The Breast (2006) 15(S2) S61–S64
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Lymphoedema in young patients with breast cancer Santilal Parbhooa,b, * a b
Lymphoedema Clinic, Cancerkin Centre, Royal Free Hospital, Pond Street, London NW3 2QG, Breast Unit, Hospital of St John and St Elizabeth, 60 Grove End Road, London NW8 9NH, UK
KEYWORDS Breast cancer; Lymphoedema; Young women; Management
Summary Gross lymphoedema is a devastating complication in patients with breast cancer and more so in young women. These patients present with more advanced disease and suffer a higher incidence of loco-regional recurrence. Young women are also subject to more traumas to the lymphatics by virtue of their being more active. Treatment contributes to a higher incidence of lymphoedema in these patients. Thrombosis and infection may complicate intravenous chemotherapy, which damages lymphatic drainage. Modern radiotherapy delivers precise dosage and site targeting of treatment so that lymphoedema is less common. However, there is a tendency to return to aggressive treatment of the nodal areas. Early detection of breast cancer and sentinel lymph node biopsy will further decrease axillary dissection. Lymphoedema treatment consists mainly of compression therapy, manual lymphatic drainage and containment. Surgery is rarely required. Lymphoedema is a chronic condition that requires long-term care with patient compliance and active cooperation. © 2006 Elsevier Ltd. All rights reserved.
Introduction Gross lymphoedema is a devastating complication in patients with breast cancer and more so in young women. Shapiro and Recht 1 , in a major NCI review of the late effects of adjuvant therapy for breast cancer in young women, cited 142 references, but did not mention lymphoedema, which is a distressing complication of treatment in these patients. Lymphoedema in breast cancer patients remains a Cinderella problem in most countries. In a cohort of 23,974 patients with breast cancer in the USA reported by Osteen et al. 2 , 7.4% were aged 40 or under. In a personal database of 2344 patients 4.14% were aged 35 years or under and 11.48% were aged 40 or under (Parbhoo 2003; unpublished data). This skewed population group may be due to our unit’s interest in young patients with breast cancer and our screening of young women with a high familial * Tel: +44 207 8302323; fax: +44 207 8302324. E-mail:
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risk of breast cancer. This paper details our experience of lymphoedema in patients aged 40 years or under in a cohort of 647 patients with breast cancer-related lymphoedema of the upper limb.
Incidence In a survey of 647 patients with upper limb lymphoedema we found 55 patients aged 26–40 years (8.5%) with a mean age of 36.2 years (Parbhoo 2005; unpublished data). In a survey of 2344 patients we found 269 women aged 20–40 years (11.47%) and 41 (15.2%) were recorded as having lymphoedema of the upper limbs. In some patients the lymphoedema was transient during the early post-operative phase.
Predisposing factors in the development of lymphoedema These young patients present with more advanced disease. Many factors contribute to a later
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S62 diagnosis in young women including pregnancy and lactation, which may mask some of the symptoms and signs of cancer. Some 70% of breast cancers in association with pregnancy and lactation are in the 30s or under 30 age group 3 . The biological behaviour (higher grade, faster proliferation rate, more lympho-vascular invasion and nodal involvement, lower incidence of endocrine-responsive disease) of the cancer is much more aggressive than in older women 4-7 . Young patients have a higher local, regional and distant relapse rate 8-10 . There is a higher incidence of inflammatory breast carcinoma in young women accompanied by more lymphovascular invasion, nodal involvement, and higher incidence of loco-regional recurrence. Cyclical increase in vascularity may also permit more vascular and lymphatic spread. Young women are also subject to more traumas to the lymphatic drainage by virtue of their being more active – domestic work, childcare, shopping, travel, sports and accidents. These activities lead to a higher incidence of injuries to the upper limb in the form of burns, bites, bruises, muscle tears and fractures, many of which precipitate or aggravate the swelling. Treatment itself contributes to a higher incidence of lymphoedema in these patients. Patients require multiple courses of intravenous chemotherapy, which use up peripheral vascular access. Central lines such as Hickman catheters or reservoirs have problems with thrombosis and infection, which further affect lymphatic drainage of the limb and breast. We have seen some unusual distributions of lymphoedema of the upper limb and trunk as a result of catheterrelated obstruction because of impingement of the catheter against one of the lesser trunks draining into the junction of the jugular and subclavian veins. As more breast conservation surgery is carried out as a result of earlier diagnosis, more breast oedema is seen. Similarly, younger patients undergo five-fold more breast reconstructions. The type of reconstruction may increase lymphoedema in some patients. Newer breast-shaping procedures during breast-conserving surgery also increase the incidence of lymphoedema in the first few months of surgery, but may persist in a small minority of patients. A higher incidence of breast oedema is also seen as a result of the primary cancer in young women. Whereas the incidence of breast cancer in young women was ~11% the incidence of inflammatory breast cancer was ~16% (Parbhoo 2005; unpublished data). Another cause of breast oedema is recurrent breast cancer.
