PRACTICE GUIDELINES

Soft tissue sarcoma: the clinically relevant basics and an update on systemic therapy options for patients with advanced disease

Summary

Sarcomas are a group of rare solid tumours arising from mesenchymal or connective tissue. This review focuses on soft tissue sarcoma and covers general topics such as the epidemiology, age distribution, site of disease, histogenesis, histological subtypes, prognosis and outcome of treatment. In more detail the article reviews current systemic treatment standards and selected adverse events of agents such as doxorubicin, ifosfamide, trabectedin and pazopanib, and briefly highlights some drugs that are used off-label in rare subtypes of sarcoma.

(BELG J MED ONCOL 2013;7(3):80–88)

Practice guidelines in the management of uveal melanoma

Summary

Uveal melanoma is a rare oncological disease. This incidence has remained stable for the past 50 years. There is no survival difference depending on the type of ocular treatment (enucleation versus radiotherapy versus tumour resection). Brachytherapy (Ru-106, I-125) presently remains the most common method for treating uveal melanoma. Despite adequate and early local treatment, half of the patients will develop metastatic recurrence with an average of 2.5 years after initial diagnosis. Clinical and histological prognostic factors have been identified, but some studies suggest that inactivation of BAP1 by chromosomal deletion or mutation is a key event driving metastasis development. Presently, no adjuvant treatment prevents those metastatic relapses. Nearly 90% of patients who relapse have only liver metastases. The median survival of those patients is about four months. Numerous trials evaluating the interest of exclusive liver treatment have failed to demonstrate an increase in survival, except surgery for solitary liver metastasis. Chemotherapy with dacarbazine remains the standard treatment of metastatic patients. New treatments targeting the signal transduction pathways or aiming at the stimulation of the immune system are under development.

(BELG J MED ONCOL 2013;7:20–26)

The role of laparoscopy in the evaluation and treatment of epithelial ovarian cancer

Laparotomy has been the standard approach for diagnosis and treatment of ovarian cancer. The goal of this article was to collect and summarise the evidence concerning the use of laparoscopy in ovarian cancer. We performed a Medline search of studies and reviews about the laparoscopic approach for evaluation of surgery of ovarian cancer.

Laparoscopy appears to be a safe, accurate and patient-friendly alternative for laparotomy in primary surgery for early-stage ovarian cancer and in accurate staging and evaluation of operability in advanced ovarian cancer. Very little evidence is available on the role of laparoscopy in advanced ovarian cancer. (BELG J MED ONCOL 2012;6:157–163)

The changing role of the axillary dissection in the treatment of breast cancer

The aim of this article is to highlight the recent changes in the surgical approach of the axilla in breast cancer patients. Axillary staging is dominated by the sentinel lymph node (SLN) biopsy, which is now widely practiced in clinically node negative patients. Most authors believe a SLN biopsy may even be performed in patients with a large or multifocal tumour, before neo-adjuvant systemic therapy, during pregnancy, after prior excisional biopsy and after prior mantle field radiotherapy of the breast. Intra-operative assessment of the SLN is recommended as it can identify half of all positive lymph nodes. It is generally accepted that it is safe to omit an axillary lymph node dissection (ALND) in patients with a negative SLN or with only isolated tumour cells (<0.2 mm) in the SLN. Moreover, in a subset of patients with a micro-/macrometastasis in the SLN it might not be necessary to perform a completion of ALND. We suggest to accept the option of omitting completion of ALND in frail patients with a positive sentinel lymph node on final pathology OR in these patients with, on final pathology, one or two positive SLNs AND a grade I or II tumour smaller than 4 cm AND adjuvant radiotherapy on the whole breast or chest wall. In conclusion, an increasingly tailored surgical approach is guiding the management of the axilla for women with early breast cancer. (BELG J MED ONCOL 2012;6:87–95)

An update on the systemic treatment of renal cell cancer

Renal cell carcinoma is a common malignancy affecting men and women sporadically or as part of an inherited syndrome. Upregulation of VEGF and other growth factors due to accumulation of HIF in combination with an activation of the mTOR pathway are known to be important parts of the pathogenesis. These signaling pathways are therapeutic targets of monoclonal antibodies, small-molecules kinase inhibitors (TKI’s) and mTOR-pathway inhibitors and currently constitute the mainstay of metastatic RCC treatment. During the last decade, treatment options for patients with advanced renal cell carcinoma, a disease resistant to cytotoxic chemotherapy, have improved significantly with increasing survival rates. Several clinical trials are ongoing and new results are expected in the coming years. In Belgium, three TKI’s, two mTOR-inhibitors and one anti-VEGF monoclonal antibody in combination with IFN-α are reimbursed for the treatment of advanced renal cell carcinoma. Sunitinib can be administered in first line and everolimus from second line on to patients with low- or intermediate risk disease. Therapy with bevacizumab/IFN-α is an alternative first line option. Temsirolimus is an option in first line for patients with high risk disease. Sorafenib has shown positive results in patients pretreated with cytokines. Recently, pazopanib has become available as a first line treatment for patients with advanced renal cell carcinoma.

(BELG J MED ONCOL 2012;6:13–21)