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Unilateral testicular enlargement in a teenager with Beckwith-Wiedemann syndrome: a case report.
Pellegrin, Maria Chiara; Spinelli, Alessandro Mauro; Tornese, Gianluca; Barbi, Egidio.
Afiliação
  • Pellegrin MC; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Via dell'Istria 65/1, 34137, Trieste, Italy. mariachiara.pellegrin@gmail.com.
  • Spinelli AM; University of Udine, Udine, Italy.
  • Tornese G; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Via dell'Istria 65/1, 34137, Trieste, Italy.
  • Barbi E; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Via dell'Istria 65/1, 34137, Trieste, Italy.
Ital J Pediatr ; 45(1): 79, 2019 Jul 10.
Article em En | MEDLINE | ID: mdl-31291982
ABSTRACT

BACKGROUND:

Beckwith-Wiedemann syndrome (BWS) is a rare congenital overgrowth disorder. A major feature is lateralized overgrowth, which can variably involve a single body district up to the entire hemisome. Visceral asymmetrical involvement has been observed, commonly represented by enlargement of one kidney or adrenal gland, rather than one gonad. CASE PRESENTATION We report the case of a pubertal boy affected by BWS, who developed a progressive testicular enlargement, ipsilateral to the pre-existing external body overgrowth. Asymptomatic unilateral testis enlargement started after regular pubertal onset and worsened over time, without any associated pathological findings in a long-term follow-up. Since biopsy is not indicated in case of benign macro-orchidism, we hypothesize that this asymmetric enlargement could be an expression of visceral lateralized overgrowth in BWS.

CONCLUSIONS:

At the best of our knowledge, this is the first detailed report of unilateral testicular overgrowth in BWS. We revised common causes of painless unilateral scrotal masses in the pediatric age. Considering both the overall frequency of neoplasia and the malignancies predisposition in BWS, a testicular cancer should be carefully ruled out through a close follow-up, before stating a benign condition. A normal ultrasound pattern, together with normal serum hormonal levels and negative tumor markers, make testicular neoplasms highly unlikely.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Testículo / Síndrome de Beckwith-Wiedemann Tipo de estudo: Diagnostic_studies Limite: Adolescent / Humans / Male Idioma: En Revista: Ital J Pediatr Assunto da revista: PEDIATRIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Itália

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Testículo / Síndrome de Beckwith-Wiedemann Tipo de estudo: Diagnostic_studies Limite: Adolescent / Humans / Male Idioma: En Revista: Ital J Pediatr Assunto da revista: PEDIATRIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Itália