Abstract
Cases of the Month, July to September, 2002
The July 2002 Case of the Month (COM). This 66‐year‐old Caucasian female presented with gradually increasing protrusion of her left eye over a one‐year period. She complained of increased tearing and foreign body sensation. The physical examination revealed a visual acuity of 20/20, normal color testing, full vision field with motility of her left eye limited in lateral gaze. Pupils were round, symmetric, with no afferent pupillary defect noted. On external examination, her left eye was grossly proptotic with resistance to retropulsion. She had 4 mm proptosis of the left eye. Computed tomography and MR imaging demonstrated a left retro‐orbital mass with gadolinium enhancement and focal remodeling of orbital bones. She underwent surgical resection of tumor with a diagnosis of solitary fibrous tumor, and postoperatively she was symptom free. The histopathological differential diagnoses of spindle cell neoplasms of the orbit are discussed. Five months after surgery, no evidence of tumor recurrence was seen on neuroimaging and her vision was 20/20.
The August 2002 COM. A 21‐year‐old male presented with a single episode of generalized tonic clonic seizures. Radiology revealed a cystic tumor with mural nodule suggestive of a pilocytic astrocytoma. However, histopathological examination and electron microscopy revealed features of an intracerebral schwannoma. Therefore, although rare, in an intracerebral cystic lesion with mural nodule, the possibility of an intracerebral schwannoma should be entertained. This is important because this is a benign tumor with favourable response to resection.
The September 2002 COM. A 24‐year‐old female presented with a history of 3 generalized seizures, the first of which had occurred 6 months before admission. Her neurological examination was normal, but upon admission her MRI showed a small cystic lesion in the left parieto‐occipital region. The lesion was hyper‐intense on T‐2 weighted images and did not show contrast enhancment. At surgery, the tumor was found to be deep to the cortex and was a cyst with amber fluid surrounded by gliotic brain. Microscopically, the tumor was well‐demarcated from the surrounding tissues, which showed reactive changes, including Rosenthal fibers. The tumor was composed of GFAP‐positive glial cells, which were arranged in a pseudopapillary fashion around blood vessels. In between, the tumor cells were positive for neuronal markers. The diagnosis was papillary glioneuronal tumor (PGNT), a relatively recently described lesion that may be a variant of ganglioglioma. The current literature on PGNTs is reviewed.
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References
- 1. Bouvier‐Labit C., Daniel L., Dufour H., Grisoli F., Figarella‐Branger D. (2000) Papillary glioneuronal tumour: clinico‐pathological and biochemical study of one case with 7‐year follow up. Acta Neuropathol 99: 321–326. [DOI] [PubMed] [Google Scholar]
- 2. Komori T., Scheithauer BW, Anthony DC, Rosenblum MK, McLendon RE, Scott RM, Okazaki H., Kobayashi M. (1998) Papillary glioneuronal tumor. A new variant of mixed neuronal‐glial neoplasm. Am J Surg Pathol 22: 1171–1183. [DOI] [PubMed] [Google Scholar]
- 3. Nelson JS, Bruner JM, Wiestier OD, VandenBerg SR (2000) Ganglioglioma and gangliocytoma. In: World Health Organization classification of tumours: Pathology and genetics of tumours of the nervous system, Kleihues P., Cavenee WK (eds.), pp.71–82, IARC Press: Lyon . [Google Scholar]