Pineal germ cell tumor in adult monochorionic monoamniotic twins at UMAE 25, Monterrey, Nuevo León: Case report and literature review
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Abstract
The pineal región is a deep midline area of the central nervous system. Because it includes the pineal gland and adjacent tissues of different embryological origins, various types of tumors may arise in this región. Germ cell tumors are functionally classified as secretory and non-secretory according to their production of tumor markers such as alpha-fetoprotein, beta- human chorionic gonadotropin, and placental alkaline phosphatase, which are characteristic of different tumor subtypes and present at varying concentrations depending on the histological subtype. The clinical presentation is related to the deep location of the lesion, commonly manifesting signs of intracranial hypertension secondary to obstructive hydrocephalus of the cerebral aqueduct. Diagnosis requires a multidisciplinar approach, including neuroimaging studies such as cranial computed tomography and brain magnetic resonance imaging, as well as paraclinical studies involving the previously mentioned tumor markers measured in serum and, with greater specificity, in cerebrospinal fluid (CSF), preferably obtained by lumbar puncture. These findings are considered when determining either cytoreductive neurosurgical treatment or conservative management with radiotherapy and chemotherapy. We present the case of two monochorionic monoamniotic twin brothers. The first twin developed a pineal tumor lesion at 25 years of age, presenting with signs of intracranial hypertension, and underwent surgical resection followed by adjuvant therapy. Five years later, his twin brother (twin number 2) presented with the same clinical picture, requiring ventricular shunting. Given his brother’s history, a diagnostic protocol including neuroimaging and lumbar puncture for CSF analysis was performed, revealing elevated tumor markers, predominantly placental alkaline phosphatase. Based on these findings, treatment with radiotherapy and chemotherapy was initiated. During outpatient follow-up, complete absence of the tumor was demonstrated, without the need for neurosurgical intervention. In this second adult twin, successful empirical treatment was achieved-an approach predominantly reported in pediatric patients-thus opening the possibility for further investigation regarding a potential associated genetic process.
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Pineal germinoma, monochorionic-monoamniotic twins, tumor markers (alpha-fetoprotein, placental alkaline phosphatase, beta-human chorionic gonadotropin)
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