Hormone Replacement and the Risk of Breast Cancer in Turner's Syndrome

Bösze, P; Tóth, András; Török, Marianna

doi:10.1056/nejmc062795

Cited by 39 publications

(17 citation statements)

References 3 publications

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“…Prometrium (100-200 mg) or medroxyprogesterone, is preferred based on decreased breast cancer risk, but the data for this are relatively weak (164, 165). However, the risk of breast cancer is low in TS (166,167,168), and long-term treatment with HRT does not seem to induce breast cancer (169). Progestin can be added for 10 days each month for withdrawal bleeding, and adult women with TS should preferably continue treatment with 17β-estradiol in combination with a sequential progestational agent.…”

Section: Preparationmentioning

confidence: 99%

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Gravholt¹,

Andersen

Conway

et al. 2017

European Journal of Endocrinology

874

1,259

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Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016. Prior to this meeting, five groups each addressed important areas in TS care: 1) diagnostic and genetic issues, 2) growth and development during childhood and adolescence, 3) congenital and acquired cardiovascular disease, 4) transition and adult care, and 5) other comorbidities and neurocognitive issues. These groups produced proposals for the present guidelines. Additionally, four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society for Endocrinology, and the European Society of Cardiology. The guideline has been formally endorsed by the European Society of Endocrinology, the Pediatric Endocrine Society, the European Society for Paediatric Endocrinology, the European Society of Human Reproduction and Embryology and Summary of recommendationsThe recommendations (R) are worded as recommend (strong recommendation) and suggest (weak recommendation). We formally graded only the evidence underlying recommendations for therapeutic choices. The quality of evidence behind the recommendations is classified as very low (⨁◯◯◯), low (⨁⨁◯◯), moderate (⨁⨁⨁◯) and strong (⨁⨁⨁⨁). See further section 'Summary of methods used for guideline development'. Diagnosis and geneticsR 1.1. We recommend considering a diagnosis of TS in phenotypic females with a karyotype containing one X chromosome and complete or partial absence of the second sex chromosome, associated with one or more typical clinical manifestations of TS (⨁⨁⨁⨁). R 1.2. We recommend against considering a diagnosis of TS in females with one X chromosome and a deletion distal to Xq24 on the other X chromosome, and in women over the age of 50 years with less than 5% 45,X mosaicism (⨁⨁◯◯). R 1.3. We suggest that the new general surveillance management guideline applies to TS patients with any karyotype (⨁⨁◯◯). R 1.4. We recommend to consider testing for Turner syndrome (TS) in a female with typical signs (Table 2) (⨁⨁⨁⨁). R 1.5. We ...

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Section: Preparationmentioning

confidence: 99%

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Gravholt¹,

Andersen

Conway

et al. 2017

European Journal of Endocrinology

874

1,259

View full text Add to dashboard Cite

show abstract

“…In contrast, women with Turner syndrome, caused by lack of one X-chromosome (X0), were found to be at a decreased risk for breast cancer [129]. Interestingly, in a small study it was noted that none of the 62 Turner women who received 20-40 years of continuous HRT developed breast cancer, even though this would have been expected based on breast cancer incidence and increased risk after HRT [130].…”

Section: Tissue Specificity and X Chromosome Dosagementioning

confidence: 99%

Genomic instability in breast and ovarian cancers: translation into clinical predictive biomarkers

Vollebergh

Jonkers

Linn

2011

Cell. Mol. Life Sci.

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Breast and ovarian cancer are among the most common malignancies diagnosed in women worldwide. Together, they account for the majority of cancer-related deaths in women. These cancer types share a number of features, including their association with hereditary cancer syndromes caused by heterozygous germline mutations in BRCA1 or BRCA2. BRCA-associated breast and ovarian cancers are hallmarked by genomic instability and high sensitivity to DNA double-strand break (DSB) inducing agents due to loss of error-free DSB repair via homologous recombination (HR). Recently, poly(ADP-ribose) polymerase inhibitors, a new class of drugs that selectively target HR-deficient tumor cells, have been shown to be highly active in BRCA-associated breast and ovarian cancers. This finding has renewed interest in hallmarks of HR deficiency and the use of other DSB-inducing agents, such as platinum salts or bifunctional alkylators, in breast and ovarian cancer patients. In this review we discuss the similarities between breast and ovarian cancer, the hallmarks of genomic instability in BRCA-mutated and BRCA-like breast and ovarian cancers, and the efforts to search for predictive markers of HR deficiency in order to individualize therapy in breast and ovarian cancer.

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“…It is crucial to the maintenance of tissue and bone integrity and has positive effects on the development of 4 Similarly, they claim that the mean age of WHI subjects was 63 years, while it is unclear whether these findings are applicable to a younger population. 8 In discord to those reports, we present a case of ductal carcinoma in-situ (DCIS) in a 40-year-old Turner patient, who had undergone over two decades of combined hormone replacement therapy and was found to have left breast ductal carcinoma in-situ. While a rare case, it is imperative for the astute clinician to keep in mind the consequences of long-term hormone replacement therapy in Turner's syndrome patients to avoid delay in diagnosis and treatment of breast cancer and for the optimum management of these patients.…”

Section: Introductionmentioning

confidence: 83%

Ductal Carcinoma In-Situ in Turner Syndrome Patient undergoing Hormone Replacement Therapy: A Case Report

Bawa¹,

Matemavi

Maizlin

2016

Int J Cancer Ther Oncol

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Cite this article as: Bawa R, Matemavi P, Maizlin I, Sung KJ. Ductal carcinoma in-situ in Turner syndrome patient undergoing hormone replacement therapy: A case report. Int J Cancer Ther Oncol. 2016; 4(1):4113. Abstract Turner's syndrome is a rare congenital disease which affects about 1 in every 2500-3000 live-born females. This happens due to chromosomal abnormalities in a phenotypic female, causing increased gonadotropin concentrations and low concentrations of estrogens from infancy. As a result, hormone replacement therapy is started in most adolescent Turner syndrome patients to initiate and sustain sexual maturation. Accordingly, most Turner's syndrome patients undergo several decades of estrogen replacement therapy, from puberty to post-menopausal age. The highly publicized findings of the Women's Health Initiative have called into question the appropriateness of hormone replacement therapy in adolescents with Turner's syndrome. Those concerns were mostly theoretical extrapolations, as few prospective studies of cancer occurrence in women with Turner syndrome have been reported. Consequently, several recent publications have challenged those extrapolations, based on the assertion that the levels of hormone replacement in Turner syndrome patients are well below the physiologic levels observed in normal menstruating women, as well as the fact that these women are significantly younger than those studied by the Women's Health Initiative. In discord to those reports, we present a case of ductal carcinoma in-situ in a 40-year-old Turner patient, who had undergone over two decades of combined hormone replacement therapy. The patient underwent an elective excisional biopsy for a palpable mass, with histopathology revealing a complex fibroadenoma with a nidus of ductal carcinoma in-situ. The lesion was noted to be estrogen receptor positive and progesterone receptor negative, with heavy staining for HER-2/Neu receptor. The patient was treated with tamoxifen. While a rare case, it is imperative for the astute clinician to keep in mind the consequences of long-term hormone replacement therapy in Turner's syndrome patients in order to avoid missed diagnosis of breast cancer for optimum management of these patients.

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Hormone Replacement and the Risk of Breast Cancer in Turner's Syndrome

Cited by 39 publications

References 3 publications

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Genomic instability in breast and ovarian cancers: translation into clinical predictive biomarkers

Ductal Carcinoma In-Situ in Turner Syndrome Patient undergoing Hormone Replacement Therapy: A Case Report

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