[Preventive surgical prcedures for inherited risk of breast cancer].

Preventive surgical procedures for inherited risk of breast cancer Forwomen with a demonstrated BRCA1 or BRCA2 mutation, the cumulative risk of developing invasive breast cancer before the age of 70 years is about 50-85% and the risk of developing invasive epithelial ovarian cancer is 20-60%. Regular surveillance including physical examination and imaging is offered to mutation carriers and the options for risk-reducing surgery are discussed. Although bilateral prophylactic mastectomy is a drastic intervention, it significantly reduces the incidence of breast cancer.

Survival and prognostic factors in BRCA1-associated breast cancer.

Background: Studies comparing survival in BRCA1-associated and sporadic breast cancer (BC) report inconsistent results and frequently concern small sample sizes. Further, the prognostic impact of the classical tumour and treatment factors is unclear in BRCA1-associated BC.

Patients And Methods: We selected 223 BC patients diagnosed between 1980 and 2001 within families with a deleterious germline BRCA1-mutation ascertained at the Rotterdam Family Cancer Clinic.

Using single-agent therapy in adult patients with advanced soft tissue sarcoma can still be considered standard care.

The group of soft tissue sarcomas in adult patients is a heterogeneous group with more than 40 different subtypes. While local treatment remains the mainstay for localized disease, systemic chemotherapy can importantly contribute in the treatment of advanced soft tissue sarcoma. For patients with metastatic disease, chemotherapy is a palliative treatment in the vast majority of the cases.

Fatigue and relating factors in high-risk breast cancer patients treated with adjuvant standard or high-dose chemotherapy: a longitudinal study.

Purpose: Determine whether standard or high-dose chemotherapy leads to changes in fatigue, hemoglobin (Hb), mental health, muscle and joint pain, and menopausal status from pre- to post-treatment and to evaluate whether fatigue is associated with these factors in disease-free breast cancer patients.

Patients And Methods: Eight hundred eighty-five patients were randomly assigned between two chemotherapy regimens both followed by radiotherapy and tamoxifen. Fatigue was assessed using vitality scale (score < or = 46 defined as fatigue), poor mental health using mental health scale (score < or = 56 defined as poor mental health) both of Short-Form 36, muscle and joint pain with Rotterdam Symptom Checklist, and Hb levels were assessed before and 1, 2, and 3 years after chemotherapy.

Phase II to III study comparing doxorubicin and docetaxel with fluorouracil, doxorubicin, and cyclophosphamide as first-line chemotherapy in patients with metastatic breast cancer: results of a Dutch Community Setting Trial for the Clinical Trial Group of the Comprehensive Cancer Centre.

Purpose: To compare the efficacy and safety of doxorubicin and docetaxel (AT) with fluorouracil, doxorubicin, and cyclophosphamide (FAC) as first-line chemotherapy for metastatic breast cancer (MBC).

Patients And Methods: Patients (n = 216) were randomly assigned to either AT (doxorubicin 50 mg/m(2) and docetaxel 75 mg/m2) or FAC (fluorouracil 500 mg/m2, doxorubicin 50 mg/m2, and cyclophosphamide 500 mg/m2); both regimens were administered on day 1, every 3 weeks.

Results: A median number of six cycles was delivered in both arms, with a median relative dose-intensity of more than 98%.

Skeletal retention of bisphosphonate (pamidronate) and its relation to the rate of bone resorption in patients with breast cancer and bone metastases.

Unlabelled: Bisphosphonate pharmacokinetics may affect individual responses. Skeletal retention of pamidronate infused monthly to patients with bone metastases was highly variable (12-98%) and did not diminish with time, showing the capacity of the skeleton to retain large amounts of bisphosphonate. Relationships between skeletal retention of pamidronate and rate of bone resorption are complex and depend on previous treatment and the total amount of retained bisphosphonate.

Primitive neuroectodermal tumor of the cervix uteri: a case report -- changing concepts in therapy.

Background: Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Between 1987 and 2002, there have been eight cases described in the English literature. The treatment policies in these eight cases differed considerably, partly due to the rarity of the disease and to differing time periods of diagnosis and treatment.

Clinical experience with venlafaxine in the treatment of hot flushes in women with a history of breast cancer.

Objective: To obtain practical experience with venlafaxine for hot flushes in breast cancer patients and incorporate this in a treatment protocol.

Method: Twenty-two women with a history of breast cancer (mean age 49.2 years, range 35-65) were referred for consideration of treatment with venlafaxine for hot flushes.

Exploring the course of psychological distress around two successive control visits in women at hereditary risk of breast cancer.

In this article we determined the course of psychological distress during a breast cancer surveillance program in women at increased risk of developing hereditary breast cancer (BC). The sample comprised of 357 unaffected women (mean age 40.5 years) adhering to a surveillance programme (MRISC-study).

[Practice guideline 'Diagnostic techniques for soft tissue tumours and treatment of soft tissue sarcomas (revision)'].

Members of the Dutch working group on soft tissue tumours developed an up-to-standard evidence-based multidisciplinary clinical practice guideline for the diagnosis of soft tissue tumours and the treatment and follow-up of soft tissue sarcomas, in cooperation with the Dutch Association of Comprehensive Cancer Centres and the Dutch Institute for Healthcare Improvement. A soft tissue sarcoma is defined as every non-epithelial tumour that does not originate in haematopoietic or lymphatic system, central nervous system or bone. The guideline lists 'alarm signals' to raise awareness of malignancy and recommends consulting a multidisciplinary team.

Outcome of surveillance and prophylactic salpingo-oophorectomy in asymptomatic women at high risk for ovarian cancer.

