Ovarian Sertoli-Leydig Cell Tumor with Elevated Inhibin B as a Cause of Secondary Amenorrhea in an Adolescent with Germ Line DICER1 Mutation

Luke, Amy M.; Moroney, John W.; Snitchler, Andrea; Whiteway, Susan L.

doi:10.1016/j.jpag.2017.05.002

Cited by 8 publications

(9 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our review of the literature revealed 29 previously published cases of bilateral ovarian SLCT (Tables 1 and 2) 14,19–42 ; two of these are included in this study (cases 2 and 3 in Table 1). Where possible, the articles and the photomicrographs were reviewed to try to determine the type of SLCT; this was sometimes difficult, given a paucity of pathological description and photomicrographs.…”

Section: Resultsmentioning

confidence: 99%

Somatic tumour testing establishes that bilateral DICER1‐associated ovarian Sertoli–Leydig cell tumours represent independent primary neoplasms

et al. 2020

View full text Add to dashboard Cite

Aims Sertoli–Leydig cell tumours (SLCTs) are rare ovarian neoplasms that are commonly associated with somatic or germline DICER1 mutations, especially when of the moderately or poorly differentiated type. A large majority are unilateral, but bilateral neoplasms have been reported, sometimes in the context of germline DICER1 mutations (DICER1 syndrome). It is currently unknown whether these represent independent neoplasms or metastasis from one ovary to the other and we aimed to elucidate this. Methods and results We report three cases of bilateral ovarian SLCT (all in patients with DICER1 syndrome) and review all reported cases of bilateral neoplasms. In the three cases (all moderately or poorly differentiated neoplasms), the time interval between the discovery of the tumours in each ovary ranged from 2.7 years to 6 years. In all cases, different DICER1 somatic hotspot mutations within the two tumours provided definitive proof that they represent independent neoplasms; this may be important clinically. Our literature review revealed that, when this information was available, all patients with bilateral SLCT had a germline DICER1 mutation. Conclusions Bilateral ovarian SLCTs represent independent rather than metastatic neoplasms, and essentially always occur in the context of DICER1 syndrome.

show abstract

Section: Resultsmentioning

confidence: 99%

Somatic tumour testing establishes that bilateral DICER1‐associated ovarian Sertoli–Leydig cell tumours represent independent primary neoplasms

et al. 2020

View full text Add to dashboard Cite

show abstract

“…3 Patients showing clinical signs of hyperandrogenism accompany increased level of androgenic hormones such as testosterone, rendering an early diagnosis and proper intervention with good prognosis possible. 4 Obesity, androgen producing adrenal tumors, congenital adrenal hyperplasia, and Cushing's syndrome are some of the disease entities that might also result in female hyperandrogenism. 5 Although virilization is the key feature of suspecting the presence of SLCTs, other symptoms such as hyperestrogenism or abdominal pain and distention due to the sheer size of the mass also occur.…”

Section: Discussionmentioning

confidence: 99%

“…3 Typical cases of SLCTs with clinical signs of hyperandrogenism accompanied by the increased level of androgenic hormones such as testosterone should not be missed, rendering an early diagnosis and proper intervention with good prognosis possible. 4 Asymptomatic Sertoli-Leydig cell tumors warrant special attention because without the clinical signs of abrupt onset of virilization it is rather difficult to be suspicious of the tumor due to the lack of accuracy in detecting it through radiologic studies. Authors hereby present a case of Sertoli-Leydig cell tumor without the clinical signs of hyperandrogenism that was incidentally found in a hysterectomy with bilateral salpingooophorectomy specimen, to emphasize the importance of the preventive bilateral salpingo-oophorectomy in postmenopausal women even with unremarkable intraoperative findings of the adnexa.…”

Section: Introductionmentioning

confidence: 99%

Incidental finding of a Sertoli-Leydig cell tumor in a postmenopausal woman with complex endometrial hyperplasia

