ABSTRACT
We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.
Subject(s)
Child , Humans , Male , Cerebellar Neoplasms , Cerebrospinal Fluid , Germ Cells , Neoplasm Metastasis , Neoplasms, Germ Cell and Embryonal , Pineal Gland , Pinealoma , Radiotherapy , Biomarkers, TumorABSTRACT
We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.
Subject(s)
Child , Humans , Male , Cerebellar Neoplasms , Cerebrospinal Fluid , Germ Cells , Neoplasm Metastasis , Neoplasms, Germ Cell and Embryonal , Pineal Gland , Pinealoma , Radiotherapy , Biomarkers, TumorABSTRACT
Lhermitte-Duclos disease of the cerebellum manifests itself as a slowly growing mass formed by abnormal neurons that appear more malformative than tumoral. Although most cases show satisfactory clinical evolution, several cases, not recognized in life, died abruptly, usually from respiratory arrest. We recently experienced a 19 year old male, who died suddenly during alcohol drinking. At autopsy, the cerebellum revealed thickening of the folia over two limited areas. Microscopically, the lesion was characterized by proliferation of abnormal neuronal cells with excessive myelination of their axons, disappearance of Purkinie cells and greatly reduced white mater. Theories regarding the pathogenesis of Lhermitte-Duclos disease were reviewed and discussed.