1. ISOLATED INTRA-OCULAR RELAPSES OF PRIMARY CEREBRAL LYMPHOMAS
N. Younan¹, S. Choquet¹, C. Soussain², V. Touitou¹, N. Cassoux1, A. Schmitt³, O. Chinot4, A. Amiel5, L. Taillandier6, K. Laribi7, T. Lamy8, H. Ghesquières9, J-P. Marolleau10, M-P. Moles11, A. Tempescul12, P. Agapé13, F. Jardin14, E. Gyan15, A. Brion16, G. Ahle16, A. El Yamani17, M. Bourniquel18, D. Genet¹, M. Le Garff¹, M. Costopoulos¹, A. Alentorn¹, K. Hoang Xuan¹, C. Houillier¹.
¹Paris, ²Saint-Cloud, ³Bordeaux, Marseille4, Toulouse5, Nancy6, Le Mans7, Rennes8, Lyon9, Amiens10, Angers11, Brest12, Saint-Herblain13, Rouen14, Tours15, Colmar16, Blois17, Toulon18, France.
Amsterdam 2017
At the end of this line, 78% of the patients were in complete response while 13% had a progressive disease, either in the brain or in the eye.
Outcomes of IIOR :
In primary CNS lymphoma (PCNSL), the preferential location is cerebral, at baseline and as well in relapses.
The prognosis is severe.
Initial intra-ocular location is also classical and can be either isolated or associated to brain location.
In contrast, isolated intra-ocular relapse (IIOR) is really rare and no research has been published about it up to now.
About the IIOR itself :
It occurs mainly in elderly people but with a preserved clinical status (median KPS of 80). For 87% of them, it’s a first relapse.
A large proportion of the patients (88%) had a symptomatic relapse with visual acuity impairement. A hyalitis was observed in 90%. An elevated rate of intra-ocular IL10 wa sobserved in 91%.
Statistical analysis
- Figure out the population at risk to develop an IIOR.
- Describe the clinical expression of the IIOR.
- Analyse their treatment and their prognosis.
We retrospectively studied patients treated between 1998 and 2017 within the French national expert network on cerebral lymphomas called “LOC”.
We selected patients who had a histologically proven primary CNS lymphoma with at least a cerebral involvement at diagnosis and an IIOR at any time of the disease. The patients were adults, immunocompetent and all had a high-dose methotrexate (HD MTX) based chemotherapy as first line treatment.
Half of patients without ASCT had received Ibrutinib or both at any time of disease.
CONCLUSIONS
At the end of this treatment, 60% were in complete response, while 40% had a progressive disease.
- IIOR of PCNSL is rare but not exceptional.
- Patients without any ocular involvement may develop an IIOR. Therefore, ocular examination at baseline and during the follow up is important.
- The IIOR are usually symptomatic but the KPS remains good even though the median age is high. That allows heavy and multiple treatments even though the median age of the patients is similar to those who relapse in the brain.
- Patients with IIOR relapse a lot, but mostly in the eye. Their BFS and OS is very good compared to those who relapse in the brain.
- The HDCT followed by ASCT doesn’t seem to improve these good results.
- Ibrutrinib and lenalidomlide are 2 news drugs that seem promising.
RESULTS
When we study the subsequent relapses, we note that the location of relapse is mainly ocular at the first relapse after IIOR and as well in all relapses.
In total we found 47 relapses in 27 patients.
46 patients met the inclusion criteria.
At diagnosis, the median age of the cohort was 65 year old. The patients had a median KPS of % of them had an associated ocular disease, while 35% didn’t. For a fifth of them, the ocular status wasn’t unknown or unsearched.
For the next 10 minutes, we’ll consider the IIOR of primary cerebral lymphomas.
In first line of treatment, they’ve all been treated by HD-MTX based chemotherapy (CT); about half of them also received Rituximab. None of them had HD-CT with autologous stem cells transplantation (ASCT).