Breast cancer in adolescents and young women
Introduction
Breast cancer in adolescence and early adulthood is a rare condition. The estimated incidence is less than 0.1 per 100 000 women below the age of 20 years, increasing to 1.4 for women 20–24 years, 8.1 for women 25–29 years and 24.8 for women 30–34 years old [1]. Breast cancer in childhood accounts for less than 1% of childhood cancers and less than 0.1% of all breast cancers 2, 3, 4. In the United States, figures from the National Cancer Institute (NCI) Surveillance, Epidemiology and End-Results (SEER) database show that less than 1% of breast cancer patients are younger than 30 years and 2.7% are younger than 35 years [1].
The most common type of breast cancer in childhood is secretory carcinoma, formerly known as juvenile carcinoma, because of its tendency to occur more frequently (although not exclusively) in children 6, 7. This is a morphologically-distinct type of breast carcinoma with a very indolent clinical behaviour. Wide local excision and axillary lymph node dissection are generally all that is required for therapy, as distant metastases are extremely rare. The prognosis is generally favourable, but patients require long-term follow-up due to the risk of late recurrence.
Invasive ductal carcinoma occurring in adolescents and young women has a more aggressive biological behaviour and a worse prognosis than breast cancer arising in older premenopausal women [8]. In previously published studies, tumours in younger women were less well differentiated, had a higher proliferating fraction and had more lymphovascular invasion than those occurring in older patients 9, 10, 11, 12, 13. Review of large databases worldwide shows that women younger than 35 years of age have more advanced disease at diagnosis and a poorer 5- year survival than older premenopausal patients 5, 10, 14, 15, 16. In several series, age remained independently prognostic when pathological variables were taken into account 13, 17, 18, 19. Consensus statements from both the National Institute of Health (NIH) and the St Gallen consensus conference have recommended that all women under the age of 35 years receive adjuvant therapy based on the evidence that these patients have biologically more aggressive disease and a poorer survival 20, 21.
In addition to these considerations, there are a number of special issues facing these very young women presenting with a diagnosis of breast cancer, including the risk of treatment-induced fertility impairment and premature menopause, and an increased risk of locoregional recurrence with breast-conserving surgery than older premenopausal patients 22, 23, 24, 25, 26, 27. Little is known about the long-term cardiac toxicity of radiation in addition to anthracyclines and taxanes. Moreover, there is evidence that young women are more vulnerable to emotional distress and have a higher risk of psychosocial problems 28, 29, 30, 31, 32. Optimal management of very young women and adolescents requires attention not only to the best treatments for such an inherently biologically aggressive disease, but also consideration of the long-term physical and psychological consequences of such treatments.
Section snippets
Diagnosis
Breast cancers in this age group tend to be larger when diagnosed and have a longer history of a palpable mass than tumours diagnosed in older women [33]. The accuracy of physical examination is lower in very young women, as they often have dense or nodular breast tissue that is subject to cyclical hormonal changes. Most discrete breast masses in this age group are fibroadenomas. In a study of 30 women under the age of 30 years with breast cancer, the clinical examination was correctly deemed
Prognosis of breast cancer in very young women
A number of studies have compared the stage and pathological characteristics of breast tumours occurring in very young women with those occurring in older premenopausal women. Colleoni and colleagues [37] looked at 1837 premenopausal women treated at the European Institute of Oncology between April 1997 and August 2000. Of these, 185 were aged less than 35 years at the time of diagnosis. When compared to older premenopausal women, young women were more likely to have tumours that were oestrogen
Management
The principles of managing invasive breast carcinoma in very young women are the same as that for older women, but there are a number of special issues which require consideration. Consensus panels of the NIH and the St Gallen conference have recommended adjuvant therapy for all patients aged under 35 years based on the evidence that they have a poorer prognosis 20, 21. The use of adjuvant therapies in young women raises issues of the long-term side-effects such as the induction of an early
Psychosocial issues
A diagnosis of breast cancer is obviously a stressful life-event for a woman at any age, but younger women are likely to face unique concerns and studies have shown them to be particularly vulnerable [90]. Young women more frequently have concerns about the impact of the diagnosis on their partner and may have practical issues related to the care of young children during their treatment. Research suggests that peer support and self-help groups decrease feelings of social isolation, depression
Management of young women that are potentially at a high risk
A further consideration is the management of young women who are at an increased risk of developing breast cancer at a young age. These include women who have germline mutations in BRCA1, BRCA2, TP53 (Li–Fraumeni syndrome) or PTEN (Cowden's syndrome). Factors which define women at potentially high risk of developing breast cancer are summarised in Table 3 [95]. For women with a mutated BRCA1 gene, clinical disease may develop in approximately 50% by age 50 years and 80% by age 70 years. The
Conclusions
Breast cancer is a rare condition in adolescents and young adults. Invasive breast cancer occurring at a young age has more aggressive biological behaviour and is associated with a worse prognosis. Even when traditional prognostic factors of size and nodal status are controlled for, young women appear to have worse DFS and OS than older premenopausal women. Women developing breast cancer prior to the age of 35 years should be offered adjuvant therapy based on the poor prognosis in this age
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