Objective: Women at high risk of ovarian cancer are currently offered two options: either surveillance or prophylactic bilateral salpingo-oophorectomy. The efficacy and outcome of surveillance remain unclear.

Methods: We performed a retrospective study.

The impact of having relatives affected with breast cancer on psychological distress in women at increased risk for hereditary breast cancer.

Purpose: Being at hereditary risk of breast cancer (BC) may lead to elevated levels of distress because of the impact of the BC-process in relatives.

Objective: Determine the association between psychological distress and BC in relatives. We studied: kind of kinship with the affected relative(s), degree of involvement with the relative's BC, time elapsed since the BC diagnosis of the relative, and loss of a relative as a consequence of BC.

Tumour characteristics and prognosis of breast cancer patients carrying the germline CHEK2*1100delC variant.

Background: The germline CHEK2*1100delC variant has been associated with breast cancer in multiple case families where involvement of BRCA1 and BRCA2 has been excluded.

Methods: We have investigated the tumour characteristics and prognosis of carriers of this germline variant by means of a prospective cohort study in an unselected cohort of 1084 consecutive patients with primary breast cancer. Data were collected for 34 patients with a germline CHEK2*1100delC mutation and for 102 patients without this mutation, stratified by age and date of diagnosis of the first primary breast cancer (within 1 year).

Psychological distress in women at increased risk for breast cancer: the role of risk perception.

The magnetic resonance imaging screening (MRISC) study evaluates a surveillance programme for women with a hereditary risk for breast cancer. The psychological burden of surveillance in these women may depend on inaccurate risk perceptions. To examine differences in risk perceptions between three predefined risk categories and associations with psychological distress.

Psychological distress and breast self-examination frequency in women at increased risk for hereditary or familial breast cancer.

Background: The Magnetic Resonance Imaging Screening study evaluates the efficacy and psychological impact of a surveillance program for women at increased risk for hereditary or familial breast cancer in the Netherlands. Surveillance consists of biannual physical examination, annual mammography, annual MRI and monthly breast self-examination (BSE).

Objective: To examine the association between psychological distress and reported BSE frequency.

High-density oligonucleotide array analysis reveals extensive differences between freshly isolated blood and hepatic natural killer cells.

Hepatic NK cells are more cytotoxic than blood NK cells against tumor cells. To understand the basis of this difference in cytotoxicity we analyzed RNA derived from freshly isolated rat blood and hepatic NK cells [high-density (HD) and low-density subpopulations] by high-density oligonucleotide arrays (Affymetrix), containing about 9,000 genes and expressed sequence tags. IL-2-treated blood NK (A-NK) cells and IL-2-treated hepatic HD cells were used as a reference of NK cell activation.

Impact of screening for breast cancer in high-risk women on health-related quality of life.

The effectiveness of intensive surveillance in women at high risk for breast cancer due to a familial or genetic predisposition is uncertain and is currently being evaluated in a Dutch magnetic resonance imaging (MRI) screening (MRISC) study, in which annual imaging consists of mammography and MRI. Unfavourable side effects on health-related quality of life may arise from this screening process. We examined the short-term effects of screening for breast cancer in high-risk women on generic health-related quality of life and distress.

A phase II study of ET-743/trabectedin ('Yondelis') for patients with advanced gastrointestinal stromal tumours.

Primary or secondary resistance to imatinib may occur in patients with gastrointestinal stromal tumours (GISTs) while these tumours have repeatedly been shown to be highly resistant to conventional doxorubicin- and ifosfamide-containing regimens. The investigation of new drugs is therefore warranted in GIST. A phase II study was conducted between May 1999 and November 2000 in eight centres of the EORTC STBSG group to establish the efficacy and safety of ET743 ('Yondelis') in GIST previously untreated with cytotoxic chemotherapy before the imatinib era.

A metastasis of an adenocarcinoma in a BRCA1 mutation carrier, a diagnostic problem not solved by morphology alone.

To increase awareness of the possible coexistence of an ovarian and breast carcinoma in a BRCA1 mutation carrier, we describe a patient with primary ovarian cancer who developed distant metastases after 3 years and again after 6 years. Surprisingly, at that time an occult breast carcinoma was identified to be the origin of the 2 metastatic locations as confirmed by molecular analysis. Eventually the patient could be treated accordingly.

Ipsilateral breast tumour recurrence in hereditary breast cancer following breast-conserving therapy.

The overall rate of an ipsilateral breast tumour recurrence (IBTR) after breast-conserving therapy (BCT) ranges from 1% to 2% per year. Risk factors include young age but data on the impact of BRCA1/2 mutations or a definite positive family history for breast cancer are scarce. We investigated IBTR after BCT in patients with hereditary breast cancer (HBC).

Weekly paclitaxel as first-line chemotherapy for elderly patients with metastatic breast cancer. A multicentre phase II trial.

Paclitaxel is a cytotoxic agent with proven antitumour activity in metastatic breast cancer. Weekly administration of paclitaxel has demonstrated sustained efficacy together with a more favourable toxicity profile (e.g.

Long-term psychological impact of carrying a BRCA1/2 mutation and prophylactic surgery: a 5-year follow-up study.

Purpose: To explore long-term psychosocial consequences of carrying a BRCA1/2 mutation and to identify possible risk factors for long-term psychological distress.

Patients And Methods: Five years after genetic test disclosure, 65 female participants (23 carriers, 42 noncarriers) of our psychological follow-up study completed a questionnaire and 51 participants were interviewed. We assessed general and hereditary cancer-related distress, risk perception, openness to discuss the test result with relatives, body image and sexual functioning.