Tumor de células de sertoli, un extraño tumor testicular: Nuestra experiencia y revision de la literatura

García, I. Gómez; Molina, Marta Romero; Moreno, Antonio López-García; González, Elena Buendía; Hidalgo, Emilio Rubio; Bolufer, E.; Crespo, Antonio Sampietro; Rodríguez, A. Gómez

doi:10.4321/s0004-06142010000500013

Cited by 8 publications

(1 citation statement)

References 12 publications

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“…The CAIS condition has been related to a higher incidence of TGCT than in the general population [68]. The most common association is reported with seminoma and gonadoblastoma, although other histological forms have been found, such as choriocarcinomas, teratomas, embryonal tumours, adenomas, and Leydig and/or Sertoli cell tumours [69,70].…”

Section: Time Of Gonadectomy and Risk Of Malignancymentioning

confidence: 99%

Different Clinical Presentations and Management in Complete Androgen Insensitivity Syndrome (CAIS)

Lanciotti

Cofini

Leonardi

et al. 2019

IJERPH

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Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive genetic disorder resulting from maternally inherited or de novo mutations involving the androgen receptor gene, situated in the Xq11-q12 region. The diagnosis is based on the presence of female external genitalia in a 46, XY human individual, with normally developed but undescended testes and complete unresponsiveness of target tissues to androgens. Subsequently, pelvic ultrasound or magnetic resonance imaging (MRI) could be helpful in confirming the absence of Mullerian structures, revealing the presence of a blind-ending vagina and identifying testes. CAIS management still represents a unique challenge throughout childhood and adolescence, particularly regarding timing of gonadectomy, type of hormonal therapy, and psychological concerns. Indeed this condition is associated with an increased risk of testicular germ cell tumour (TGCT), although TGCT results less frequently than in other disorders of sex development (DSD). Furthermore, the majority of detected tumoral lesions are non-invasive and with a low probability of progression into aggressive forms. Therefore, histological, epidemiological, and prognostic features of testicular cancer in CAIS allow postponing of the gonadectomy until after pubertal age in order to guarantee the initial spontaneous pubertal development and avoid the necessity of hormonal replacement therapy (HRT) induction. However, HRT is necessary after gonadectomy in order to prevent symptoms of hypoestrogenism and to maintain secondary sexual features. This article presents differential clinical presentations and management in patients with CAIS to emphasize the continued importance of standardizing the clinical and surgical approach to this disorder.

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Section: Time Of Gonadectomy and Risk Of Malignancymentioning

confidence: 99%