S. Parbhoo The early days of radical mastectomy were followed by a period of extensive and radical radiotherapy. Some of these young patients have survived and show the ravages of treatment in the form of gross lymphoedema, brachial plexus palsy and, rarely, angiosarcoma. Modern radiotherapy delivers a much more precise dosage and site targeting of treatment so that lymphoedema is less common. There is much less in the way of overlap fibrosis, which is a fibrous band of tissue in the infraclavicular area and predisposes to both upper limb and breast oedema. However, there is a tendency to return to much more aggressive and extensive treatment involving the supra- and infraclavicular nodal areas, which will affect lymphatic drainage from the limb. Radiotherapists need to be aware of the lymphatic anatomy and use radiotherapy more judiciously with fully informed patient consent. Since survival from breast cancer is improving, local and regional treatment should not cause major depreciation in the quality of life of patients. Most long-term survival depends on the control of distant micrometastases. All trials of surgery, radiotherapy and chemotherapy should include lymphoedema as an outcome measure.
Prevention Early detection of breast cancer results in smaller cancers with little or no nodal involvement. Hence, these patients require less lymph node surgery and no radiotherapy to the nodal areas. The use of sentinel lymph node biopsy will further decrease the need for full axillary dissection. Early diagnosis depends on screening young women at high risk and following up patients who have had breast cancer. Obese patients should be advised to lose weight, as the relationship between pre-operative weight and development of lymphoedema has been demonstrated. Several studies have indicated that obesity contributes to the development of lymphoedema after breast cancer treatment 11-15 . In their review of the records of 1551 patients treated for breast cancer, Say and Donegan 11 reported that those over 90 kg in body weight had a significantly higher incidence of upper limb oedema than those who weighed less than 90 kg (36.6% versus 16.9%). Werner et al. 12 , in a study of 282 patients who had undergone surgery and radiotherapy for breast cancer, showed a direct relationship between obesity and the development of upper limb oedema. They found that obese women had double the risk of developing lymphoedema than non-obese patients
Lymphoedema in young patients with breast cancer (27.4% versus 12.5%). They concluded that a high BMI was the most powerful predictor for the development of lymphoedema after breast cancer management. Johansson et al. 15 reported on a series of 142 patients including controls that those with a higher BMI at the time of surgery had a higher incidence of developing lymphoedema of the upper limbs. The mechanisms and relationship of obesity to the development and persistence of lymphoedema are complex 16 . Patients should be advised about triggering and precipitating factors such as local trauma and infections. When infections develop these should be treated aggressively to minimise further lymphatic destruction. All patients at risk should be provided with a supply of antibiotics for emergency home treatment.
Treatment Treatment for lymphoedema has improved enormously over the last 20 years, but gold standard treatment is only available in a few centres in the UK. It is essential that patients undergo medical and therapeutic assessment prior to treatment of their lymphoedema. About 10% of the patients seen in our clinic have evidence of active or recurrent cancer, which has not been recognised or treated. In addition, we need to establish whether any removable obstruction to the lymphatic pathway is present. Reduction of the lymphoedema, called complex decongestive therapy (CDT), consists mainly of compression therapy, manual lymphatic drainage and containment 17 . Patients must play a part in treatment such as weight reduction, care of skin and exercises. Exercises including swimming with containment garments in situ are an important part of home treatment. Patients or their partners can also be taught self massage as an aid to reduction of oedema at home. Surgery is rarely required, but can produce dramatic results in selected patients 18 . Direct lymphatic or lymphatico-venous anastomoses can be carried out in specialised centres after careful selection of patients. Good results have been obtained with microsurgery provided extensive fibrosis is absent at the time of surgery. Vascular flaps such as the latissimus dorsi flap can be used to cover fibrotic post-radiotherapy chest wall and overcome limb lymphoedema.
Comment The multidisciplinary team looking after young patients with breast cancer needs to focus
S63 on the complication of lymphoedema, which can be devastating for the young mother or worker. Earlier diagnosis, sentinel lymph node biopsy, appropriate surgery, radiotherapy and chemotherapy will decrease the incidence of lymphoedema. However, more breast oedema is inevitable as more breast conservation, shaping and reconstruction are done. Lymphoedema is a chronic condition that requires long-term care with patient compliance and active co-operation to ensure treatment success. Fortunately, the vast majority of patients have sufficient lymphatic transport reserve to overcome loss of some lymphatics and lymph nodes and do not develop lymphoedema. The majority of patients with lymphoedema can be helped by simple measures.
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