Case 367 -- A Man in His 50s with Progressive Aphasia and Right-sided Hemiplegia

Contributed by Leena Lourduraj, MD
Published on line in October 2003


PATIENT HISTORY:

A man in his 50s was diagnosed with Glioblastoma Mulitforme of the left temporal lobe for which he underwent chemotherapy and external beam radiation therapy. He presented suddenly nine months later with progressive aphasia and right-sided hemiplegia. Following a physical and neurological examination it was noted that the patient again had a mass situated in the left temporal lobe (in the previous site of the surgery) associated with significant compression of the brainstem.

The CT scan of the brain showed a left temporal lobe mass, 4.9 x 4.1 cms with central areas of necrosis and extensive T2 prolongation around the lesion due to vasogenic edema. There was significant mass effect on the left superior cerebral peduncle and the left aspect of the pons. These findings were compared with the previous study done at the time of the initial diagnosis of Glioblastoma Multiforme and appeared to be significantly worsened.

The possibility of a recurrence /relapse was entertained and the patient was taken up for surgery.

The patient underwent left temporal craniotomy for debulking of the tumor. The tumor was noted to be calcified and necrotic and was mostly involving the middle fossa, the frontal lobe and the lateral portion of the cavernous sinus. The tumor was adherent to the internal carotid artery and middle cerebral artery.

MICROSCOPIC DESCRIPTION:

Paraffin sections of the specimen showed a biphasic tumor with distinctive glial and sarcomaotus, well defined components. One area was characterized by atypical cells with varying degrees of anaplasia and mitosis (Fig. 1). The tumor cells expressed GFAP(Fig. 2, right side of the picture shows the gliomatous component and the left side the sarcomatous component). The sarcomatous component of the tumor was not reactive to GFAP and was characteristically reticulin rich area (Fig. 3) and composed of spindle-shaped tumor cells showing herringbone pattern similar to fibrosarcoma (Figs. 4 and 5). The sarcomatous component of the tumor also showed areas of osteosarcoma (with evidence of malignant osteiod) (Figs. 6 and 7) and chondorsarcoma (composed of malignant cartilage) (Figs. 8 and 9). The cartilaginous areas of the tumor were positive for S100 (Fig. 10). The Ki 67 (Fig. 11) stain performed showed more than 50% proliferative activity in the tumor cell nuclei. The p53 stain (Fig. 12) was positive and was further confirmed by molecular genotyping, which showed allelic loss in 17p13. The tumor in addition showed extensive radiation effect (Fig. 13).

FINAL DIAGNOSIS


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