OT-06 * MULTICENTRIC PRIMARY CENTRAL NERVOUS SYSTEM DIFFUSE LARGE B CELL LYMPHOMA IN AN INMUNOCOMPETENT ADOLESCENT, CASE REPORT

Abstract

BACKGROUND: Primary central nervous system lymphoma (PCNSL) accounts for 4 to 7% of all primary intracranial neoplasms and 1 to 2% of all lymphomas. PCNSL incidence has increased tenfold over the last three decades in both immunocompetent and immunocompromised individuals. CASE REPORT: We present the case of a female adolescent with no risk factors for immunosuppression. Her immunological profile was normal. Her main symptom was headache two years before being diagnosed with a primary central nervous system diffuse large b cell lymphoma. Left ptosis, facial paralysis, disarthria and diplopia appeared one year later. She developed paresthesias in both hands with loss of strength. One month before she had seizures. In the magnetic resonance a multilobulated heterogeneous mass in left ventricule was found with high contrast enhancement. In spinal cord at C1-C2, an intramedular heterogeneous lesion was also observed. Stereotactic biopsy was done reporting CD20+ primary central nervous system diffuse large b cell lymphoma with reactive gliosis. Cerebrospinal fluid was negative. No viral association was detected. DISCUSSION: PCNSL are a rare malignant neoplasm in pediatric age, and more common in male. Worldwide, near fourteen cases are diagnosed each year. Most of these cases appear in immunocompromised patients. This is a special case because of the association of lymphoma in an immunocompetent girl. Clinical features differs in the evolution time which is longer than expected for a lymphoma. Radiological imaging suggests a multicentric presentation or a previous non-diagnosed disease. These cases suggests that lymphoma have a different clinical, radiological and histological presentation when the localization is in central nervous system. Malignant tumors have a non-predictable evolution in this tissue. Do we have to treat it in the same way?