P09.13 Clear cell renal carcinoma (CCRC) after diagnosis of Oligodendroglioma; real or false association?

Abstract

Oligodendrogliomas are more common in the 35–44 age group while the average age at diagnosis of CCRC is 60–64 years. However, around 7% of sporadic CCRC are diagnosed in patients younger than 40 years.

There are a high proportion of colorrectal cancer and nervous system cancers across all subtypes but no prior studies has established the relationship between CCRC and Oligodendrogliomas.

Between January 1997 and January 2016 we retrospectively identified 3 patients with Oligodendroglioma who developed CCRC. Inherited syndromes such as NF I and II, tuberous sclerosis, Li-Fraumeni, Turcot and Cowden syndrome were ruled out by imagen and clinical evaluation.

Patient 1: Male. Oligodendroglioma´s diagnosis at 38y. IDH1+ Codeletion 1p19q. He was treated with surgery alone without adjuvant treatment. At relapse he received Temozolomide monthly for 2 years. Twelve years after diagnosis of glioma left radical nephrectomy was done due to CCRC.

Patient 2: Male. Oligodendroglioma´s diagnosis at 53y. IDH1+ No codeletion 1p19q. He was treated with Surgery and Stupp protocol. Five years after diagnosis of glioma right partial nephrectomy was done due to CCRC.

Patient 3: Male. Oligodendroglioma´s diagnosis at 36y. IDH1+ No codeletion 1p19q. He was treated with Surgery and Stupp protocol. Five years after diagnosis of glioma right radical nephrectomy was done due to CCRC.

All three patients had mutated IDH1 and are alive at 19, 8 and 9 years from initial glioma diagnosis.

We did not find in the literature and PUBMED any reports associating CCRC with Oligodendrogliomas.

These results could suggest than CCRC may have an association with Oligodendrogliomas.

We don`t know if the presence of kidney cancer in patients with oligodendroglioma tumors may indicate a novel association as a cancer susceptibility trait.