Trilateral retinoblastoma with unilateral eye involvement.

Trilateral retinoblastoma (TRb) is a rare combination of unilateral or bilateral retinoblastoma with an ectopic midline intracranial neuroblastic neoplasm (primitive neuroectodermal tumour) usually in the area of pineal gland or sellar region. TRb can occur with both familial and sporadic forms of retinoblastoma. An occurrence of this rare tumour in a 12-year-old boy who had unilateral retinoblastoma in association with ectopic suprasellar primitive neuroectodermal tumour (PNET) is reported here. To the best of our knowledge, this is the first case report in Pakistan on TRb with suprasellar mass.

Sphenoidal intracranial chondrosarcoma.

A 40-year-old male presented with headache, complete loss of vision in the right eye and decreased vision of the left eye. On examination, fundoscopy revealed secondary optic atrophy in the right eye and pale temporal disc in the left eye. MRI brain revealed a soft tissue mass arising from sphenoid sinus, eroding the overlying bone and extending into the sellar and parasellar regions up to the third ventricle. The MRI scan favoured the diagnosis of craniopharyngioma, most likely, while CT scan showed sellar floor erosion and calcification which favoured a pituitary lesion. The mass was also compressing the optic apparatus. The patient underwent excision biopsy through subfrontal approach. The histopathology report revealed it to be a chondrosarcoma. Chondrosarcoma is a rare malignant mesenchymal tumour of cartilageproducing cells; only 7% tend to involve craniocervical region. Maximal surgical resection followed by proton radiation therapy have clinically shown promising outcome.