Androblastomas and Thyroid Disease in Postmenopausal Sisters

Rw, Whitcomb; Jw, Calkins; Bp, Lukert; Jl, Kyner; Rn, Schimke

doi:10.1097/00006250-198603001-00026

Cited by 9 publications

(3 citation statements)

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“…Familial SCLT has also been reported (Table 2) Whether familial SLCT results from genetic predisposition is not answered by our study as the families described here have only one case of SLCT per kindred. We did screen an affected familial SLCT proband 34 but did not identify a DICER1 mutation; it remains possible that DICER1 mutations will be implicated in other familial SLCT, or that there are other genes responsible for familial SLCT with genes in the miRNA processing pathway, such as PASHA/DGCR8 or DROSHA , being reasonable candidates.…”

Section: Commentmentioning

confidence: 99%

<emph type="ital">DICER1</emph> Mutations in Familial Multinodular Goiter With and Without Ovarian Sertoli-Leydig Cell Tumors

Frio

Bahubeshi

Kanellopoulou

et al. 2011

JAMA

300

150

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Section: Commentmentioning

confidence: 99%

<emph type="ital">DICER1</emph> Mutations in Familial Multinodular Goiter With and Without Ovarian Sertoli-Leydig Cell Tumors

Frio

Bahubeshi

Kanellopoulou

et al. 2011

JAMA

300

150

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“…14 SLCTs are consistently associated with mutations of the DICER1 gene. 3,15,16 A report from the International Ovarian and Testicular (OTST) Registry found germline DICER1 mutations in approximately half of patients with SLCTs. 4 In this group, a spectrum of other cancers has been reported, of which thyroid cancer was seen in four of 25 patients.…”

Section: Ovarian Sex Cord Stromal Tumors (Oscsts)mentioning

confidence: 99%

“…SLCTs are consistently associated with mutations of the DICER1 gene 3,15,16 . A report from the International Ovarian and Testicular (OTST) Registry found germline DICER1 mutations in approximately half of patients with SLCTs 4 .…”

Section: Ovarian Sex Cord Stromal Tumors (Oscsts)mentioning

confidence: 99%

Consensus recommendations from the EXPeRT/PARTNER groups for the diagnosis and therapy of sex cord stromal tumors in children and adolescents

Schneider

Orbach

Ben-Ami

et al. 2021

Pediatric Blood & Cancer

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As part of the European Union‐funded project designated Paediatric Rare Tumours Network ‐ European Registry (PARTNER), the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) is continuously developing consensus recommendations in order to harmonize standard care for very rare solid tumors of children and adolescents. This paper presents the internationally recognized recommendations for the diagnosis and treatment of sex cord stromal tumors (SCST). The clinical approach to sex cord stromal tumors of the testis (TSCST) and ovary (OSCST) depends on histological differentiation and tumor stage. Virtually all TSCSTs present as localized nonmetastatic tumors, with excellent prognosis after complete resection. In contrast, the prognosis of OSCSTs may be adversely affected by tumor spillage during surgery or presence of metastases. In these cases, cisplatin‐based chemotherapy is recommended. Of note, some SCSTs may develop in the context of tumor predisposition syndromes, for example, DICER‐1, so that specific follow‐up is indicated. SCSTs should be diagnosed and treated according to standardized recommendations that include reference pathology, genetic testing for tumor predisposition syndromes in selected cases, and stratified adjuvant chemotherapy in patients with unfavorable risk profile. To ensure high quality of diagnosis and therapy, patients should be enrolled into prospective registries.

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