Pineal choriocarcinoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Synonyms and keywords: Pineal embryonal cell carcinoma; Pineal gland tumor; Brain tumor

Overview

• Pineal choriocarcinoma is a relatively rare, malignant, highly vascular neoplasm and accounts for a small proportion of all intracranial germ cell tumors. It is an aggressive tumor and has a propensity to metastasise systemically. Pure pineal choriocarcinoma tumors secrete β-HCG.
• On microscopic histopathological analysis, pineal choriocarcinoma is characterized by presence of intimately related syncytiotrophoblasts and cytotrophoblasts without formation of definite placental type villi. Syncytiotrophoblasts are large multi-nucleated cells with eosinophilic cytoplasm. They often surround the cytotrophoblasts, reminiscent of their normal anatomical relationship in chorionic villi. Cytotrophoblasts are polyhedral, mononuclear cells with hyperchromatic nuclei and a clear or pale cytoplasm.
• Pineal choriocarcinoma accounts for 5% of all pineal masses.
• The peak age at diagnosis for pineal choriocarcinoma is 20-30 years.
• Common complications of pineal choriocarcinoma include:

• CSF metastasis
• Intraventricular hemorrhage
• Coma
• Systemic metastasis

• The clinical presentation of pineal choriocarcinoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct. Symptoms of pineal choriocarcinoma include headache, vomiting, ptosis, and weakness.
• Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome.
• Common physical examination findings of pineal choriocarcinoma include papilledema, signs of precocious puberty, restricted extraocular movements, sluggish pupillary light reflex, stiff neck, hemiparesis, and abducent nerve palsy.
• Laboratory findings consistent with the diagnosis of pineal choriocarcinoma include abnormal CSF analysis, demonstrating xanthochromia.
• Head CT scan and brain MRI may be helpful in the diagnosis of pineal choriocarcinoma.
• On head CT scan, pineal choriocarcinoma is characterized by a round, calcified, homogeneous, enhancing mass in the third ventricle associated with the dilation of the lateral and third ventricles and periventricular lucency.
• On brain MRI, pineal choriocarcinoma is characterized by hyperintensity on T1-weighted images and signal drop-out and blooming on T2* sequences.
• Biopsy is generally done to confirm the diagnosis of pineal choriocarcinoma.
• The mainstay of therapy for pineal choriocarcinoma is radiotherapy and/or chemotherapy. Sometimes, surgical resection may be done.

References

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