(still) recruiting: Paclitaxel in Treating Patients With Ovarian Stromal Cancer - Full Text View - ClinicalTrials.gov (granulosa/sertoli-leydig, sex cord...)

Criteria

DISEASE CHARACTERISTICS:

• Histologically confirmed ovarian stromal cancer not amenable to surgery
  • Granulosa cell tumor
  • Granulosa cell theca cell tumor
  • Sertoli-Leydig cell tumor (androblastoma)
  • Gynandroblastoma
  • Unclassified sex cord stromal tumor
  • Sex cord tumor with annular tubules
  • Steroid (lipid) cell tumor
• Recurrent disease after no more than 1 prior chemotherapy regimen
• Measurable disease
  • At least 1 cm in diameter

abstract: DICER1 in ovarian cancers : Nature Genetics (sertoli-leydig/juvenile granulosa/sex-cord stromal tumors) : Nature Publishing Group

Abstract:

"Germline mutations in DICER1 underlie a rare syndrome associated with susceptibility to pleuropulmonary blastoma, cystic nephroma and ovarian sex-cord stromal tumors. David Huntsman, Gregg Morin and colleagues (N. Engl. J. Med., published online 21 December 2011; doi:10.1056/NEJMoa1102903) now show that somatic mutations in DICER1 occur at high frequency in non–epithelial ovarian cancers. The authors performed transcriptome and exome sequencing in two Sertoli-Leydig cell tumors, four juvenile granulosa cell tumors and eight primitive germ cell tumors and identified four missense mutations in DICER1 affecting the metal-binding region of the RNase IIIb domain. They subsequently sequenced the portion of DICER1 encoding this region in 101 additional non-epithelial ovarian tumors and identified somatic mutations in 60% of Sertoli-Leydig cell tumors........ On the basis of these findings, the authors propose that these mutations result in an oncogenic miRNA profile whose pathogenicity may be restricted to specific cell types or developmental settings, thereby accounting for the high prevalence of these mutations in particular tumor types."

abstract: DICER1 Mutations in Familial Multinodular Goiter (thyroid) With and Without Ovarian Sertoli-Leydig Cell Tumors

define: 

multinodular goiter - A multinodular goiter is  a thyroid gland that is usually enlarged and contains multiple thyroid nodules.

Pleuropulmonary blastoma (PPB) is a rare cancer originating in the lung or pleural cavity....
en.wikipedia.org/wiki/Pleuropulmonary_blastoma

 

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Objective To determine whether familial MNG  (multinodular goiter) with or without SLCT (Sertoli-Leydig cell tumor of the ovary) in the absence of PPB (pleuropulmonary blastoma)  was associated with mutations in DICER1. 

 

Conclusions DICER1 mutations are associated with both familial MNG and MNG with SLCT, independent of PPB. These germline DICER1 mutations are associated with dysregulation of miRNA expression patterns.

Sex cord stromal tumors of the ovary in children - abstract (sertoli-leydig most aggressive cell type)

CONCLUSIONS: Completeness of resection and histology were important prognostic factors; in our series the Sertoli-Leydig Cell tumor was the most aggressive variety. Hormonal signs (precocious puberty, telarca, menarche) were common in younger patients and led to an early diagnosis. Cisplatin based chemotherapy seemed to be effective for locally advanced tumors.

abstract: Clinical syndromes associated with ovarian neoplasms: a comprehensive review

Radiographics. 2010 Jul-Aug;30(4):903-19.

Functional ovarian neoplasms have unique clinical manifestations related to hormone overproduction and may give rise to a broad spectrum of clinical syndromes.

Sex cord-stromal tumors, the most common functional ovarian neoplasms, are associated with either hyperestrogenism (as in granulosa cell tumor and thecoma) or hyperandrogenism (as in Sertoli-Leydig cell tumor and Leydig cell tumor). Other, less common ovarian neoplasms that may have endocrine or nonendocrine syndromic manifestations include germ cell tumors associated with the excessive production of human chorionic gonadotropin (eg, choriocarcinoma, dysgerminoma), monodermal teratomas (eg, carcinoid tumor, struma ovarii) associated with carcinoid syndrome and hyperthyroidism, and primary epithelial ovarian cancers associated with paraneoplastic syndromes.

The application of diagnostic algorithms based on patient demographic information, clinical manifestations, laboratory findings, and cross-sectional imaging features may help identify ovarian neoplasms in complex clinical settings.