Pineocytoma Grade

This benign tumor originates from mature parenchymal cells with neuroendocrine and photosensory functions. More common in adolescents and young adults, it accounts for 15% to 30% of parenchymal tumors. It is slowly growing, grossly circumscribed, and may extend into the third ventricle (Fig. 11.26A). Histologically, the tumor consists of uniform cells with a small nucleus and a moderate amount of cytoplasm containing secretory granules. In silver-stained sections, the cells display short processes with club-like endings. They form

figure 1 i.26a

A. Pineocytoma. An 11-year-old girl presented with a 1-week history of headaches, vomiting, and confusion. She had a stiff neck, papilledema, slowly reacting pupils, and vertical gaze palsy (Parinaud syndrome). She died of hypothalamic dysfunction 2 weeks following a shunting procedure. An egg-sized pineal gland tumor compresses the midbrain tectum and extends into the third ventricle. It is loosely attached to the wall of the ventricle. B. The tumor cells, resembling pineocytes, form groups separated by mesenchymal septa (HE).

figure 1 i.26a

A. Pineocytoma. An 11-year-old girl presented with a 1-week history of headaches, vomiting, and confusion. She had a stiff neck, papilledema, slowly reacting pupils, and vertical gaze palsy (Parinaud syndrome). She died of hypothalamic dysfunction 2 weeks following a shunting procedure. An egg-sized pineal gland tumor compresses the midbrain tectum and extends into the third ventricle. It is loosely attached to the wall of the ventricle. B. The tumor cells, resembling pineocytes, form groups separated by mesenchymal septa (HE).

rosettes around acellular areas or are separated into lobules by thin-walled vessels. They immunoreact for synaptophysin and retinal S antigen, and variably for neurofilament protein and chromogranin A.

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