enteropathy with Noonan Syndrome

Hasegawa

Nagaoka

Maruyama

et al. 2009

Clin Pediatr Endocrinol

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Noonan syndrome is characterized by facial dysmorphology, congenital heart disease and growth failure. Although it is also accompanied by deranged lymph-vessel formation, protein-losing enteropathy (PLE) with Noonan syndrome is rarely reported. We report clinical information about a boy with Noonan syndrome and late-onset lymphedema and PLE after standing for long periods of time during athletic practice sessions. The boy recovered from lymphedema and PLE after administration of 2.5 g of albumin followed by resting and raising his legs. They did not recur after he began walking again. Standing for long periods of time congested the lymph stream at the abdominal lymph vessel, whose formation is frequently disturbed in Noonan syndrome, and the increased pressure caused lymphedema and PLE. PLE is one of the clinical manifestations of Noonan syndrome.

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Testicular Lymphangiectasis in Noonan’s Syndrome

Cited by 17 publications

References 9 publications

Bilateral Lymphangiomatous Testicular Lesions in a Lamb

Bilateral Lymphangiomatous Testicular Lesions in a Lamb

Glandular Changes Associated with the Spontaneous Interstitial Cell Tumor of the Rat Testes

Late-onset Lymphedema and Protein-losing Enteropathy with Noonan Syndrome

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