EB and Cancer

The EB-SCC Tissue and Cell Bank

What is EB-SCC?

Many patients with dystrophic, and sometimes junctional, EB develop skin cancer, also called squamous cell carcinoma (SCC). EB associated SCC (EB-SCC) behave differently from other SCC, which are commonly induced by chronic sun exposure. In individuals with EB, SCC develops at an earlier age and behaves in an aggressive manner. The reasons for the tumour behaviour in EB are not known. Therefore, it has not been possible to develop efficient therapeutic regimens. For these reasons, research on skin cancer in EB is urgently needed

What is the EB-SCC bank?

The EB-SCC bank exists for the benefit of researchers working to elucidate the cellular and molecular mechanisms underlying EB-SCC, as a prerequisite for the development of targeted, biologically valid therapies.

The EB-SCC bank is currently hosted centrally by the EB-Centre Freiburg (coordination centre of the German National EB Network), located in the Department of Dermatology at the University of Freiburg. Some tissue samples and cell lines are held at other collaborating centres in London and Dundee. The EB-SCC bank is currently supported through a project grant made by DebRA UK to the University of Freiburg. The project was approved by the ethical committee of Freiburg University Hospital and Medical Faculty, Approval Nr. 127/04, on June 2, 2004. For further information on the EB-SCC bank project, go to www.debra.org.uk/

What materials does the EB-SCC bank hold?

Samples of normal and EB-SCC tissue of patients with RDEB-HS, and EB-SCC of a patient with nH-JEB, have been analysed by conventional histopathology, prior to preparation for storage.

Tissue samples are stored as:

• formaldehyde fixed blocks for (immuno)-histopathology,
• some samples also as glutaraldehyde-fixed blocks for EM
• native frozen tissue for IF and biochemical analysis
• Limited numbers of cells or cell lines have been established from EB-SCC samples.

It should be noted that not all of the above exist from every tumour.

Enquiries about the materials held, or further information on the EB-SCC bank should be sent to:
eb-scc@uniklinik-freiburg.de

What data exist on EB-SCC bank materials?

The following (pseudonymised) data are collected for each EB-SCC:

• precise EB subtype;
• the underlying mutation(s);
• age of the patient and course of EB;
• expression pattern of the relevant protein(s) in the skin;
• clinical history and description of the EB-SCC;
• histopathology of the EB-SCC and possible metastases.

The German EB Network (www.netzwerk-eb.de) provides the patient registry and information system for the EB-SCC bank, with clinical and genetic information, histology of the tumours, and other information for the materials held. All patient information is pseudonymised with coded entries, in accordance with German federal and state legislation on data security and privacy of medical information.

How do I apply for research materials from the EB-SCC bank?

The quantity of EB-SCC materials is limited by many factors, notably the rarity of EB as an orphan disease, and difficulties in obtaining good-quality tissue samples of SCC from often chronically infected wound areas, and in establishing cell lines from such samples. Therefore, any research group wishing to access materials must provide a detailed proposal, which will be evaluated in terms of the likely contribution of the planned research to fulfilling the aims of the EB-SCC bank. This research-proposal review is carried out by an advisory panel appointed and managed by DebRA Europe, and by external peer-review.

Materials are normally provided free of charge for approved applications. However, if the request requires excessive work on the part of the EB-SCC bank, or the delivery/ transport method requested by the applicant is expensive, a charge may be made. The applicant will be notified of this charge on approval of the application and prior to delivery.

Unless you already know which tissue sample, cells, or cell line you require, the first step is to send an enquiry e-mail about the materials held to:
eb-scc@uniklinik-freiburg.de You will be sent confidential information about the materials, which you must not disseminate by e-mail, hard-copy, verbally, or in any other form, to enable you to complete your application form.

All applications should be made using this form, which should be sent by e-mail to:
clare.robinson@debra.org.uk