CASE 1: 52 year old male presented with headaches and progressively increasing confusion. He complained of numbness and weakness on left lower and upper extremities. Radiological studies showed an ill-defined, variably enhancing mass with surrounding edema and mass effect in the right frontoparietal region (See MRI). A craniotomy was performed and a tissue sample was sent for frozen section.
[MRI, frozen section (For view of FS click here) and smear prep(For view of Smear click here] The permanent sections showed a malignant small cell neoplasm. The tumor was rather discohesive and had a predilection to perivascular regions, displaying an "angiocentric" pattern. However, in other areas there were pleomorphic cells with ample acidophilic cytoplasm and evidence of more cohesive architecture.
Immunohistochemical studies showed that most tumor cells were negative with antibodies against lymphoid, neuroendocrine and epithelial markers. There was diffuse GFAP positivity that was focally strong.
CASE 2: 8 year old male presented with a five-month history of decreased visual acuity, color vision, and headaches. Neurological exam revealed evidence of increased intracranial pressure. An MRI demonstrated a 4x4-cm mass associated with the optic chiasm and extending anteriorly and superiorly into the right frontal lobe. The mass had increased signal intensity on T2-weighted images, and showed marked enhancement [MRI]
The tumor was subtotally resected. Two years later, the tumor showed radiological evidence of progression and he underwent a second surgery. He remained symptoms-free after the second surgery except for decrease in his visual acuity in the right eye. Postoperative MRI revealed residual tumor. He underwent a third surgical resection due to radiological increase in the size of residual tumor three years after initial surgery and received postoperative radiation. Nine years after the initial surgery, he is alive and without symptoms or any substantial disability.
Microscopic evaluation of the smear(click here for smear H&E) and microscopic sections shows a rather cellular, solid neoplasm with somewhat biphasic appearance.(Click here for low power H&E slide)
(Click here for medium power H&E slide)
