Intravascular lymphomatosis (IL) is a rare entity only recently included in
lymphoma classification, whose main feature is the exclusive or predominant growth of neoplastic cells within blood vessels. The vast majority of the patients affected by IL belong to the 7th or 8th decade of life and present with
skin rash or CNS diffuse necrotic or demyelinating lesions. Case report. SS, a 13-year-old girl, was admitted to a Neurosurgery Unit because of endocranic
hypertension, where, after CT and MRI documenting a IV ventricle 3 cm diameter
tumor, she was submitted to complete
tumor excision: extemporary diagnosis was suggestive of
medulloblastoma. When referred to us she had persistent
fever with normal blood and spinal fluid cultures. Whole CNS MRI did not give evidence of residual or metastatic disease while CSF cytology showed only pleiocytosis. Treatment was started according to our ongoing protocol for
medulloblastoma with pre-radiation
chemotherapy. Before delivering
radiotherapy (RT), upon review of histologic specimens, the definitive diagnosis of IL B-phenotype was made. The girl was re-admitted and, after a complete re-staging,
chemotherapy was intensified according to our schedule for high-grade
B-cell lymphoma and CNS was irradiated up to a total dose of 25 Gy. She remained alive in continuous complete remission at 21 months after diagnosis. The case here reported is unique for age,
tumor presentation, and, so far, favourable outcome, in spite of the delayed histological diagnosis.