Kim

Lee

Chung

2018

Int J Reprod Contracept Obstet Gynecol

View full text Add to dashboard Cite

Sertoli-Leydig cell tumors (SLCTs) arise from the non-germ cell component of the ovary and typically present in young women with evidence of hyperandrogenism such as precocious puberty, amenorrhea, hirsutism and virilization. It is very rare accounting for less than 0.2% of all ovarian tumors, and because of the rarity, no standardized treatment approach has reached a consensus. The prognosis is generally good with complete reversion of symptoms after surgery, although some cases have been reported to be malignant. Recently the need for DICER1 mutations testing in paediatric patients has been emphasized for the surveillance of possible synchronous tumors and affected family members. Authors present here a case of Sertoli-Leydig cell tumor incidentally found while performing a hysterectomy with bilateral salpingo-oophorectomy in a postmenopausal woman with endometrial hyperplasia that caused intractable vaginal bleeding.

show abstract

“…Tumores dos cordões sexuais são um grupo de neoplasias raras e clinicamente heterogêneas com diversos tipos patológicos. Diferentemente dos outros tumores ovarianos que aparecem entre a sexta e a sétima décadas de vida, os tumores ovarianos dos cordões sexuais frequentemente aparecem em adolescentes e adultos jovens (LUKE et al, 2017). Os 2 tipos mais frequentes são os tumores de células da granulosa e o tumor de células de Sertoli-Leydig, representando 85% dos tumores de cordões sexuais em crianças e adolescentes (SCHULTZ et al, 2016).…”

Section: Tumor Ovariano Dos Cordões Sexuaisunclassified

Amenorreia secundária: revisão das etiologias / Secondary amenorrhea: etiologies review

Valadão¹,

Tostes²,

Menezes³

et al. 2022

BJDV

View full text Add to dashboard Cite

Introdução: amenorreia consiste na ausência de menstruação, sendo indicativo de uma disfunção no eixo hipotalâmico-hipofisário-ovariano-uterino em mulheres na menacme, não gestantes/lactantes e que não estejam em uso de medicamento hormonal. Classifica-se em primária ou secundária de acordo com a presença ou não da menarca e com o desenvolvimento de caracteres sexuais secundários. Objetivo: coletar informações da literatura sobre as etiologias da amenorreia secundária, sua propedêutica e terapêutica. Método: trata-se de uma revisão bibliográfica, em que foram selecionados livros e artigos da literatura mundial, obtidos nas bases de dados Scielo, Pubmed, e UpToDate, publicados entre 2015-2021, com Qualis superior a B3 ou fator de impacto superior a 1,5. Foram excluídos revisões de literatura do tema tratado nesse artigo. Desenvolvimento: amenorreia secundária é um sintoma prevalente na população e possui vários diagnósticos etiológicos. Podendo ser derivados de: disfunções no complexo controle hormonal do ciclo menstrual ou alterações anatômicas. Dada a complexidade, frequentemente, necessita de investigação elaborada. A determinação da etiologia é importante porque permite estipular o plano terapêutico apropriado. Conclusão: a investigação e tratamento da amenorreia pode demandar uma equipe multiprofissional envolvida. É preciso educar as pacientes a respeito da sua condição e possíveis prejuízos a saúde em longo prazo. Mesmo que trabalhoso é importante tratar a causa base da amenorreia ainda que não seja uma queixa importante para a paciente.

show abstract

Ovarian Sertoli-Leydig Cell Tumor with Elevated Inhibin B as a Cause of Secondary Amenorrhea in an Adolescent with Germ Line DICER1 Mutation

Cited by 8 publications

References 6 publications

Somatic tumour testing establishes that bilateral DICER1‐associated ovarian Sertoli–Leydig cell tumours represent independent primary neoplasms

Somatic tumour testing establishes that bilateral DICER1‐associated ovarian Sertoli–Leydig cell tumours represent independent primary neoplasms

Incidental finding of a Sertoli-Leydig cell tumor in a postmenopausal woman with complex endometrial hyperplasia

Amenorreia secundária: revisão das etiologias / Secondary amenorrhea: etiologies review

Contact Info

Product

Resources

About