Risk of Malignancy and Need for Surgery in Pediatric Patients with Morris or Y-chromosome Turner Syndrome: A Multicenter Survey

Esposito, Ciro; Escolino, Maria; Bagnara, Vincenzo; Eckoldt-Wolke, Felicitas; Bagłaj, Maciej; Saxena, Amulya K.; Patkowski, Dariusz; Schier, Felix; Settimi, Alessandro; Martelli, Hélène; Savanelli, Antonio

doi:10.1016/j.jpag.2014.09.015

Cited by 6 publications

(12 citation statements)

References 15 publications

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“…The risk of gonadal neoplasia is not confined to patients with a 46,XY karyotype but extends to patients with any mosaic karyotype Chertin et al containing a Y-chromosome or the SRY antigen (11). We found in the literature that gonadoblastoma and seminomas are the most frequently occurring malignant tumors in gonadal intersex disorders (34).…”

Section: Discussionmentioning

confidence: 61%

“…Pathology revealed also benign anomalies comprising 1 benign amartoma and 1 Sertoli cell hyperplasia in 2 patients with PAIS (30,34). All results are summarized in Table 2.…”

Section: Incidence Of Gonadal Tumorsmentioning

confidence: 88%

“…We recorded 23 studies, but only 10 papers (27)(28)(29)(30)(31)(32)(33)(34)(35)(36) were included in the analysis since they responded to the selected inclusion criteria.…”

Section: Literature Analysismentioning

confidence: 99%

“…Of the analyzed series, 7/10 reported postoperative diagnosis of gonadal tumors (27)(28)(29)(30)(34)(35)(36). The histopathologic examinations revealed 15 cases of gonadoblastoma, 7 cases of dysgerminoma and 2 cases of seminoma.…”

Section: Incidence Of Gonadal Tumorsmentioning

confidence: 99%

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Laparoscopic approach for gonadectomy in pediatric patients with intersex disorders

Caballero‐Calvo

Escolino²,

Settimi³

et al. 2017

Transl. Pediatr.

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The birth of a child with a disorder of sex development (DSD) prompts a long-term management strategy that involves a myriad of professionals working with the family. There has been progress in diagnosis, surgical techniques and in understanding psychosocial issues related to this condition. However, since these kinds of disorders are rare and have many anatomical variations, individual care is necessary, especially regarding surgical management. Gonadectomy is indicated in a number of intersex disorders with a Y chromosome to reduce the associated risk of cancer. Recently, laparoscopy has gained wide acceptance in pediatric urology. Laparoscopy is also reported to be a useful tool for diagnosing and treating DSD because of its minimal invasiveness and favorable cosmetic outcome. However, reports of evaluation and management using laparoscopy for large numbers of DSD patients are limited and debate is still open about indications and timing of gonadectomy. In this study, we reviewed the literature of the last 10 years about the role of laparoscopic gonadectomy in patients with DSD. In the analyzed papers, all the procedures were accomplished successfully using laparoscopy. No conversions to open surgery neither intra-operative complications were reported in all series. Post-operative complications were reported only in one series and included 1 umbilical port infection [2% (1/50)] and 1 pelvic abscess [2% (1/50)], both treated with antibiotic therapy (grade I Clavien-Dindo). Of the analyzed series, 7/10 reported postoperative diagnosis of gonadal tumors. The histopathologic examinations revealed 15 cases of gonadoblastoma, 7 cases of dysgerminoma and 2 cases of seminoma. Analyzing the single series, the incidence of these tumors varied between 10% and 33%. The results of our review confirmed the safety and efficacy of laparoscopic gonadectomy in DSD patients. In our mind, laparoscopic gonadectomy should be accepted as the treatment of choice in children and adolescents with these rare conditions. It thereby eliminates the risk of malignancies of gonadal origin with the advantages of a minimally invasive procedure, with lower morbidity, quicker postoperative recovery and excellent cosmetic results.

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Section: Discussionmentioning

confidence: 61%

“…Pathology revealed also benign anomalies comprising 1 benign amartoma and 1 Sertoli cell hyperplasia in 2 patients with PAIS (30,34). All results are summarized in Table 2.…”

Section: Incidence Of Gonadal Tumorsmentioning

confidence: 88%

“…We recorded 23 studies, but only 10 papers (27)(28)(29)(30)(31)(32)(33)(34)(35)(36) were included in the analysis since they responded to the selected inclusion criteria.…”

Section: Literature Analysismentioning

confidence: 99%

Section: Incidence Of Gonadal Tumorsmentioning

confidence: 99%

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Laparoscopic approach for gonadectomy in pediatric patients with intersex disorders

Caballero‐Calvo

Escolino²,

Settimi³

et al. 2017

Transl. Pediatr.

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“…Endogenous hormones generally produce more steady puberal development than hormone replacement therapy. Development of malignancies is rare in the pediatric 8 adults, however, have higher chances of developing testicular malignancies if gonadectomy is not performed.…”

mentioning

confidence: 99%

Anterior transperitoneal laparoscopic gonadectomy in a patient with complete androgen insensitivity syndrome (CAIS): case report and surgical technique

et al. 2017

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Introduction: complete androgen insensitivity syndrome is an X-linked mutation that causes a female phenotype, despite normal serum androgen levels. Patients have a 46,XY karyotype, female external genitalia, ectopic testes and absent Müllerian duct remnants. Case report: we present the case and surgical procedure of a 28-year-old patient who underwent laparoscopic gonadectomy in order to prevent gonadal malignancy. Conclusion: laparoscopy allows for the removal of gonads with the advantages of minimally invasive procedures.

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Prophylactic Bilateral Gonadectomy for Ovotesticular Disorder of Sex Development in a Patient With Mosaic 45,X/46,X,idic(Y)q11.222 Karyotype

Becker

Akhavan

2016

Urology Case Reports

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Ovotesticular disorder of sex development is historically thought to confer a relatively low risk of germ cell malignancy relative to other disorders of sex development. This is likely due in part to the high prevalence of a normal 46,XX karyotype in these patients. However, disorders of sex development represent a broad phenotypic spectrum, and often patients cannot be neatly categorized with a single diagnosis. We report an atypical case of ovotesticular disorder of sex development in a child with ambiguous genitalia and 45,X/46,XY mosaic karyotype. Prophylactic bilateral gonadectomy was performed at age 14 months.

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Risk of Malignancy and Need for Surgery in Pediatric Patients with Morris or Y-chromosome Turner Syndrome: A Multicenter Survey

Cited by 6 publications

References 15 publications

Laparoscopic approach for gonadectomy in pediatric patients with intersex disorders

Laparoscopic approach for gonadectomy in pediatric patients with intersex disorders

Anterior transperitoneal laparoscopic gonadectomy in a patient with complete androgen insensitivity syndrome (CAIS): case report and surgical technique

Prophylactic Bilateral Gonadectomy for Ovotesticular Disorder of Sex Development in a Patient With Mosaic 45,X/46,X,idic(Y)q11.222 Karyotype

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