RESUMO
Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease (LDD) especially in children are extremely rare. In this report we add one further case to this rare entity. A three year old boy with a history of cerebellar and brain stem compression signs was presented in unconscious condition. Computerized tomography (CT) scan revealed a well defined lesion mixed with area of calcification in the right cerebellum and severe obstructive hydrocephalus. Ventricular shunting was performed followed by gross total tumor removal three days after shunting. In the follow up period, the patient showed almost total resolution of all neurological deficits. MRI has been an imaging tool to preoperatively diagnose this disease; otherwise it would be uncertain preoperative diagnosis. However, in this report we would like to emphasize that not all uncertain diagnosis of LDD leads to palliative treatment. Well demarcated lesion may account for the safe surgical resection of this disease.
RESUMO
Solitary osseous plasmacytoma of the orbit is very rare. It can be part of generalized disease (multiple myeloma) or as localized disease, presenting as orbital tumor. We report a case of solitary osseous plasmacytoma of the orbit with multiple myeloma. This 43-year old male presented with 6 months history of proptosis and decreased visual acuity. CT scan showed an osteolytic, well demarcated lesion with homogenous contrast enhancement in the superolateral part of the right orbit. The tumor including the osteolytic lesion was totally removed. The pathological report revealed a plasmacytoma. Further investigation showed that the plasmacytoma was part of generalized multiple myeloma. The patient was treated with radiation and combination chemotherapy postoperatively. Twenty months after surgery, the local tumor remained well controlled. However, there was systemic progression of the disease, with multiple cutaneous mass and pleural effusion. These resulted in the patient’s deterioration and fi nal demise.
