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RARE BREAST CANCER

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News

28/10/2003 - Lessons learned from the management of a rare genetic cancer.

An age-related progression from C-cell hyperplasia to medullary thyroid carcinoma is associated with various germ-line mutations in the RET proto-oncogene that could be used to identify the optimal time for prophylactic surgery. A European multicenter study conducted from July 1993 to February 2001, enrolled patients who had a RET point mutation in the germ line, were 20 years of age or younger, were asymptomatic, and had undergone total thyroidectomy after confirmation of the RET mutation. Exclusion criteria were medullary thyroid carcinomas of more than 10 mm in greatest dimension and distant metastasis. Altogether, 207 patients from 145 families were identified. There was a significant age-related progression from C-cell hyperplasia to medullary thyroid carcinoma and, ultimately, nodal metastasis in patients whose RET mutations were grouped according to the extracellular- and intracellular-domain codons affected and in those with the codon 634 genotype. No lymph-node metastases were noted in patients younger than 14 years of age. The age-related penetrance was unaffected by the type of amino acid substitution encoded by the various codon 634 mutations. The codon-specific differences in the age at presentation of cancer and the familial rates of concomitant adrenal and parathyroid involvement suggest that the risk of progression was based on the transforming potential of the individual RET mutation. These data provide initial guidelines for the timing of prophylactic thyroidectomy in asymptomatic carriers of RET gene mutations. Read the paper and the accompanying editorial on N Engl J Med of October 16, 2003.

News Archive

3/8/2001 WHY?
17/11/2001 W.H.O. Collaborating Centre for Cancer Control of Uncommon Tumours
10/12/2001 On November 30th, in the hall of the S. Pio X Private Hospital of Milan, Italy, the
18/12/2001 Post-meeting survey
3/12/2002 QUIZ
5/5/2002 Dear Colleagues,Notebook Veri Kurtarma
29/01/2003 New publication on rare cancers !
28/07/2003 Thyroid Cancer Treatment Receives Orphan Drug Status
02/08/2003 Breast cancer in men Kiralık Ofis Ankara
24/09/2003 GISTs: The revolution continues
17/11/2003 Successful photodynamic therapy for nonresectable cholangiocarcinoma.
07/01/2004 Gene associated with higher risk of melanoma in men
08/03/2004 Susceptibility to Common Adult Cancers in Retinoblastoma Survivors
29/03/2004 FDA Approves First Drug for mesothelioma
25/08/2004 Gene therapy cures malignant melanoma in mice
20/10/2004 FDA grants orphan drug status to two new anticancer drugs.
15/11/2004 New experimental drug for myeloproliferative disorder
20/01/2005 New leukemia treatment granted FDA orphan drug status.
28/02/2005 U.S. F.D.A. grants new orphan drug designation for a targeted therapeutic for head and neck cancer.
02/05/2005 Possible targets for a multiple myeloma vaccine
17/10/2005 Orphan drug troxacitabine approved for AML in EU
03/11/2005 FDA designes interleukin 21 as an orphan drug
01/12/2005 Zactima has received FDA orphan drug designation
25/01/2006 CETT Program for Rare Genetic Diseases
23/02/2006 New research studies of multiple myeloma