ABSTRACT
Cerebellar mutism is a rare phenomenon often described in children following surgical intervention in the posterior fossa. In this report we present a very unusual case of pre-operative cerebellar mutism in an adult in the context of cognitive-affective syndrome caused by cystic hemangioblastoma.
Subject(s)
Affective Symptoms/etiology , Cerebellar Neoplasms/psychology , Cognition Disorders/etiology , Hemangioblastoma/psychology , Mutism/etiology , Cerebellar Neoplasms/diagnosis , Cerebellar Neoplasms/surgery , Hemangioblastoma/diagnosis , Hemangioblastoma/surgery , Humans , Male , Middle Aged , SyndromeABSTRACT
Chordoid glioma of the third ventricle is a rare glial tumour whose precise histogenesis remains uncertain. We describe two cases that presented recently to our department and review the background literature. The neoplasm tends to occur in women and its clinical presentation is variable, resulting from acute hydrocephalus or impingement upon local structures. However, the radiological appearance is distinct, with an ovoid shape, hyperdensity and uniform contrast enhancement on computerized tomography and magnetic resonance imaging. Intraoperative smear diagnosis is difficult because of the lack of specific features, although the presence of metachromatic extracellular mucin may be useful. The characteristic histological appearance is that of cords and clusters of cohesive, oval-to-polygonal epithelioid cells with abundant eosinophilic cytoplasm and a mucinous background. There is often a mixed chronic inflammatory infiltrate with lymphocytes and plasma cells with Russell bodies. The main differentials for histological diagnosis include chordoid meningiomas, pilocytic astrocytomas and ependymomas. An immunohistochemical panel including antibodies to glial fibrillary acidic protein, CD 34, epithelial membrane antigen, pan cytokeratin, S100 and vimentin can be used to distinguish between these possibilities. Ultrastructurally the tumour cells have basal lamina and microvilli, reminiscent of ependymomas. The clinical outcome in our cases was poor because of the location of the lesion and its close relation to the hypothalamus. Limited follow-up after surgery with or without radiotherapy suggests that as-full-as-possible resection favours a better outcome, although surgery in this area carries significant operative risks.
Subject(s)
Choroid Plexus Neoplasms/pathology , Choroid Plexus Neoplasms/physiopathology , Glioma/pathology , Glioma/physiopathology , Third Ventricle/pathology , Adult , Choroid Plexus Neoplasms/ultrastructure , Diagnosis, Differential , Female , Glioma/ultrastructure , Humans , Immunohistochemistry , Magnetic Resonance Imaging , Microscopy, Electron, Transmission , Third Ventricle/ultrastructureABSTRACT
TSH-secreting pituitary tumours are rare but difficult to treat due to a combination of refractory hyperthyroidism and low surgical cure rates. We describe the case of a 21-year-old woman who, despite twin pregnancy, became euthyroid and had dramatic tumour shrinkage on octreotide treatment. To our knowledge, this is the first description of the use of octreotide for a TSH-secreting pituitary adenoma throughout pregnancy.
